exam 3 study guide

seizures:

definition of epilepsy

***traditional definition: the tendency to ***experience recurrent unprovoked seizures; “a recurring predisposition to seizures”

• diagnosis required 2 or more unprovoked seizures >24 hours apart

new definition:

• ***2 unprovoked seizures >24 hours apart

• ***OR 1 unprovoked (or reflex) seizure and probability of recurrence at least 60% over 10 years

  • same rate of recurrence as having had 2 seizures

  • usually based on MRI and/or EEG findings (e.g., a brain tumor, abnormal brain scan findings)

• OR diagnosis of a specific epilepsy syndrome

partial or generalized

• based on seizure types and EEG findings

• about 70% partial; 30% generalized

primary or secondary

• primary

  • idiopathic/no obvious cause

  • neurologically normal except for seizures (many of these syndromes are now known to have genetic influence)

• secondary

  • symptomatic

  • some obvious cause/lesion (e.g., brain injury, brain tumor, brain developmental problem)

  • neurologically abnormal (subtle or grossly apparent)

seizures:

characteristics of focal vs general onset

differences in origin

• focal

  • focal aware:

    • seizures that come from 1 part of the brain

  • focal impaired awareness:

    • seizures that come from 1 part of the brain

  • focal to bilateral tonic-clonic:

    • seizure starts in 1 spot but then spreads to the whole brain

  • ~70% originate in temporal lobes

  • ~30% originate in frontal lobes

  • <1% originate in parietal/occipital lobes

• general onset

  • whole brain fires off at once

clinical manifestation

• focal

  • focal aware:

    • person doesn’t lose consciousness/are aware of what’s going on during the seizure

  • focal impaired awareness:

    • person loses consciousness/have impaired awareness of what’s going on during the seizure

  • ~70% originate in temporal lobes

    • olfactory, gustatory, experiential, auditory, visual (language if dominant hemisphere)- very primitive functions!

      • relatively, things smell funny, things taste funny, experience deja vu, hear hums and ringing, see flashing lights or colors

    • behavioral arrest, asymmetric posturing, automatisms (automated repetitive behaviors)

      • typically, akinetic- lack of physical movement

  • ~30% originate in frontal lobes

    • may be bizarre, hyperkinetic, appear non-epileptic

    • frequently occur in sleep

    • usually brief, typically lasting seconds (whereas temporal lobe seizures lasts for minutes)

  • <1% originate in parietal/occipital lobes

    • sensory, visual

• general onset

  • tonic-clonic:

    • stiffening followed by rhythmic jerking + convulsions

  • tonic:

    • abrupt stiffening/posturing

  • clonic:

    • rhythmic/repetitive jerking movements

  • myoclonic:

    • brief jerking movements- like 1 lightning strike

    • may be followed by tonic-clonic or atonic pattern

  • atonic:

    • sudden loss of muscle tone

  • absence:

    • staring, behavior arrest

    • typical or atypical

    • myoclonic or with eyelid myoclonia

    • more common in kids

seizures:

safety procedures during a seizure

basic epilepsy management

• seizure calendar

  • when did it happen and what were you doing when it happened?

• seizure triggers

  • stress

  • sleep deprivation

  • intercurrent illness (e.g., having a fever at the same time)

  • menses (e.g., more likely to occur when you’re on your period)

  • drugs and alcohol

  • treatment non-compliance (e.g., not taking medication)

• safety

• seizure first aid

  • DO:

    • look for medical ID

    • protect from injury (e.g., move them to a safer location), especially their head

    • loosen ties, collars

    • turn on side (e.g., get in recovery position + open up airway)

    • reassure the person when consciousness returns

    • call ambulance if:

      • seizures >5 minutes

      • multiple seizures

      • pregnant

      • diabetic

      • injured

    • time the seizure

  • DON’T:

    • don’t try to restrain the person

    • don’t try to give food or liquids

    • don’t put anything in the person’s mouth or try to hold their tongue

    • don’t use artificial respiration unless not breathing after seizure ends or they have inhaled water

• antiepileptic drugs (AEDs)

• psychosocial issues

seizure:

typical signs and symptoms

seizure= the clinical manifestation of abnormal and excessively synchronized activity in a set of cortical neurons

• focal

  • focal aware:

    • person doesn’t lose consciousness/are aware of what’s going on during the seizure

  • focal impaired awareness:

    • person loses consciousness/have impaired awareness of what’s going on during the seizure

  • ~70% originate in temporal lobes

    • olfactory, gustatory, experiential, auditory, visual (language if dominant hemisphere)- very primitive functions!

      • relatively, things smell funny, things taste funny, experience deja vu, hear hums and ringing, see flashing lights or colors

    • behavioral arrest, asymmetric posturing, automatisms (automated repetitive behaviors)

      • typically, akinetic- lack of physical movement

  • ~30% originate in frontal lobes

    • may be bizarre, hyperkinetic, appear non-epileptic

    • frequently occur in sleep

    • usually brief, typically lasting seconds (whereas temporal lobe seizures lasts for minutes)

  • <1% originate in parietal/occipital lobes

    • sensory, visual

• general onset

  • tonic-clonic:

    • stiffening followed by rhythmic jerking + convulsions

  • tonic:

    • abrupt stiffening/posturing

  • clonic:

    • rhythmic/repetitive jerking movements

  • myoclonic:

    • brief jerking movements- like 1 lightning strike

    • may be followed by tonic-clonic or atonic pattern

  • atonic:

    • sudden loss of muscle tone

  • absence:

    • staring, behavior arrest

    • typical or atypical

    • myoclonic or with eyelid myoclonia

    • more common in kids

seizure:

predisposing conditions

acute symptomatic (aka provoked) seizures may result from any CNS insult; acute symptomatic seizures are usually tonic-clonic

• fever, especially in childhood (febrile seizures)

• alcohol/drug withdrawal

• substance abuse

• metabolic disturbances

• toxic reactions

• acute CNS infection, injury, stroke, etc.

causes of epilepsy (all ages, from most to least)

• idiopathic

• vascular

• congenital

• trauma

• neoplastic

• degenerative

• infection

epilepsy risk factors

• perinatal insult

• developmental delay

• febrile seizure

• CNS infection

• severe head injury

• family history of epilepsy

seizure:

progression of disease

epilepsy morbidity and mortality

• neurological (including cognitive) impairments

• AED adverse effects

• falls, fractures, contusions, lacerations, burns

• drowning

• vehicular accidents

• status epilepticus

• anxiety, depression, psychosis, suicidality

• cardiac/respiratory compromise with seizures

• sudden unexpected death (SUDEP)

other quality of life issues

• stigma

• fear, anxiety, loss of control

• educational impairment

• unemployment, underemployment

• loss of driving privileges

• decreased leisure activities, socialization

• sexual dysfunction; reproductive issues

seizure:

diagnostic tests and therapeutic interventions

how epilepsy is diagnosed

• history

  • recurrent stereotyped episodes (same symptoms everytime)

    • description of event (witness often better than patient)

  • risk factors

    • perinatal insult, developmental delay, febrile seizure, CNS infection, severe head injury, family history of epilepsy

• physical and neurologic examination

  • may be normal

• laboratory studies

  • frequently normal

• electroencephalogram (EEG)

• neuroimaging (MRI)

• recording seizures with continuous video- EEG monitoring

  • if diagnosis uncertain or not responding to treatment

interventions

• antiepileptic drugs

• surgery

• epilepsy treatment should be individualized, with evaluation for other options if >- medications fail

MS:

pathophysiology (autoimmune attack on myelin)

MS is a disorder of the central nervous system (CNS)- specifically, an autoimmune disorder, meaning the immune system attacks the healthy parts of the body

• primary target is the protective sheath (*myelin) that covers nerve fibers (axons)

  • damage to myelin sheath causes communication problems between the brain and the rest of your body

    • the damaged areas develop scar tissue, which gives the disease its name

*myelin typically promotes efficient transmission of nerve impulses along the axons

MS:

pseudobulbar affect (emotional lability)

neurological condition characterized by sudden, uncontrollable outbursts of laughter or crying that are often disproportionate or inappropriate to the situation

• can be socially isolating and distressing for individuals with MS, as the sudden and uncontrollable outbursts can be embarrassing and difficult to manage

MS:

typical signs and symptoms

damage may produce a variety of neurological symptoms that vary, depending on:

• amount of nerve damage

• which nerves are affected

common symptoms (from attack of the myelin sheath)

• muscle weakness

• sensory changes

  • numbness or tingling

  • 60-80% of people with MS experience heat sensitivity

  • Uhtoff’s Syndrome= worsening of neurological symptoms when people are overheated due to weather, exercise, fever, saunas, and hot tubs

• vision problems

• fatigue (MS fatigue= subjective lack of physical and/or mental energy that is perceived by person to impact ability to participate in usual and desired activities)

• dizziness and vertigo

• sexual problems

• pain

• gait difficulties

• spasticity

• bladder problems

• bowel problems

• cognitive decline

  • decreased attention span

  • decreased short-term memory (NOT long-term memory)

  • difficulty with learning process- inability to make sense of new information (NOT general intelligence)

  • decreased information processing skills

  • verbal fluency (NOT conversational skills)

    • impaired word-finding (NOT reading comprehension)

  • impaired multi-tasking skills/higher-level problem-solving skills

• “MS Hug” but it really hurts (i.e., like an electrical sensation)

less common symptoms

• speech deficits

• tremors

• breathing issues

• headaches

• seizures

• hearing loss

symptoms corresponding to location of damage

• optic nerve —> vision loss (optic neuritis)

• spinal cord —> numbness and tingling

• spinal cord or brain motor pathways —> weakness

• cerebellum and brainstem —> imbalance and incoordination

• brainstem —> double vision

secondary symptoms

• depression

• unemployment

• decreased organizational skills/clutter

• decreased nutrition

• decreased sexual function

• decreased socialization

• decreased quality and quantity of sleep

MS:

predisposing conditions

prevalence of MS:

• MS may affect more than 2.8 million people worldwide, with approximately 1 million cases originating in the US

• average age of onset: 30 years old

  • most people with MS are diagnosed between the ages of 20 and 45

  • people are occasionally diagnosed outside of this age range

• 3:1 female to male ration but the symptoms/trajectory are much worse in men

etiology of MS: there is no single cause- it is an illness that occurs in genetically vulnerable individuals exposed to certain environmental conditions

• cause of MS is unknown, however it is believed to be due to a combination of biological and environmental factors

• genetics

  • there is not one single gene, but a combination of genes that predisposes one to get MS

  • close relatives of a person with MS (parent, child, sibling) have ~3% risk of getting the disease

  • identical twins have 25-30% chance of acquiring MS if the other twin is diagnosed

  • non-identical twins have a 4% change if the other twin has the disease

• infectious agents: strong evidence for a role of Epstein-Barr virus (EBV) triggering MS

  • EBV infection is a leading trigger of MS, but it does not act alone

• environment

  • occurs more frequently in areas that are furthest from the equator

  • believed that the risk depends on where a person spends the first 15 years of their life

  • rate of being diagnosed is twice as high in northern states in USA, compared to southern states

  • incidence is higher in colder climates

  • possible link to vitamin D deficiency

• other risk factors:

  • obesity

  • sodium intake

  • smoking

MS:

progression of the disease and how demyelination of axons plays a role

disease courses:

• *relapsing-remitting MS (RRMS)

  • no symptoms —> symptoms —> no symptoms

• primary-**progressive MS

  • constantly becoming worse, no going back to 0

• secondary-**progressive MS (SPMS)

• [clinically isolated syndrome]

*relapsing course can be:

• active or inactive

• worsening or not worsening

• what is a relapse?

  • neurological disturbance (or flare-up, MS attack, exacerbation)

  • may be a subjective report or an objective observation

  • must last at least 24 hours

    • in absence of fever or infection

  • must be a novel symptom

    • fluctuation of symptoms is not considered to be a relapse (i.e., weakness, sensory changes)

    • must be a new presentation

**progressive courses can be:

• active with or without progression

• not active with or without progression

demyelination

== the loss of myelin sheath insulation around nerve fibers (axons) disrupts nerve signal transmission and contributes to the development of neurological symptoms

• leads to slowed or blocked nerve conduction, causing symptoms like numbness, paralysis, and vision loss

• chronic demyelination can damage axons themselves, leading to long-term neurological disability

MS:

diagnostic tests and therapeutic interventions

diagnostic tests

• MRI

• clinical examination

• evoked potential

• spinal tap

McDonald et al. criteria of diagnosis:

• separation in time: 2 or more neurological symptoms or signs; onset separated by more than 30 days

• separation in space: abnormalities found in 2 or more different sites in the nervous system

• no better explanation: doctor has considered other possibilities and found no evidence of another cause (i.e., needs to be proven that it is not anything else before you are diagnosed with MS)

Expanded Disability Status Scale (EDSS)

• method of quantifying disability

• scales from 0 (no disability) to 10 (death)- measured in .5 unit increments

• limitations: reliance on walking as the main measure of disability

treatment

• no cure for MS at this time

• medical management involves treating and preventing relapses and symptom management

• steroids (i.e. solumedrol)

  • usually used to treat acute relapses

    • tend to get better over time w/ steroids

• disease-modifying therapy (DMT):

  • purpose is to reduce likelihood of relapse (no reversal of anything you had before- goal is to just reduce MS attacks)

• medications

  • injectables (OG treatment)

  • infusion (newer treatments)

  • oral (newer treatments)

  • medical marijuana

    • reduce chronic pain

    • help manage spasticity

    • helps control bladder/bowel management

    • increases appetite

    • reduces tremors

    • reduces insomnia

    • functional considerations (negative):

      • can impact mood

      • can increase fatigue levels

      • can increase blood pressure and heart rate

      • acute use can affect memory, learning, and attention

• MS care team

  • MS neurologist

  • nursing team

  • social worker

  • patient services/navigation

  • infusion center team

  • physical therapy

  • speech therapy

  • urology

  • behavioral health

  • primary care

  • pain management

MS:

OT goals/focus for specific conditions

OTs role when evaluating individuals with MS- ESPECIALLY in the context of independence in ADLs

• physical functioning:

  • positioning

  • ROM

  • strength

  • coordination

  • spasticity

• vision:

  • acuity

  • oculomotor ROM and coordination

  • eye teaming

  • visual fields

  • depth perception

• sensation:

  • light touch

  • proprioception

  • kinesthesia

  • temperature

  • steroegnosis

• fatigue:

  • Self-Report Scale

  • ask:

    • “does fatigue impact your ability to perform usual and desired activities?

    • “are you ever too tired to cook or eat a meal?”

    • “are you ever too fatigued to shower? to brush your teeth? to change out of your pajamas?”

• cognition:

  • safety awareness

  • attention

  • memory

  • executive functioning

  • problem-solving skills

  • ask:

    • “do you ever forget to turn off the stove when you are cooking?”

    • “do you ever forget to bring your wallet/ keys/ phone with you when leaving the house?”

• other:

  • ask:

    • “do you avoid drinking or eating when out in public to avoid having urinary/bowel accidents?

    • “do you avoid inviting friends over because of clutter

    • “how often do you engage in socialization?”

    • “when is the last time you had a cup of coffee with a friend?

    • “do you have difficulty falling or staying asleep?”

    • “in the past 2 weeks, have you forgot to take any of your medication?”

OT specific interventions

• fatigue management

  • education- essneital to validate and help define MS fatigue, as it is an invisible symptoms and can be difficult for people to be aware of; even harder for family members/caregivers to understand

    • what is MS fatigue?

      = subjective lack of physical and/or mental energy that is perceived by person to impact ability to participate in usual and desired activities

    • what are cofactors related to MS that can be addressed?

      • sleep

      • nutrition

      • dehydration

      • stress

      • deconditioning

      • heat sensitivity

    • resources for further understanding/education of family and caregivers

  • energy conservation strategies

    • planning

    • pacing

    • prioritizing

    • positioning

    • permission

  • create an individualized plan

    • weekly schedule

    • fatigue diary

    • strategies for improving self-monitoring of fatigue levels

    • practice implementation of strategy use in specific ADLs and IADLs

• cooling techniques (for those very sensitive to heat/temperature)

  • adjust the temperature in your environment

    • rest in rooms that are out of direct sunlight

    • take lukewarm showers/baths

    • wear layered clothing

  • drink plenty of fluids

  • adding rest breaks during exercise/daily activity

  • planning day accordingly to avoid being outside during peak temperature hours

  • prescription/trial of cooling products

• bladder management

• clutter management

• functional cognitive retraining (not just attention; attention to do ADLs)

  • awareness training

  • task-specific training

    • medical management

    • meal prep

    • bill paying

  • strategy training

    • use of memory aids:

      • alarms

      • schedules

      • reminder systems

      • applications

• vision

  • visual skills retraining

  • compensatory techniques/technology

• exercise:

  • strengthening

  • coordination retraining

  • !!! very beneficial for symptom management and improving overall wellbeing in MS !!!

    • can preserve and enhance function and mobility impacted by MS

    • can promote recovery from relapse

    • may reduce spasticity

    • may have a positive effect on cognition

    • beneficial for fatigue management

    • aerobic exercise can improve mood and reduce stress levels

  • considerations

    • disregard phase “no pain no gain”!

    • avoid overheating (e.g., adjust outfits or time of day when exercising)

    • implement fatigue management strategies throughout exercise routines

• ADL retraining: combination of restorative and compensatory approaches each session

  • compensatory

  • restorative

  • e.g., patient goal: “i want to improve my ability to get dressed independently”

    • intervention may include:

      • core strengthening (e.g., important for lower body dressing)

      • coordination retraining (e.g., important for fastening belts)

      • functional visual retraining (e.g., convergence, saccades)

      • fatigue management education (e.g., having enough energy to do things)

      • fall prevention education (e.g., being safe while doing activities)

      • adaptive equipment (e.g., button hook, sock aide, shirts with magnetic fasteners)

      • education on body mechanics/activity adaptation

  • e.g., patient goal: “i want to be able to cook a meal by myself”

    • intervention may include:

      • cognitive interventions

        • use of compensatory strategies (e.g., alarns, printed recipe, checklists)

        • awareness training

      • activity modification

        • cooking seated

        • top-down approach vs bottom-up approach?

      • environmental modification

        • organizing kitchen

        • use of adaptive equipment

      • restorative interventions

        • addressing balance, AROM, strength, coordination, ataxia, visual deficits

      • education

        • fall prevention, fatigue management, body mechanics

seizures and MS quiz:

Multiple Sclerosis (MS) is a disorder of the

• Central nervous system (CNS)

• Peripheral nervous system (PNS)

• Muscular System

• Nervous System

Central nervous system (CNS)

seizures and MS quiz:

Damage to _________________ causes communication problems between the brain and the rest of your body

• axon

• nerve root

• nerve cell nucleus

• myelin sheath

myelin sheath

seizures and MS quiz:

possible symptoms of MS

• sexual disfunction

• tremors

• weakness

• all of the above

all of the above

• sexual disfunction

• tremors

• weakness

seizures and MS quiz:

Epilepsy is the tendency to experience recurrent provoked seizures

true

false

false: it is the tendency to experience recurrent unprovoked seizures

seizures and MS quiz:

Seizures can be triggered by:

• Drugs & Alcohol

• Stress

• Sleep deprivation

• All choices are correct

 

All choices are correct

• Seizure triggers

  • Stress

  • Sleep deprivation

  • Intercurrent illness

  • Menses

  • Drugs & Alcohol

  • Treatment non-compliance

peds CP:

medical treatments for spasticity

selective dorsal rhizotomy (SDR):

• goal is decreased spasticity in selected muscle groups

• cutting nerve roots

• potential long term complications

  • lower spine changes

  • scoliosis

• eligibility criteria:

  • pure spasticity, where spasticity is not used for function; some centers recommend limiting SDR to children with spastic diplegia

  • selective motor control, adequate trunk balance, and lack of profound underlying weakness

  • 3-8 years old

antispasticity meds

• baclofin infusion

  • pump and catheter to deliver antispasticity meds

  • pump is refilled monthly (more common with MS clients than children with CP)

  • adolescents and adults are more likely candidates

• side effects

  • drowsiness

  • dizziness

  • increased drooling

botulinum toxin A (botox) injections

• blocks nerve from firing by preventing release of acetylcholine at the motor nerve terminal and therefore weakening the muscle

• primarily used for lower extremity, but research suggests that it can also be effective in reducing spasticity in the upper extremity

• advantages

  • temporary, reversible; effects last 3-4 months

  • works best with hemis and diplegics with potential for ADL improvement and ambulation

  • less expensive than surgery; used at times to assess what effects surgery would have prior to actually doing a procedure

• disadvantages

  • temporary, requires re-injection

  • pain management protocols needed

  • potential formation of antibodies

  • there are limits to dosage that can be given to a specific muscle and in total

other

• biofeedback

• Functional Electrical Stimulation (FES)

peds CP:

classifications of CP based upon muscle tone

can be by motor type, distribution, or by type of lesion

• motor type:

  • ***spastic= tense, contracted muscles (most common type of CP)

    • increased muscle tone (w/ frequent low proximal muscle tone in the trunk)

    • stereotypic movement patterns and limited movement (lack of mobility)

    • atypical movement patterns

    • decreased active and passive range of motion

    • persistence of primitive and tonic reflexes, frequently obligatory

    • poor development of postural mechanisms

    • usually asymmetrical distribution of tone

    • oral motor/feeding problems- depending on severity

    • distribution

      • quadriplegia

      • diplegia

      • hemiplegia

  • ***athetoid= constant, uncontrolled motion of limbs, head, and eyes

    • fluctuating muscle tone with underlying low muscle tone

    • asymmetrical tonal distribution with risk for scoliosis

    • poor initiation and cessation of movement

    • difficulty co-contracting and stabilizing joints

    • poorly executed and coordinated purposeful movement

    • wide ranges of movement; poor control in mid-ranges

    • pathological reflexes usually present

    • feeding and speech problems common

    • all 4 limbs usually involved

    • involuntary movement of various types

    • types of athetoid cerebral palsy

      • pure athetoid

      • athetosis with spasticity- mixed type of cerebral palsy

      • athetosis with tonic spasms

      • choreoathetosis

  • ***ataxic= poor sense of balance, often causing falls and stumbles

    • disturbances of coordinated movement patterns

    • incoordination- difficulty sustaining co-activation of muscles for coordinated limb movement

    • limb ataxia, tremors

    • hypotonia most frequently; tone approximate normal

    • poor balance with poorly coordinated equilibrium reactions

    • slow, labored, dysarthric speech

    • slow initiation of movement

  • ***atonic (hypotonic)

    • joint hypermobility, instability, and poor co-activation

    • slow responses to sensation

    • atypical movement patterns with a predominance of extensor movement and compensatory pattern; may move quickly due to lack of stability

    • feeding and respiratory problems in young children

  • mixed

peds CP:

typical signs and symptoms

= a non-progressive disorder of movement and posture secondary to static encephalopathy occurring in the prenatal, perinatal, or post-natal period of development; expressed through variable impairments in the coordination of muscle action and sensation

• has an impact on sensory system, tone, posture, and coordinated movement

• ***spastic

  • increased muscle tone (w/ frequent low proximal muscle tone in the trunk)

  • stereotypic movement patterns and limited movement (lack of mobility)

  • atypical movement patterns

  • decreased active and passive range of motion

  • persistence of primitive and tonic reflexes, frequently obligatory

  • poor development of postural mechanisms

  • usually asymmetrical distribution of tone

  • oral motor/feeding problems- depending on severity

  • distribution

    • quadriplegia

    • diplegia

    • hemiplegia

• ***athetoid

  • fluctuating muscle tone with underlying low muscle tone

  • asymmetrical tonal distribution with risk for scoliosis

  • poor initiation and cessation of movement

  • difficulty co-contracting and stabilizing joints

  • poorly executed and coordinated purposeful movement

  • wide ranges of movement; poor control in mid-ranges

  • pathological reflexes usually present

  • feeding and speech problems common

  • all 4 limbs usually involved

  • involuntary movement of various types

  • types of athetoid cerebral palsy

    • pure athetoid

    • athetosis with spasticity- mixed type of cerebral palsy

    • athetosis with tonic spasms

    • choreoathetosis

• ***ataxic

  • disturbances of coordinated movement patterns

  • incoordination- difficulty sustaining co-activation of muscles for coordinated limb movement

  • limb ataxia, tremors

  • hypotonia most frequently; tone approximate normal

  • poor balance with poorly coordinated equilibrium reactions

  • slow, labored, dysarthric speech

  • slow initiation of movement

• ***atonic (hypotonic)

  • joint hypermobility, instability, and poor co-activation

  • slow responses to sensation

  • atypical movement patterns with a predominance of extensor movement and compensatory pattern; may move quickly due to lack of stability

  • feeding and respiratory problems in young children

associated deficits

• hearing problems common in rubella and hyperbilirubinemia, kernicterus, post-meningitis

• 50% have vision problems, including squints, strabismus, lack of conjugate eye movements, hemianopsia; may see fixed ocular patterns

• 25% have oral-motor and speech problems

  • dysarthria (motor speech disorder caused by weakness or incoordination of the muscles involved in speech production, leading to difficulty with articulation, voice, and prosody) & oral dyspraxia (difficulty in planning and coordinating voluntary movements of the tongue, soft palate, or lips), most common in athetoids with severe articulation problems

• feeding and respiratory problems

• 25-50% of cases (hemis and quads) have seizure disorder

• 50-75% of cases have below average intelligence + cognitive deficits

• range of learning difficulties- children with hemiplegia are most likely to have learning disabilities

• orthopedic deformities

  • scoliosis and kyphosis

  • asymmetries

  • hip dislocation

  • contractures and foot deformities

• sensory processing

  • tactile, vestibular, and proprioceptive issues

  • decreased sensory awareness, particularly in hemiplegia with unilateral neglect

• social/emotional

  • emotional liability

• adjustment to disability issues

• limited participation

• medical

  • reflux, increased upper respiratory problems, nutritional issues, particularly if feeding problems

• educational delays

• developmental delays

peds CP:

predisposing conditions

risk factors

• preterm, low birth weight

• intrauterine growth restriction and infection

• hypoxic ischemia (brain injury that occurs when the brain doesn't get enough oxygen or blood flow), cerebrovascular insults, or brain injury during pregnancy and in early infancy

• genetic mutations

peds CP:

progression of disease

functional classification system

• GMFCS

  • Level I

    • children walk at home, school, outdoors, and in the community

    • they can climb stairs without the use of a railing

    • children perform gross motor skills, such as running and jumping, but speed, balance, and coordination are limited

  • Level II

    • children walk in most settings and climb stairs holding onto a railing

    • they may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces

    • children may walk with physical assistance, a hand-held mobility device, or used wheeled mobility over long distances

    • children only have minimal ability to perform gross motor skills, such as running and jumping

  • Level III

    • children walk using a hand-held mobility device in most indoor settings

    • they may climb stair holding onto a railing with supervision or assistance

    • children use wheeled mobility when traveling long distances and may self-propel for shorter distances

  • Level IV

    • children use methods of mobility that require physical assistance or powered mobility in most settings

    • they may walk for short distances at home with physical assistance or use powered mobility or a body support walker when positioned

    • at school, outdoors, and in the community, children are transported in a manual wheelchair or use powered mobility

  • Level V

    • children are transported in a manual wheelchair in all settings

    • children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements

• MACS classification system

  • Level I

    • handles objects easily and successfully

  • Level II

    • handles objects, but with somewhat reduced quality and/or speed of achievement

  • Level III

    • handles objects with difficult; needs help to prepare and/or modify activities

  • Level IV

    • handles a limited selection of easily managed objects in adapted situations

  • Level V

    • does not handle objects and has severely limited ability to perform even simple actions

peds CP:

diagnostic tests and therapeautic interventions

diagnosis

• ultrasound

• CAT scan

• MRI

• cortical and brainstem evoked potentials

• EEG

surgical and medical interventions

• hip surgery for dislocation, soft tissue releases

• de-rotation osteotomies (femoral, tibial)

• hamstring releases to improve ambulation (act of walking or moving about) and posture in sitting

• heel cord lengthening

• ankle fusions for valgus deformities

• scoliosis surgery

• upper extremity/hand surgery- not very common

• selective dorsal rhizotomy

therapeutic interventions (PT/OT/ST)

• goal-directed therapies

• constraint induced therapy

• bilateral intensive programs

• sensory processing

• functional/compensatory approach

• myofascial release

• yoga

• kinesio-taping

• conductive education

• feldenkrais

• massage

peds CP:

OT goals/focus for specific conditions

OTPF-4

• occupations

  • play

• contexts

  • environmental factors

  • personal factors

• performance patterns

  • habits

  • routines

  • roles

  • rituals

• performance skills

  • motor skills

  • process skills

  • social interaction skills

• client factors

  • values, beliefs, and spirituality

  • body functions

  • body structures

cp quiz:

Cerebral Palsy is defined as:

• a non-progressive disorder of movement and posture secondary to static encephalopathy occurring in the prenatal, perinatal or post-natal period of development

• a progressive disorder of movement and posture secondary to static encephalopathy occurring in the prenatal, perinatal or post-natal period of development

• a condition created by a viral infection leading to developmental delays

• a complication from the birthing process leading to damage to the central nervous system

a non-progressive disorder of movement and posture secondary to static encephalopathy occurring in the prenatal, perinatal or post-natal period of development

cp quiz:

Classification of CP looks at:

• Functional Mobility

• Lesion and impact on tone

• Topographical

• All are correct

All are correct

cp quiz:

Dystonia a neurological movement disorder with involuntary muscle contractions resulting slow repetitive movements that are not voluntary.

• True

• False

True

cp quiz:

Cognitive and seizure disorders are not commonly associated with CP.

• True

• False

False

TBI:

criteria for classifications of TBI (mild, moderate, severe)

mild: goes unnoticed, overlooked (imaging looks normal), can be masked; you’re supposed to rest

• structural imaging

  • normal

• loss of consciousness (LOC)

  • 0-30 min

• alteration of consciousness/mental state

  • a moment up to 24 hrs

• post-traumatic amnesia (PTA)

  • 0-1 day

• Glasgow Coma Scale (GCS)

  • 13-15

moderate

• structural imaging

  • normal or abnormal

• loss of consciousness (LOC)

  • >30 min and <24 hrs

• alteration of consciousness/mental state

  • >24 hrs; severity based on the criteria

• post-traumatic amnesia (PTA)

  • >1 day and <7 days

• Glasgow Coma Scale (GCS)

  • 9-12

severe

• structural imaging

  • normal or abnormal

• loss of consciousness (LOC)

  • >24 hrs

• alteration of consciousness/mental state

  • >24 hrs; severity based on the criteria

• post-traumatic amnesia (PTA)

  • >7 days

• Glasgow Coma Scale (GCS)

  • <9

TBI:

Ranchos Scale (levels of cognitive functioning)

Level I

• response

  • no response

• assistance needed

  • needs total assistance

Level II

• response

  • generalized response

• assistance needed

  • needs total assistance

Level III

• response

  • localized response

• assistance needed

  • needs total assistance

Level IV

• response

  • confused-agitated response

• assistance needed

  • needs maximal assistance

Level V

• response

  • confused-inappropriate response

• assistance needed

  • needs maximal assistance

Level VI

• response

  • confused-appropriate response

• assistance needed

  • needs moderate assistance

Level VII

• response

  • automatic-appropriate response

• assistance needed

  • needs minimal assistance

Level VIII

• response

  • purposeful-appropriate response

• assistance needed

  • needs stand-by assistance

TBI:

diffuse axonal injury (pathophysiology, prognosis)

a type of injury to the brain

• diffuse stretching, tearing, and shearing of axons (nerve fibers)

• damage is microscopic and NOT initially visible on traditional CT or MRI

• such white mater damage can be seen on more modern neuroimaging techniques (DTI and others)

• common cause of disability following a TBI

• usually associated with high-velocity injuries

TBI:

Glasgow Coma Scale (GCS)

• criteria for eye opening, verbal and motor responses

== measures consciousness level; normal response is the highest number

• reliability and validity improve with formal training in performing and scoring scale

• should be performed as quickly as possible after the onset of TBI

• observations initially should be repeated frequently to establish if the patient is stable, to detect any trends of improvement, or to detect any deterioration from developing complications

• when a stable pattern emerges as time passes, the frequency can be reduced

eye opening

• 4 pts: spontaneous- open with blinking at baseline

• 3 pts: opens to verbal command, speech, or shout

• 2 pts: opens to pain, not applied to face

• 1 pt: none

verbal responses

• 5 pts: oriented

• 4 pts: confused conversation, but able to answer questions

• 3 pts: inappropriate responses, words discernible

• 2 pts: incomprehensible speech

• 1 pt: none

motor responses

• 6 pts: obeys commands for movement

• 5 pts: purposeful movement to painful stimulus

• 4 pts: withdraws from pain

• 3 pts: abnormal (spastic) flexion, decorticate posture

• 2 pts: extensor (rigid) response, decerebrate posture

• 1 pt: none

TBI:

Heterotopic Ossification (HO)

• epidemiology, signs/symptoms, impacts on ROM and function

epidemiology

• clinically significant HO in 10-20% of mod/severe TBI

• risk factors: LOC >2 weeks, fractures, spasticity, edema

  • spasticity: an upper motor neuron sign due to injury to CNS, resulting in loss of control over both upper and lower motor neuron synergy

    • creates an instability of the muscle spindles and golgi tendons to regulate muscle tension accurately, thus a hypertonic condition of spasticity occurs

    • velocity-dependent increased resistance to passive stretch

    • other upper motor neuron signs include:

      • clonus

      • pronator drift

      • co-contraction

      • flexor and extensor spasms

      • dystonia

      • associated reactions

      • muscle stiffness

      • joint contractures

sign/symptoms

• rapid loss of ROM

• pain

• redness/warmth to the joint

TBI:

typical signs and symptoms

common outcomes: cognitive, behavioral, and physical impairments

• cognitive impairments

  • memory, attention, executive function

• behavioral impairments

  • irritability, mood swings, depression

• physical impairments

  • spasticity, visual issues, headaches

severe TBI

• GCS 3-8

  • comatose, unable to follow simple commands

  • often associated with other traumatic injuries

moderate TBI

• GCS 9-12

  • typically able to follow simple commands but confused and emotional episodes

  • may have focal neurological deficits

  • epidemiology

  • 10% of TBI patients seen in the ER

mild TBI

• GCS 13-15

  • ± brief LOC

  • less than 24 hours of post-traumatic amnesia

  • no focal neurologic signs

  • epidemiology

• concussion: complex pathophysiological process affecting the brain, induced by biomechanical forces and includes several common features:

  • 1. concussion may be caused either by a direct hit to the head, face, neck, or anywhere else on body with an “impulsive” force transmitted to the head

  • 2. typically results in rapid onset of short-lived impairment of neurological function that resolves spontaneously, in some cases, signs and symptoms may evolve over minutes to hours, even days in adolescents/kids

  • 3. functional damage to the brain, not a structural damage; head CT or MRI is normal

  • most common type of TBI

  • form of a mild TBI

  • under-reported

  • headache (most common)

  • dizziness

  • tinnitus

  • hearing loss

  • blurred vision

  • altered taste, smell

  • sleep disturbance

  • insomnia

  • fatigue

  • sensory impairments

  • attention, concentration deficits

  • slowed mental processing

  • memory impairment (mostly short-term memory)

  • emotional lability

  • depression

  • irritability

  • anxiety

visual impairments

• accommodative dysfunction

• vergence dysfunction

  • convergence

  • exophoria (when covering one of your eyes makes it drift outward (away from your nose) and out of alignment)

• photosensitivity

• visual/vestibular

common medical conditions

• spasticity

• neuroendocrine dysfunction

• hydrocephalus

• post-traumatic seizures

• autonomic nervous system dysfunction

• disorders of consciousness (loss of consciousness occurs <10%)

  • coma

    • no sleep-wake cycle on EEG

    • eyes remain closed

    • no spontaneous movement or localization to noxious stimuli

    • no evidence of language comprehension or expression

    • damage to reticular activating system (RAS)

  • vegetative state

    • some sleep-wake cycle on EEG

    • no awareness of self or environment

    • no purposeful behavior

    • opens eyes (spontaneously or to noxious stimuli)

    • verbal or auditory startle, but no localization or tracking

    • diffuse cortical injury, bilateral thalamic lesions

  • minimally conscious state

    • minimal but definite evidence of self or environmental awareness

    • inconsistent but reproducible purposeful behaviors (command following, object manipulation, intelligible verbalization, gestural or verbal yes/no response)

    • ± visual fixation, tracking, emotional or motor behaviors triggered by specific and reproducible stimulus

• DVT and HO

  • HO

    • epidemiology

      • clinically significant HO in 10-20% of mod/severe TBI

      • risk factors: LOC >2 weeks, fractures, spasticity, edema

        • spasticity: an upper motor neuron sign due to injury to CNS, resulting in loss of control over both upper and lower motor neuron synergy

          • creates an instability of the muscle spindles and golgi tendons to regulate muscle tension accurately, thus a hypertonic condition of spasticity occurs

          • velocity-dependent increased resistance to passive stretch

          • other upper motor neuron signs include:

            • clonus

            • pronator drift

            • co-contraction

            • flexor and extensor spasms

            • dystonia

            • associated reactions

            • muscle stiffness

            • joint contractures

      sign/symptoms

      • rapid loss of ROM

      • pain

      • redness/warmth to the joint

neuro-psychological dysfunction

• agitation

• cognitive impairments including executive function

• depression/anxiety

• behavioral changes (mood, inhibition, etc.)

common rehabilitation impairments

• motor dysfunction (paresis, coordination, ataxia, dysmetria, etc.)

• expressive receptive aphasia

• motor and verbal apraxia

• visual field deficits

• cranial nerve palsies

• monocular/binocular vision deficits

• visual/body neglect

• COGNITIVE DEFICITS

• behavioral issues

frontal lobe syndrome: dysexecutive syndrome

== damage of higher functioning processes of the brain, such as motivation, planning, social behavior, and language/speech production

• lesions to the dorsolateral prefrontal cortex

• concrete thinking

• poor insight and judgment

• memory problems

• temporal problems

• can have a blunted/apathetic affect

• emotional lability and outbursts

second-impact concussion syndrome

• may occur when a person has a second concussion (of any grade) before symptoms of the first has subsided

• rare but catastrophic

• brain swells rapidly

• may be treated if symptoms are recognized very quickly

• morbidity around 100%, mortality around 50%

• young people seem more likely to experience this syndrome

cranial nerve palsies

• common

  • I (olfactory)

  • VII (facial)

  • VIII (vestibulocochlear)

• intermediate

  • II (optic)

  • III (oculomotor)

  • IV (trochlear)

  • VI (abducens)

• affected eye movements and visual skills

  • oculomotor/versional

    • fixation

    • pursuits

      • slower pursuits

    • saccades

      • slower saccades

      • poor timing and rhythm

      • correlated with post-concussive symptom load and perceived health status/QOL

    • nystagmus

TBI:

predisposing conditions

demographics

• etiology: caused by an external traumatic force

  • MVAs

  • falls

  • violence

  • suicide

  • sports

  • blasts

  • gun shot wounds

• people age 75 yrs and older had the highest numbers and rates of TBI-related hospitalizations (usually from falls)

• males were nearly 2x more likely to be hospitalized and 3x more likely to die from a TBI than females

  • especially in white and American Indigeonous males

• highest rate of injury occurs between the ages of 15-24 years

• persons under the age of 5 or over 75 are also at higher risk

types of injuries to brain

• skull fractures

• focal brain injury

  • subdural hematoma

    • occurs when bridging veins between brain and dura are ruptured and blood escapes into the subdural space

    • typically occurs with rapid acceleration

      • falls in the elderly

      • shaken baby in infants

    • symptoms depend on rate of bleeding, location, and amount of associated injury

• diffuse brain injury

  • cerebral contusion diffuse brain injury

    • brain “bruise”

    • may occur at site of impact (coup) or opposite (contracoup)

    • most commonly involves frontal (coup), temporal, and occipital (countracoup) lobes

    • leads to cognitive and behavioral deficits

  • diffuse axonal injury (DAI)

    • diffuse stretching, tearing, and shearing of axons (nerve fibers)

    • damage is microscopic and NOT initially visible on traditional CT or MRI

    • such white matter damage can be seen on more modern neuroimaging techniques (DTI and others)

    • common cause of disability after a TBI

    • usually associated with high-velocity injuries

• penetrating brain injury

TBI:

progression of disease

what factors influence prolonged recovery

• prior concussion

  • how long it takes to recover from a concussion

    • most people will recover spontaneously

    • 80-90% will resolve in 7-10 days

    • the other 10-20% —> post-concussion syndrome

      = a condition on which symptoms remain after a 10-day period of time

• female vs male

• age

• history of:

  • ADHD

  • headaches

  • prior concussion

  • other neurological condition

• psychiatric co-morbidity

• prolonged loss of consciousness

TBI:

diagnostic tests and therapeutic interventions

severe TBI

• medical management

  • cardiopulmonary resuscitation

  • CT scan

  • neurosurgical intervention often needed

  • intracranial pressure monitoring (if increased, may need drain, other management)

moderate TBI

• medical management

  • ensure cardiopulmonary stability before neurologic exam

  • 40% will have an abnormal CT scan

  • admission for observation (even if CT is normal)

  • may need neurosurgical evaluation

mild TBI

• medical management

  • usually observational, self-limited

  • imaging typically normal

  • consider CT scan (especially if LOC, significant amnesia, headache)

  • *concussion a subset of the mildest form of TBI

neuropsychological testing

• may not find unequivocal results

• most with mild TBI won’t show memory deficits

• lack of baseline

• helpful in more significant injuries

treatment

• first line of treatment- therapy:

  • positioning, strengthening antagonist, bracing, casting, taping, e-stim

• intrathecal baclofen

  • baclofen is stored in a pump reservoir, implanted subcutaneously, and dosed intrathecally continuously

  • cather placement tip can be lumbar to cervical

TBI:

OT goals/focus for specific conditions

reduction of muscle over-activity without affecting voluntary motor control

• improve

  • function

  • sitting

  • ambulation

  • positioning

  • cleanliness

• facilitate fitting of an orthotic device

common visual complaints

• “I get tired when trying to read”

• “When I turn too quickly I get dizzy”

• “I feel like it is difficult to find things”

• “I am seeing double”

• “My vision is blurry”

• “My depth is off”

• “I am skipping lines when reading”

common functional complaints

• “I can’t read for more than 5 minutes at a time”

• “The light from the computer and my phone bothers me”

• “I love to cook and have a hard time following the recipe, I skip steps”

• “I can’t tweeze my eyebrows”

• “When I go to tie my shoes I get really dizzy and feel disoriented”

• “I’m having a hard time putting my make-up on”

• “I have a hard time paying attention in class”

SCI:

levels of SC and related movements and function

• C1-C8

• T1-T12

• L1-L5

• S1-S5

+ potential functional highlights

C1-C8

• C1-C4: diaphragm

  • often vent-dependent (phrenic nerve)

• C5: elbow flexion

  • independent with feeding and grooming (with adaptive equipment)

  • mobility in power wheelchair

• C6: wrist extension

  • tenodesis grasp

  • if highly motivated, can live independently

• C7: elbow extension

  • can usually live independently

• C8 and T1: finger flexion and abduction

  • independent with all ADLs (e.g., bowel and bladder)

T1-T12

• trunk muscles

L1-L5

• L1:

• L2: hip flexion

  • ambulation

• L3: hip extension

  • ambulation

• L4: ankle dorsiflexion

• L5: toe extension

S1-S5

• S1: ankle plantarflexion

• S2: bowel and bladder function

• S3: bowel and bladder function

• S4: bowel and bladder function

• S5: bowel and bladder function

SCI:

American Spinal Injury Association (ASIA) Classification

• complete vs incomplete

== determines functional levels of injury

• motor, sensory, and neurological levels

  • motor level: level of the lowest innervated muscle having grade 3 or better strength where all muscles above are graded 5

  • sensory level: lowest dermatomal level with normal sensation to pinprick and light touch with all dermatomes above being normal

  • neurological level: highest of either the sensory or motor levels

== determines grade (A-E)

• A: complete. no sensory or motor function at S4/5

• B: sensory incomplete. sensory, but no motor function at S4/5

• C: motor incomplete. more than half of key muscles below the single neurological level have a grade less than 3

• D: motor incomplete. half or more of key muscles below the single neurological level have a grade greater than or equal to 3

• E: normal. all components of International Standards Exam are normal

incomplete vs complete

• incomplete spinal cord syndromes:

  • central cord

  • Brown-Sequard

  • anterior cord

why we do it

• functional

• common language

• well tolerated

• good inter-rater reliability

• prognosticating tool

  • predict function

  • predict neural recovery

motor testing

• requires examination of 10 key muscles on each side

  • 5 upper limb

  • 5 lower limb

• each key muscle represents a single myotime

  • upper limb key muscles represent the C5-T1

  • lower limb key muscles represent the L2-S1

motor grading

• each key muscle is scored 0-5

• 0: no visible or palpable contraction

• 1: any visible or palpable contraction

• 2: full ROM w/ gravity eliminated

• 3: full ROM against gravity, without resistance

• 4: full ROM against resistance, but not normal

• 5: normal strength

• 5*: normal strength, if identified inhibiting factor not present (e.g., pain or disuse)

• NT: not testable (e.g., immobilization, severe pain, amputation)

sensory grading

• examine 28 dermatomes on each side (C2 —> S4/5)

• 1 key sensory point represents each dermatome

• light touch (LT) and pinprick (PP) are tested

• each dermatome is scored either 0, 1, or 2

  • 0 —> absent sensation

  • 1 —> impaired sensation

  • 2 —> normal sensation

SCI:

orthostatic HTN

== transient reflex depression in BP triggered by tilting the patient upright

—> mechanism

• —> upright position causes decrease in BP

• —> aortic and carotid baroreceptors sense decrease in BP

• —> usually increases sympathetic outflow; however, efferent pathway interrupted by SCI

• —> parasympathetic still inhibited, resulting in tachycardia

—> improves with time due to development of spinal postural reflexes, with improved auto-regulation of cerebrovascular circulation in the presence of low perfusion pressure

symptoms:

• lightheaded

• dizziness

• nausea

• pallor

signs:

• hypotension

• tachycardia

• syncope

management/treatment

• reposition —> Trendelenburg position

  • tilt-in-space or reclining wheelchair

  • tilt table

• abdominal binder, elastic stockings, ace wrap

• fluid resuscitation if hypovolemic

• medications:

  • salt tablets

  • midodrine (stimulates vasoconstriction)

  • florinef (mineralocorticoid)

  • use caution! once orthostasis improves, the patient may be at risk for hypertension

SCI:

autonomic dysreflexia

• causes

• signs

• emergency response

• massive balance in reflex sympathetic discharge

• typically occurs in patients with complete SCI >- T6 level

• affects 48-90% of susceptible patients

• degree of symptoms vary significantly

• timing

  • onset after spinal shock

  • may appear within 2-4 weeks post-injury

  • if occurs, will present within 1st year in >90% of cases

• mechanism:

  • noxious stimuli causes:

    —> increase in sympathetic tone

    —> causing local vasoconstriction and a marked rise in BP

  • the body tries to respond by:

    —> stimulating baroreceptors to slow heart; however, this is insufficient to combat the increase in BP

  • in summary:

    —> the brainstem is unable to send messages through the spinal cord to the affected area to decrease sympathetic outflow and allow vasodilation to decrease BP

• common causes

  • tight-fitting clothes

  • bladder (e.g., blocked catheter)

  • bowel (e.g., fecal impaction)

  • pressure ulcers

  • urinary tract infections

  • ingrown toenails

  • abdominal emergency (e.g., appendicites, cholecystis, pancreatitis, gastric ulcers)

  • labor

  • fractures

  • orgasm

  • epidydmitis

  • bladder stones

• signs and symptoms

  • elevated blood pressure + LOW heart rate

  • headache

  • sweating, piloerection, and facial flushing

  • sinus congestion

  • pupillary constriction —> blurry vision

  • can range from mild to severe symptoms

• management of AD

  • identify and remove noxious stimulus:

    • sit patient upright and loosen all tight-fitting clothing and devices (e.g., elastic band from urine leg bag, TEDS, abdominal binder)

    • bladder evaluation- flush indwelling catheter if present; catheterize patient if catheter is not already present

    • bowel evaluation

  • monitor BP every 2-5 minutes during episode

  • monitor for recurrent symptoms for at least 2 hours after resolution to ensure that it does not recur

• if hypertension is untreated, can lead to:

  • CVA

  • seizure

  • retinal hemorrhage

  • MI

  • death

  • can predispose patients to atrial fibrillation by altering normal pattern of repolarization of the atria

SCI:

types of SCI

• central cord

• Brown-Sequard

• anterior cord

• conus medullaris

• cauda equina

central cord- incomplete

• occurs with:

  • hyperextension injury

  • compression of the cord

  • low velocity injuries

  • fractures and dislocations

• focus of injury:

  • central grey matter of cord

• results in:

  • at injury: LMN weakness

  • below injury: UMN spasticity

  • lower limb functions less severely impacted

Brown-Sequard- incomplete

• occurs with:

  • hemisection of the cord

• focus of injury:

  • lateral half of the cord

• results in:

  • same side deficits:

    • motor control

    • proprioception

    • vibratory sensation

  • opposite side deficits:

    • pain sensation

    • temperature sensation

anterior cord- incomplete

• occurs with:

  • vascular injury or occlusion

  • burst injury

• focus of injury:

  • anterior 2/3 of cord

• results in:

  • loss of motor function

  • loss of pain and temperature sensation

  • preservation of light touch and joint position sense

conus medullaris

cauda equina

• occurs with:

  • central disc herniation

  • lumbar burst fractures

• focus of injury:

  • individual nerve roots below the spinal cord itself

• results in:

  • LMN flaccid paralysis

  • partial or complete loss of sensation

SCI:

tenodesis (hand function in cervical-level SCI)

SCI:

typical signs and symptoms

complication and concerns

• autonomic dysreflexia

  • massive balance in reflex sympathetic discharge

  • typically occurs in patients with complete SCI >- T6 level

  • affects 48-90% of susceptible patients

  • degree of symptoms vary significantly

  • timing

    • onset after spinal shock

    • may appear within 2-4 weeks post-injury

    • if occurs, will present within 1st year in >90% of cases

  • mechanism:

    • noxious stimuli causes:

      —> increase in sympathetic tone

      —> causing local vasoconstriction and a marked rise in BP

    • the body tries to respond by:

      —> stimulating baroreceptors to slow heart; however, this is insufficient to combat the increase in BP

    • in summary:

      —> the brainstem is unable to send messages through the spinal cord to the affected area to decrease sympathetic outflow and allow vasodilation to decrease BP

  • common causes

    • tight-fitting clothes

    • bladder (e.g., blocked catheter)

    • bowel (e.g., fecal impaction)

    • pressure ulcers

    • urinary tract infections

    • ingrown toenails

    • abdominal emergency (e.g., appendicites, cholecystis, pancreatitis, gastric ulcers)

    • labor

    • fractures

    • orgasm

    • epidydmitis

    • bladder stones

  • signs and symptoms

    • elevated blood pressure + LOW heart rate

    • headache

    • sweating, piloerection, and facial flushing

    • sinus congestion

    • pupillary constriction —> blurry vision

    • can range from mild to severe symptoms

  • management of AD

    • identify and remove noxious stimulus:

      • sit patient upright and loosen all tight-fitting clothing and devices (e.g., elastic band from urine leg bag, TEDS, abdominal binder)

      • bladder evaluation- flush indwelling catheter if present; catheterize patient if catheter is not already present

      • bowel evaluation

    • monitor BP every 2-5 minutes during episode

    • monitor for recurrent symptoms for at least 2 hours after resolution to ensure that it does not recur

  • if hypertension is untreated, can lead to:

    • CVA

    • seizure

    • retinal hemorrhage

    • MI

    • death

    • can predispose patients to atrial fibrillation by altering normal pattern of repolarization of the atria

• orthostatic hypotension

  • == transient reflex depression in BP triggered by tilting the patient upright

    —> mechanism

    • —> upright position causes decrease in BP

    • —> aortic and carotid baroreceptors sense decrease in BP

    • —> usually increases sympathetic outflow; however, efferent pathway interrupted by SCI

    • —> parasympathetic still inhibited, resulting in tachycardia

    —> improves with time due to development of spinal postural reflexes, with improved auto-regulation of cerebrovascular circulation in the presence of low perfusion pressure

    symptoms:

    • lightheaded

    • dizziness

    • nausea

    • pallor

    signs:

    • hypotension

    • tachycardia

    • syncope

    management/treatment

    • reposition —> Trendelenburg position

      • tilt-in-space or reclining wheelchair

      • tilt table

    • abdominal binder, elastic stockings, ace wrap

    • fluid resuscitation if hypovolemic

    • medications:

      • salt tablets

      • midodrine (stimulates vasoconstriction)

      • florinef (mineralocorticoid)

      • use caution! once orthostasis improves, the patient may be at risk for hypertension

• bladder and bowel dysfunction

  • sympathetic

    • T11-L2

    • hypogastric nerve

    • urinary storage

  • parasympathetics

    • S2-S4

    • pelvic nerve

    • control of micturition

  • somatics

    • S2-S4

    • pudendal nerve

    • control of external urethral sphincter

• higher rates of infection (UTI, GI, skin)

• pressure ulcers

• sexual dysfunction and male infertility

• increase risk of GI pathology

• increase risk of pulmonary pathology

  • resp failure, sleep apnea

• hypercalciuria, hypercalcemia, hyperglycemia

• osteoporosis —> fractures

• heterotopic ossification

  • == abnormal growth of bone in the non-skeletal tissues, including muscle, tendons, or other soft tissue; when HO develops, new bones grow at 3x the normal rate, resulting in jagged, painful joints

  • approx. 20% of SCI will result in some level of HO

  • most common joints affected are hips and elbows

• deep vein thrombosis (DVT)/pulmonary embolism

• PAIN

injury impacts to autonomic nervous system

• cardiovascular control:

  • neurogenic shock

  • orthostatic hypotension

  • autonomic dysreflexia

  • cardiac dysrhythmia

• sweating abnormalities:

  • hyperhidrosis

  • hypohidrosis

  • reflex sweating below the neurological level

• bowel dysfunction:

  • loss of bowel sensation

  • bowel incontinence

  • constipation

• bladder dysfunction:

  • detrusor sphincter hypoactivity

  • detrusor sphincter hyperactivity

  • detrusor sphincter dyssynergia

• temperature control:

  • cold or heat intolerance

  • poikliothermia

  • hypo or hyperthermia

• sexual function:

  • erectile function

  • ejactulation

  • vaginal lubrication

psycho-social impact

• stage theory

  • 1. shock and denial

  • 2. depression

  • 3. anxiety

  • 4. anger

  • 5. bargaining

  • 6. adaptation

• depression

• suicide

• drug/substance abuse

motor coordination and disorders

• upper vs lower motor neuron signs

• basal ganglia: tremors, rigidity, chorea

• cerebellum: ataxia, dysmetria, intention tremor

  • under (hypometria) and over (hypermetria) shooting of a target (dysmetria) and the decomposition of movement (the breakdown of the movement into its parts with impaired timing and integration of muscle activity) are seen with appendicular ataxia

• screen tests: finger-nose, finger-finger, rapid alternating movements

SCI:

predisposing conditions

gender

• more than 4:1 male to female ratio

age

• average age of injury increased from 29 to 43

race (from most to least)

• non-hispanic white

• non-hispanic black

• hispanic origin

• asian

• other

• native american

etiology (from most to least)

• vehicular

• falls

• violence

• sports

• other

• medical/surgical

SCI:

progression of disease

in traumatic SCI, nerve injury is the slowest thing to heal

primary phase of SCI injury

• traction and compression forces (e.g., being in a car accident and receiving blunt and compression forces)

• fractured and displaced bone fragments and disc material cause direct compression on the spinal cord

• blood vessel axons are damaged

• micro-hemorrhages occur in the grey matter, resulting in rapid swelling

secondary phase of SCI injury

• inflammatory cells infiltrate the site of injury; these cells release inflammatory cytokines, resulting in more neuronal damage (6-12 hrs after injury and remain elevated up to 4 days post injury)

• loss of ionic homeostasis results in cell death with mitochondrial dysfunction

• glutamate and aspartate are released from damaged cell,s causing further cellular death to adjunct neurons

SCI:

diagnostic tests and therapeutic interventions

ASIA exam used to determine level and severity

motor system screening

• muscle tone:

  • normal to spastic (spasticity is velocity and direction dependent)/rigid (rigidity is not velocity or direction dependent)

• spasticity assessment:

  • quick stretch, modified Ashworth scale

    • modified Ashworth scale

      • 0: no increase in muscle tone

      • 1: slight increase in muscle tone, manifested by a catch and release or by minimal resistance at the end of the range of motion when the affected part(s) is moved in flexion or extension

      • 1+: slight increase in muscle tone, manifested by a catch, followed by minimal resistance throughout the remainder (less than half) of the ROM

      • 2: more marked increase in muscle tone through most of the ROM, but affected part(s) easily moved

      • 3: considerable increase in muscle tone, passive movement difficult

      • 4: affected part(s) rigid in flexion or extension

• reflex testing:

  • biceps, triceps, knee, ankle, Babinski

medical interventions

• ABCs

• immobilization

• early surgical decompression

  • evidence indicates it can reduce the impact secondary phase of injury

• steroids

  • evidence suggests it may reduce some impact of secondary phase of injury

research

• stem cells

• biomaterials

• pharmacological therapies

• cell therapies

SCI:

OT goals/focus for specific conditions

consider the importance of psycho-social impact of SCI both short term and life long adjustments

TBI/SCI quiz:

After a TBI or SCI, which medical sequelae may occur that is associated with joint immobility, and redness and swelling?

• Heterotopic ossification (HO)

• Venous thromboembolic disease (DVT/PE)

• Endocrine dysfunction

• Autonomic Dysreflexia

Heterotopic ossification (HO)

TBI/SCI quiz:

Before you see your client, you read in their chart that they have a GCS score of 11. When you see them, they are awake and able to follow simple commands but appear confused. They most probably has experienced a _______TBI.

• Moderate

• Severe

• Mild

• Concussion

Moderate

TBI/SCI quiz:

You see a male in his mid-20s with a history of concussions who is complaining of difficulty focusing on things visually, resulting in sometimes seeing things as blurry or double vision. What is the most likely reason for this?

• Cortically, the eyes are not coordinating properly together to see clear binocularly

• Cranial nerve 9 and 10 palsy

• He is near sightedness so he is having difficulty seeing items far away.

• May be suffering from far sightedness

Cortically, the eyes are not coordinating properly together to see clear binocularly

TBI/SCI quiz:

Glascow Coma Scale (GCS) 13-15, brief loss of consciousness and no focal neurological signs. You would classify a person as a _____ TBI.

• Mild

• Moderate

• Severe

• Vegative State

Mild

TBI/SCI quiz:

What statement best describes TBI Coup vs Contrecoup Cerebral Contusion Injury

• Frontal impact resulting in both frontal and occipatal lobe damageoccipital

• Frontal impact resulting in frontal lobe damage

• Side impact resulting in frontal and occipital lobe damage

• Posterior impact resulting in temporal lobe damage

Frontal impact resulting in both frontal and occipatal lobe damage

TBI/SCI quiz:

Anterior cord syndrome commonly results from direct contusion to the anterior cord by bone fragments or from damage to the anterior spinal artery. 

• True

• False

True

TBI/SCI quiz:

This syndrome is caused by a complete hemisection of the spinal cord, resulting in a greater ipsilateral proprioceptive motor loss and a greater contralateral loss of pain and temperature sensation. 

• Brown-Sequard Syndrome

• Central Cord Syndrome

• Mixed Syndrome

• Anterior Cord Syndrome

Brown-Sequard Syndrome

TBI/SCI quiz:

Mr. Urban is a 26 year-old male with a C6 complete spinal cord injury. Which muscles are impacted?

• wrist extensors

• deltoid

• biceps

• diaphragm

wrist extensors

neuromuscular/movement disorders:

parkinson’s disease (pathophysiology, cardinal signs, medical management)

***parkinson’s disease

• neurodegenerative disorder of the basal ganglia involving the dopaminergic (dopamine-secreting) nigrostriatal pathway

  • loss of dopaminergic neurons in the substantia nigra pars compact

  • presence of Lewy bodies that accumulate in the brain

• dopamine loss in brainstem, thalamus, and cortex

• results in motor and non-motor symptoms

• ***pathophysiology

  • abnormal clumps of alpha-synuclein (neuronal protein) in substantial nigra and other parts of the brain associated with neurodegeneration

  • degeneration of dopaminergic nigrostriatal pathway in basal ganglia

    • underactivity of the direct motor pathway (decreased facilitation of movement)

    • overactivity of the indirect motor loop (increased inhibition of movement)

  • results in inhibition of the motor cortex (bradykinesia and rigidity)

  • subthalamic nucleus overactivity

    • leads to motor deficits

    • influences the limbic system (emotional signs and symptoms)

• ***cardinal signs

  • resting tremor

    • involuntary, rhythmic oscillatory movement (“pill rolling” tremor)

    • increased by stress/anxiety

  • rigidity

    • increased resistance to passive movement of a joint

    • plastic rigidity: constant, “lead-pipe” resistance throughout range of motion

    • cogwheel rigidity: brief palpable jerks, accompanied by tremor

  • bradykinesia/akinesia

    • slowness or poverty of voluntary movement

    • micrographia

    • extreme under-activity

    • “wooden” sensation as if moving against resistance

    • rapid severe fatigue

    • freezing (inability to continue movement)

  • loss of postural reflexes

    • stooped posture (flexed, forward leaning)

    • inability to make postural adjustments to maintain balance

    • festinating gait: short, accelerating steps

  • depression

    • inherent part of disease rather than situational response

  • dementia

    • disorientation, memory loss, distractibility, executive dysfunction

  • fatigue, pain, autonomic dysfunction, poor sleep

• ***medical management

  • medications provide symptomatic relief only

    • converted to dopamine

    • mimics dopamine

    • prevent breakdown of dopamine

    • management of psychiatric symptoms

  • psychosocial support and education

  • rehabilitation

    • exercise is critical and may have a neuroprotective effect

    • benefit of external cues such as rhythm and music

    • amplitude-based movement training

  • deep brain stimulation (DBS)

    • surgery that helps control motor symptoms and can improve quality of life

      • electrodes implanted in the brain

      • reversible and can be adjusted based on symptoms

      • pulse generator

      • programming device adjusted signals and can turn the device on and off

    • “pacemaker for the brain”

      • electrical impulses disrupt abnormal activity in the brain

    • subthalamic nucleus is targeted to reduce output

neuromuscular/movement disorders:

ALS (upper/lower motor neuron involvement, progression)

== disorder occurs at the motor neuron cell body

• progressive neurodegenerative disease

  • progressive muscle weakness and respiratory failure

  • wheelchair bound by 12-18 months

  • death usually within 2-5 years from onset

  • 10% survive for 10 or more years

• amyotrophic (lower motor neuron)

  • progressive muscle wasting- “no muscle nourishment”

  • lower motor degeneration

    • loss of anterior horn cells and cranial nerve motor nuclei (CN V, VII, IX, X, XII)

    • degeneration of axons and denervation of motor units

  • lower motor neuron signs/clinical presentation

    • flaccid paresis, muscle atrophy, fasciculations, muscle cramping

    • flaccid parersis may mask spasticity (usually mild)

• lateral sclerosis (upper motor neuron)

  • scarring of lateral corticospinal tract in the lateral column oft the spinal cord

  • upper motor degeneration

    • demyelination with sclerosis (scarring) along the lateral and anterior corticospinal tracts and corticobulbar (AKA corticonuclear) tract

  • upper motor neuron signs/clinical presentation

    • increased muscle tone, spasticity, hyperactive DTRs, clonus (involuntary rhythmic muscle contractions)

• paresis usually begins in a single muscle group, with corresponding muscle groups asymmetrically affected

  • all striated muscles eventually become involved, except extraocular muscles and heart

  • oculomotor neurons typically spared

    • eye movement remains intact

    • allows for use of communication devices

  • impaired respiratory function

• initial signs/symptoms

  • difficulty walking with lower extremity weakness

  • hand weakness, clumsiness

  • slurred speech, difficulty swallowing

  • muscle cramps, twitching in upper extremities and tongue

  • difficulty maintaining posture

• cognitive and behavioral changes

  • impaired executive function in 30-50%

• painless, progressive, generalized weakness, asymmetric at onset

• progress to paralysis with no remissions

neuromuscular/movement disorders:

guillain-barre syndrome (acute inflammatory demyelinating polyradiculoneuropathy)

== disorder occurs at the axon

• autoimmune disease causing rapidly progressive polyneuropathy

  • immune system attacks Schwann cells (produce myelin sheath) of peripheral nerves

  • causes demyelination or axonal loss

  • results in acute flaccid neuromuscular paralysis

• muscle undergoes denervation and atrophy

  • if LMN cell body survives, peripheral nerve can regenerate

• subtypes

  • acute inflammatory demyelinating polyneuropathy (AIDP)

    • most common in US

  • acute motor (sensory) axonal neuropathy (AMAN, AMSAN)

    • less than 10% of cases

  • miller fisher syndrome: rare variant

    • involves oculomotor weakness, ataxia, areflexia

• clinical presentation

  • loss of sensation evolving over days

    • parethesias, dysthesias (tingling, burning, shock-like sensations), pain, numbness

  • symmetric weakness of extremities with progressive paralysis and decreased DTRs

  • cranial nerve weakness, facial weakness with difficulty chewing, swallowing, coughing

  • autonomic dysfunction

    • due to demyelination of the vagus nerve

    • cardiac arrhythmia (tachycardia or bradycardia), hypotension, hypertension, abnormal sweating, GI and bladder dysfunction

  • neuromuscular respiratory failure

    • causes shallow breathing with poor gas exchange

    • tachypnea with paradoxical breathing (chest out, abdomen in)

    • ventilator support for 10-30%

neuromuscular/movement disorders:

myasthenia gravis (autoimmune attack on neuromuscular junction)

== disorder occurs at the neuromuscular junction

• chronic autoimmune neuromuscular disease

  • “grave muscle weakness”

  • antibodies block acetylcholine receptors (AChR) at the neuromuscular junction (NMJ)

  • NMJ: synapse between motor neuron and muscle fiber

  • causes weakness of skeletal muscle

• subtypes: generalized, ocular, neonatal

• pathophysiology

  • formation of antibodies postsynaptic membrane

  • antibodies block ACh receptors and eventually destroy the receptor
    site, reducing the number of receptors

    • diminished transmission of nerve impulses across the NMJ

    • muscle depolarization is incomplete or not achieved

  • action potential in motor neuron does not result in muscle
    contraction

neuromuscular/movement disorders:

huntington’s disease (genetic basis, choreiform movements)

huntington’s disease

• genetic disorder that causes progressive degeneration of neurons in the brain

• characterized by involuntary movements, psychiatric symptoms, and progressive dementia

• HTT gene provides instructions for making protein called huntingtin

• mutation produces abnormally long proteins that is cut into toxic fragments and accumulates into neurons

• age of onset related to length of repeated sequences

  • increased length leads to earlier disease presentation

• causes basal ganglia degeneration in the brain

  • neuronal cell death in basal ganglia (especially caudate and putamen nuclei), cerebral cortex, and other areas of the brain

• excess dopaminergic activity leads to hyperkinesia

• loss of excitatory glutamate impairs modulation of movement

• clinical presentation

  • chorea

    • irregular involuntary “dancelike” movements

    • begins in face/arms, eventually affects entire body

  • frontal lobe dysfunction

    • attention deficits, slow thinking

    • short-term memory loss

    • decreased executive function, impulsivity

    • restlessness, irritability

  • affect

    • euphoria

    • depression, apathy

neuromuscular/movement disorders:

types of involuntary movements (tremors, chorea, athetosis, dystonia)

movement disorders

• hyperkinesia (excess movement)

  • chorea: brisk jerking random movements of extremities and face

  • athetosis: slow writhing movements of extremities, fluctuating grimaces

  • ballism: violent flailing movement of extremities and trunk

  • tremor: rhythmic oscillating movement

• hypokinesia (decreased movement)

  • akinesia: absence or poverty of voluntary muscle movement

  • bradykinesia: slowed, labored voluntary movement; difficulty initiating, continuing, or synchronizing movements

neuromuscular/movement disorders:

typical signs and symptoms

ASL

• progressive neurodegenerative disease

  • progressive muscle weakness and respiratory failure

  • wheelchair bound by 12 to 18 months

  • death usually within 2 to 5 years from onset

  • 10% survive for 10 or more years

• amyotrophic (lower motor neuron)

  • progressive muscle wasting- “no muscle nourishment”

  • lower motor degeneration

    • loss of anterior horn cells and cranial nerve motor nuclei (CN V, VII, IX, X, XII)

    • degeneration of axons and denervation of motor units

  • lower motor neuron signs/clinical presentation

    • flaccid paresis, muscle atrophy, fasciculations, muscle cramping

    • flaccid parersis may mask spasticity (usually mild)

• lateral sclerosis (upper motor neuron)

  • scarring of lateral corticospinal tract in the lateral column oft the spinal cord

  • upper motor degeneration

    • demyelination with sclerosis (scarring) along the lateral and anterior corticospinal tracts and corticobulbar (AKA corticonuclear) tract

  • upper motor neuron signs/clinical presentation

    • increased muscle tone, spasticity, hyperactive DTRs, clonus (involuntary rhythmic muscle contractions)

• paresis usually begins in a single muscle group, with corresponding muscle groups asymmetrically affected

  • all striated muscles eventually become involved, except extraocular muscles and heart

  • oculomotor neurons typically spared

    • eye movement remains intact

    • allows for use of communication devices

  • impaired respiratory function

• initial signs/symptoms

  • difficulty walking with lower extremity weakness

  • hand weakness, clumsiness

  • slurred speech, difficulty swallowing

  • muscle cramps, twitching in upper extremities and tongue

  • difficulty maintaining posture

• cognitive and behavioral changes

  • impaired executive function in 30-50%

• painless, progressive, generalized weakness, asymmetric at onset

• progress to paralysis with no remissions

• types

  • sporadic ALS (SALS): 90% of cases

    • environmental causes

    • possible contributors: mercury, manganese, pesticides, physical/dietary factors

    • increased incidence in Gulf War veterans

  • familial ALS (FALS): 10% of cases

    • characterized by mode of inheritance and chromosome location

    • can be autosomal dominant, autosomal recessive, or x-linked

GBS

• subtypes

  • acute inflammatory demyelinating polyneuropathy (AIDP)

    • most common in US

  • acute motor (sensory) axonal neuropathy (AMAN, AMSAN)

    • less than 10% of cases

  • miller fisher syndrome: rare variant

    • involves oculomotor weakness, ataxia, areflexia

• clinical presentation

  • loss of sensation evolving over days

    • parethesias, dysthesias (tingling, burning, shock-like sensations), pain, numbness

  • symmetric weakness of extremities with progressive paralysis and decreased DTRs

  • cranial nerve weakness, facial weakness with difficulty chewing, swallowing, coughing

  • autonomic dysfunction

    • due to demyelination of the vagus nerve

    • cardiac arrhythmia (tachycardia or bradycardia), hypotension, hypertension, abnormal sweating, GI and bladder dysfunction

  • neuromuscular respiratory failure

    • causes shallow breathing with poor gas exchange

    • tachypnea with paradoxical breathing (chest out, abdomen in)

    • ventilator support for 10-30%

MG

• clinical presentation

  • ocular symptoms

    • ptosis (drooping of upper eyelid)

    • weakness of eye movements

    • diplopia (double vision)

  • facial and bulbar weakness (dysphagia/ dysarthria)

    • affects muscles of face, mouth, throat, and neck

    • facial droop, expressionless face, difficulty chewing/swallowing, weight loss, drooling, choking/aspiration

  • painless fatigue and proximal weakness with exertion

  • worse with exercise, heat, and time of day (evening)

  • weakened respiratory muscles cause impaired ventilation

    • atelectasis – collapsed lung

    • congestion

  • myasthenia crisis

    • severe muscle weakness causes quadriparesis or quadriplegia, respiratory
      insufficiency, difficulty swallowing

    • can lead to respiratory arrest

movement disorders

• hyperkinesia (excess movement)

  • chorea: brisk jerking random movements of extremities and face

  • athetosis: slow writhing movements of extremities, fluctuating grimaces

  • ballism: violent flailing movement of extremities and trunk

  • tremor: rhythmic oscillating movement

• hypokinesia (decreased movement)

  • akinesia: absence or poverty of voluntary muscle movement

  • bradykinesia: slowed, labored voluntary movement; difficulty initiating, continuing, or synchronizing movements

huntington’s disease

• genetic disorder that causes progressive degeneration of neurons in the brain

• characterized by involuntary movements, psychiatric symptoms, and progressive dementia

• clinical presentation

  • chorea

    • irregular involuntary “dancelike” movements

    • begins in face/arms, eventually affects entire body

  • frontal lobe dysfunction

    • attention deficits, slow thinking

    • short-term memory loss

    • decreased executive function, impulsivity

    • restlessness, irritability

  • affect

    • euphoria

    • depression, apathy

parkinson’s disease

• ***cardinal signs

  • resting tremor

    • involuntary, rhythmic oscillatory movement (“pill rolling” tremor)

    • increased by stress/anxiety

  • rigidity

    • increased resistance to passive movement of a joint

    • plastic rigidity: constant, “lead-pipe” resistance throughout range of motion

    • cogwheel rigidity: brief palpable jerks, accompanied by tremor

  • bradykinesia/akinesia

    • slowness or poverty of voluntary movement

    • micrographia

    • extreme under-activity

    • “wooden” sensation as if moving against resistance

    • rapid severe fatigue

    • freezing (inability to continue movement)

  • loss of postural reflexes

    • stooped posture (flexed, forward leaning)

    • inability to make postural adjustments to maintain balance

    • festinating gait: short, accelerating steps

  • depression

    • inherent part of disease rather than situational response

  • dementia

    • disorientation, memory loss, distractibility, executive dysfunction

  • fatigue, pain, autonomic dysfunction, poor sleep

neuromuscular/movement disorders:

predisposing conditions

ALS

• etiology

  • unknown, thought to be multifactorial

  • potentially: oxidative stress, glutamate excitotoxicity, mitochondrial dysfunction, apoptosis, inflammation

• incidence

  • 1-3 per 100,000 new cases of ALS each year

GBS

• etiology

  • unknown, typically preceded by viral or bacterial infection

• incidence

  • disorder of young adulthood and early middle age

  • affects 1-2 in 100,000; male > female

myasthenia gravis

• etiology

  • known/spontaneous

  • associated with the thymus gland

    • role in disease not fully understood

  • may have genetic susceptibility to variants in AChR genes

• incidence

  • 14 to 20 per 100,000

  • 36K to 60K in the US

  • under diagnosed, prevalence probably higher

  • occurs at any age, including children

huntington’s disease

• etiology

  • autosomal dominant disorder

  • 50% change of inhering from parent

• incidence

  • 4-8 per 100,000

  • onset between 25-45 years

• etiology

  • majority are sporadic/idiopathic (10% familial)

• incidence

  • typically occurs after 40 years of age, mean onset at 60 years

  • equal incidence in both sexes

  • one of the most prevalent of primary CNS disorders

  • 1-2% of US population older than 60 years

neuromuscular/movement disorders:

progression of disease

== impact motor neuron cell body, axon, neuromuscular junction, or muscle cell function

• disorder may occur at:

  • motor neuron cell body: ALS, SMA

  • axon: guillain-barre syndrome

  • neuromuscular junction: myasthenia gravis

  • muscle cell: muscular dystrophy

ASL

• progressive neurodegenerative disease

  • progressive muscle weakness and respiratory failure

  • wheelchair bound by 12 to 18 months

  • death usually within 2 to 5 years from onset

  • 10% survive for 10 or more years

• initial signs/symptoms

  • difficulty walking with lower extremity weakness

  • hand weakness, clumsiness

  • slurred speech, difficulty swallowing

  • muscle cramps, twitching in upper extremities and tongue

  • difficulty maintaining posture

• cognitive and behavioral changes

  • impaired executive function in 30-50%

• painless, progressive, generalized weakness, asymmetric at onset

• progress to paralysis with no remissions

GBS

• weakness usually plateaus or improves by 4th week

  • strength improves over days to months

  • most return to pre-disease function

• most have near complete recovery with mild symptoms in 3-6 months

  • redisual weakness is common

  • 80% can walk independently within 6 months

  • 60% recovery full strength within 1 year

• 5-10% have delayed or incomplete recovery

• mortality estimated at 3-15%

huntington’s disease

• age of onset related to length of repeated sequences

• increased length leads to earlier disease presentation

neuromuscular/movement disorders:

diagnostic tests and therapeautic interventions

ALS

• clinical features

  • LMN and UMN degeneration

  • progressive spread of symptoms

• electrodiagnostic testing

  • EMG analysis (electromyography): needle electrode inserted into muscle; records electrical activity

  • nerve conduction studies: measures conduction speed of an electrical impulse through a nerve; can determine nerve damage/destruction

• differential diagnosis

  • rule out other potential causes

• genetic testing

  • familial ALS

• medical management

  • riluzole

    • may decrease nerve damage by decreasing glutamate release

    • may slow disease progression in some people

  • edaravone

    • newly approved drug, free-radical scavenger

    • may slow functional decline

    • efficacy appears limited

  • palliative care

    • symptom relief, prevention of complications

    • maintenance of maximal function and quality of life

    • psychosocial support

  • management of feeding and communication problems

  • noninvasive ventilation (NIV)

    • can improve survival

      • associated with 25% reduction in rate of death

      • positive pressure ventilation delivers oxygen to the lungs non-invasively

    • may be denied by insurance

GBS

• diagnosis

  • nerve conduction studies and needle electromyography

  • clinical criteria

    • progressive weakness of more than 1 limb

    • areflexia (decreased DTRs)

• medical management

  • plasmapheresis (plasma exchange)

    • removes and replaces affected plasma in the blood

  • intravenous immunoglobulin (IVIG)

    • intravenous blood product, contains pooled immunoglobulins (antibodies) from donor plasma

  • respiratory support, mechanical ventilation

  • ROM of extremities, prevention of blood clots, therapy, and use of adaptive devices as needed

myasthenia gravis

• diagnosis

  • neurological exam

  • blood test (presence of acetylcholine receptor antibodies)

  • EMG and nerve conduction studies

  • imaging: MRI/CT of thymus gland

  • pulmonary function tests

• medical management

  • anticholinesterase drugs (increase level and duration of ACh action at
    NMJ), steroids, immunosuppressant drugs

  • IVIG and Plasmapheresis

  • thymectomy (surgical removal of thymus gland)

  • 1/3 improve spontaneously

huntington’s disease

• medication (symptom management only)

• tetrabenazine

  • approved to treat chorea, but may trigger depression

• antipsychotic drugs

• antidepressants

• mood stabilizing drugs

• novel drug trials in progress

• therapy (Speech, Psychotherapy, OT, PT)

  • exercise and physical activity can have physical and social benefits

parkinson’s disease

• ***medical management

  • medications provide symptomatic relief only

    • converted to dopamine

    • mimics dopamine

    • prevent breakdown of dopamine

    • management of psychiatric symptoms

  • psychosocial support and education

  • rehabilitation

    • exercise is critical and may have a neuroprotective effect

    • benefit of external cues such as rhythm and music

    • amplitude-based movement training

  • deep brain stimulation (DBS)

    • surgery that helps control motor symptoms and can improve quality of life

      • electrodes implanted in the brain

      • reversible and can be adjusted based on symptoms

      • pulse generator

      • programming device adjusted signals and can turn the device on and off

    • “pacemaker for the brain”

      • electrical impulses disrupt abnormal activity in the brain

    • subthalamic nucleus is targeted to reduce output

neuromuscular/movement disorders:

OT goals/focus for specific conditions

• adaptive ADL techniques and equipment

• social engagement, community resources

• psychosocial and caregiver support

• energy conservation, compensatory strategies

• contracture prevention, skin integrity

• appropriate exercise program

• environmental design, power mobility

CVA:

FAST

(B): ***balance- loss of balance, headache, dizziness

(E): ***eyes- sudden loss of vision in 1 or both eyes

F: ***face- drooping? look uneven?

A: ***arm- weakness

S: ***speech- difficulty

T: ***time- to call 911

CVA:

***neglect

impaired awareness/attention of stimuli on 1 side of the body or environment; usually left side

CVA:

***asomatognosia

lack of awareness of one’s own body parts

CVA:

***anosognosia

decreased awareness or denial of deficits

CVA:

causes of pneumonia for CVA patients

• aspiration

• reduced mobility

CVA:

risk factors

• ***age

  • three-quarters of strokes occur in people ages 65 and older

• ***geography

  • the highest U.S. death rates from stroke occur in the southeastern United States

• ***sex

  • men are more likely than women to have a stroke.

• ***race/ethnicity

  • african americans have 2x the risk of having a first stroke

  • hispanic americans and american indian/alaska natives are at greater risk than white people but are at less risk than african americans

• ***risk factors

  • ***hypertension

  • ***atrial fibrillation

  • hyperlipidemia

  • smoking

  • excessive drinking

  • obesity

  • ***diabetes

  • sedentary life-style

  • contraception with high estrogen

CVA:

***TPA

***Tissue Plasminogen Activator (alteplase, activase) (tPA)

• “mega clot buster”, FDA approved

• gold standard treatment for ischemic stroke, when combined with mechanical thrombectomy

• administered within first 3 hours after symptom onset

• this drug quickly restores blood flow to the brain by dissolving the blood clot

CVA:

typical signs and symptoms

***transient ischemic attack

= thrombus temporarily obstructs blood vessel

• "mini strokes"

• transient episode of neurological dysfunction caused by focal brain, spinal cord, or retinal ischemia without acute infarction

• —> higher risk of stroke

***hemorrhagic stroke

= blood vessel rupture causes bleeding inside the brain

• intracerebral (bleeding inside the brain)

• or subarachnoid (bleeding in the subarachnoid space)

• 13% of strokes

***ischemic stroke

= thrombus or atherosclerotic plaque obstructs blood flow to the brain

1. blood clots form in the heart and travel to the brain

2. these block a cerebral artery and disrupt blood flow to part of brain

• 87% of strokes

CVA:

predisposing conditions

• disease of cerebral vasculature

  • failure to supply oxygen to brain cells, which leads to death

***modifiable risk factors

• high blood pressure

• cigarette smoking

• diabetes

• carotid or other artery disease

• peripheral artery disease

• atrial fibrillation

• other heart disease

• sickle cell disease/sickle cell anemia

• high blood cholesterol

• poor diet

• physical inactivity and obesity

***predictors of recovery

• stroke sub-type

  • intracranial hemorrhage (ICH) has worse initial stroke severity as compared to cerebral infarct (CI), but in long run has similar functional outcomes

• stroke size, severity and resulting deficits

  • neglect, aphasia, dysphagia, dementia (shown to lead to poor functional outcomes)

• cognitive decline and depression

• cardiac comorbidities (HTN, MI, a-fib, cardiomyopathy)

• history of previous stroke

• age

• timing and intensity of rehabilitation program

• pre-stroke physical activity

CVA:

progression of disease

***medical complications after stroke

• medical complications are common after stroke

• contribute to adverse patient outcomes, delayed functional recovery, and increased morbidity and mortality

• acute care complications

  • urinary tract infection

  • myocardial infarction

  • pulmonary embolism

  • chest infection (PNA)

  • other infection

  • fall

  • pain

  • seizure

  • deep vein thrombosis (DVT)

***secondary stroke prevention

• 1 out of 4 stroke survivors experience a recurrence

• control vascular risk factors such as HTN, diabetes, smoking cessation, dyslipidemia

  • platelet antiaggregant (Aspirin)

  • antihypertensive

  • statins

• anticoagulation: if the stroke was caused by cardioembolism due to atrial fibrillation, mechanical valves, or cardiac thrombus

• lifestyle modification

CVA:

diagnostic tests and therapeutic interventions

**immediate diagnostics

• brain imaging as quickly as possible

• blood glucose levels

• oxygen saturation

• platelet count

• markers of cardiac ischemia

• prothrombin time/International Normalized Ratio (PT/INR)

• activated partial thromboplastin time (aPTT)

• electrocardiography (ECG)

***initial stroke management

• recognition of stroke symptoms

• diagnosis of stroke (type, subtype, location)

• admit to stroke unit

  • “golden hour”: door-to-needle <= 60 mins

    • eval within 10 min

    • stroke team notified in 15 min

    • CT scan in 25 min

    • CT and labs interpreted in 45 min

    • medication given if eligible by 1 hour after arrival

• medical treatment of stroke

• early rehabilitation

***national institute of health stroke scale (NIHSS)

• a systematic assessment tool that provides a quantitative measure of stroke-related neurologic deficit

• used as a clinical assessment tool to evaluate acuity of stroke patients, determine appropriate treatment, and predict patient outcome

• levels of…

  • consciousness: state of wakefulness, awareness, alertness

  • language

    • aphasia: loss of ability to understand or express speech

      • broca’s: (expression) difficulty forming words; labored speech

      • wernicke’s: (comprehension) difficulty understanding speech

      • global: severe; impairments in speech, comprehension, reading, writing, naming, repetition

    • motor speech

      • dysarthria: difficult or unclear articulation of speech caused by muscle weakness

  • neglect

    • extinction and inattention

      • neglect: impaired awareness of stimuli on 1 side of the body or environment; usually left side

      • extinction: only responds to 1 sided stimulus when 2 stimuli are simultaneously presented on opposite sides of the body

      • anosognia: decreased awareness of deficits

  • visual-field loss

    • gaze palsy: inability to move eyes horizontally or vertically

    • hemianopsia: loss of vision in half of visual field in both eyes

  • extraocular movement

  • motor strength/function

    • hemiparesis: weakness, mild loss of strength on 1 side of the body

    • hemiplegia: complete loss of strength on 1 side of the body

  • ataxia: lack of muscle control or coordination

    • finger-nose-finger

    • heel to shin

  • dysarthria

  • sensory loss

• provides common language among health professionals

***therapeautic interventions/treatment: ISCHEMIC STROKE

• Tissue Plasminogen Activator (alteplase, activase) (tPA)

  • “mega clot buster”, FDA approved

  • gold standard treatment for ischemic stroke, when combined with mechanical thrombectomy

  • administered within first 3 hours after symptom onset

  • this drug quickly restores blood flow to the brain by dissolving the blood clot

• antithrombotic therapy

  • antiplatelet and anticoagulation

  • prevents future blood clots from forming and growing

• non-pharmacological interventions

  • mechanical thrombectomy

    • thread catheter through artery to directly remove the clot from the blocked blood vessel

    • beneficial for people with large clots

    • often performed in combination with injected tPA

    • recommended within 16 hours and reasonable up to 24 hours in selected patients with AIS with large vessel occlusion

  • carotid endarterectomy (CEA)

    • this surgery removes the plaque blocking a carotid artery and may reduce the risk of ischemic stroke

      • more invasive

      • involves risks, especially for people with heart disease or other medical conditions

***therapeautic interventions/treatment: HEMORRHAGIC STROKE

• medication

  • lower blood pressure

  • lower cranial pressure

  • reduce vasospasm

  • prevent seizure

• surgery

  • endovascular embolization

    • coiling- embolization by coil

    • clipping- embolization by clip

  • arteriovenous malformation removal

  • craniotomy

***secondary stroke prevention

• 1 out of 4 stroke survivors experience a recurrence

• control vascular risk factors such as HTN, diabetes, smoking cessation, dyslipidemia

  • platelet antiaggregant (Aspirin)

  • antihypertensive

  • statins

• anticoagulation: if the stroke was caused by cardioembolism due to atrial fibrillation, mechanical valves, or cardiac thrombus

• lifestyle modification

CVA:

OT goals/focus for specific conditions

***best practice recommendations

• patients should receive a recommended three hours per day of direct task-specific therapy, five days a week, delivered by the interprofessional stroke team

• the team should promote the practice and transfer of skills gained in therapy into the patient’s daily routine

• it is recommended that patients be given opportunities to repeat rehabilitation techniques learned in therapy and implement them while supervised by stroke rehabilitation nurses

• therapy should include repetitive and intense use of novel tasks that challenge the patient to acquire the necessary skills needed to perform functional tasks and activities

• management of UE following stroke

  • training should encourage the use of patients’ affected limb during functional tasks and be designed to simulate partial or whole skills required in activities of daily living

  • prevention of Hemiplegic Shoulder Pain and Subluxation

  • ex: Repetitive Task Practice, Bimanual Training, CIMT, Mirror Therapy, Virtual Reality

• management of LE following stroke

  • patients should engage in training that is meaningful, engaging, progressively adaptive, intensive, task-specific and goal-oriented in an effort to improve transfer skills and mobility (mobility, balance, transfers)

  • aerobic training

• rehabilitation of visual perceptual deficits

  • patients with suspected perceptual impairments (visuo-spatial impairment, agnosias, body schema disorders and apraxias) should be assessed using validated tools

  • treatment of neglect can include visual scanning techniques, phasic alerting, cueing, imagery, virtual reality, hemispheric (limb) activation and trunk rotation

  • patients with suspected limb apraxia should be treated using errorless learning, gesture training and graded strategy training

• falls prevention

• management of dysphagia and nutrition

stroke and movement disorder rq:

Bob presents with severe left-sided neglect and mild left-sided hemiparesis, which appears to impact his ability to perform activities of daily living. which therapy would he benefit most from?

• upper body dressing

• manipulating and placing coins in a jar

• simulating a driving activity

• having him track from left to right to find items in an overhead cabinet

having him track from left to right to find items in an overhead cabinet

stroke and movement disorder rq:

below are all risk factors for lumbar degenerative disc disease EXCEPT:

• obseity

• lack of physical activity

• excessive cardio exercise

• smoking

excessive cardio exercise