CHP 7 neurological system disorders

frontal lobe main elements (3)

• precentral gyrus: primary motor cortex for voluntary muscle activation

• prefrontal cortex: controls emotions, judgements, higher-order cognitive fxn

• premotor cortex: planning movements

  • brocas area: motor aspect of speech

parietal lobe elements

• postcentral gyrus: primary sensory cortex for integration

• receives fibers conveying touch, prop, pain, and temp from opposite side of body

temporal lobe (3)

• primary auditory cortex: receives/processes auditory stimuli

• associative auditory cortex: processes auditory stimuli

• wernicke’s area: language comprehension

occipital lobe (2)

• primary visual cortex: receives/processes visual stimuli

• visual association cortex: processes visual stimuli

limbic system

• consists of…

  • limbic lobe

  • hippocampal formation

  • amygdaloid nucleus

  • hypothalamus

  • anterior nucleus of thalamus

• instincts and emotions contributing to preservation of individual

• basic functions:

  • feeding, aggression, emotions, endocrine of sexual response, long-term memory formation

subcortical white matter

• what

• elements (3)

• myelinated nerve fibers located centrally

• elements

  • corpus callosum: connects hemispheres to allow communication

  • projection fibers: connect cerebral hemispheres with other portions of brain and spinal cord

  • association fibers: connect different portions of cerebral hemispheres, allowing cortex to function as integrated whole

basal ganglia

• masses of gray matter deep within cerebral hemispheres

• basic function:

  • initiates voluntary movement, controls postural adjustments, refines coordination, forms and stores motor plans, and produces dopamine

• disorders associated:

  • parkinson’s disease: reduced dopamine production

  • huntington’s chorea: degeneration of caudate nucleus

thalamus

• sensory nuclei: integrates and relays sensory information from body, face, retina, and taste receptors to cerebral cortex (no smell)

• motor nuclei: relays motor info from cerebellum and globus pallidus to precentral motor cortex

hypothalamus

• integrates and controls functions of autonomic NS and neuroendocrine system

• maintains body homeostasis

substantia nigra

• large motor nucleus connecting with basal ganglia and cortex

• important for motor control and muscle tone

pons

• connects medulla oblongata and midbrain for passage of ascending and descending tracts

• basic functions:

  • controlling autonomic fxn (pain and arousal)

  • relay system

  • REM sleep

medulla oblongata

• acts as vital cardiac, respiratory, and vasomotor center

• controls reflex actions (vomiting, swallowing, gagging, coughing)

• important for head movements and gaze stabilization

cerebellum

• anterior, posterior, and flocculonodular lobes

• functions:

  • proprioceptive regulation (posture and voluntary movement)

  • motor planning, timing and coordination

  • trunk control, balance, equilibrium, muscle tone

• common disorders:

  • friedreichs ataxia

  • spinocerebellar ataxia

spinal cord

• five sections:

  • cervical C1-C8

  • thoracic T1-T12

  • lumbar L1-L5

  • sacral S1-S5

  • **coccygeal: not SC but nerve roots that form cauda equina

• ventral (anterior) horn = efferent (motor) neurons

• dorsal (posterior) horn = afferent (sensory) neurons

• ascending and descending tracts

ascending tracts (4)

• sensory pathways from body to brain

• types:

  • dorsal columns/medial lemniscal: convey sensations of prop, kinesthesia, vibration, pressure, and discrimination (tactile & prop)

  • spinothalamic: conveys sensations of pain, temperature, and crude touch (tactile)

  • spinocerebellar: unconscious prop, touch, and pressure from lower body

  • spinoreticular: deep and chronic pain

descending tracts (4)

• motor pathways from brain to body

• types

  • corticospinal: cross at medulla > contralateral voluntary motor control

  • vestibulospinal: muscle tone, antigravity muscles, postural reflex

  • rubrospinal: contralateral

  • reticulospinal: preparatory and movements for activity and postural control

complete cord lesion: UMN

• complete bilateral loss of all sensory modalities

• bilateral loss of motor function with spastic paralysis below level of lesion

• loss of bladder and bowel

central cord lesion

• UMN

• caused by hyperextension

• more UE deficits than LE

• LOSS

  • bilateral loss of pain and temp

  • bilateral loss of motor function

• KEEP:

  • prop and discriminatory sensations

brown-sequard syndrome

• UMN

• caused by trauma resulting in hemisection of spinal cord (one side is injured, vertical)

• LOSS

  • ipsilateral loss of tactile discrimination, pressure, vibration, and prop

  • ipsilateral loss of motor function and spastic paralysis BLOI

  • contralateral loss of pain and temp

  • bilateral loss of pain and temp

anterior cord syndrome

• UMN

• caused by flexion injuries

• hemisection of cord (horizontal) loss of anterior cord

• LOSS

  • bilateral motor function w/spastic paralysis BLOL

  • bilateral pain and temp

• KEEP

  • prop, kinesthesia, vibration

posterior cord syndrome

• UMN

• least frequent

• loss of dorsal column bilaterally

• LOSS

  • bilateral prop, vibration, pressure, stereognosis, discrimination

• KEEP

  • motor function, pain, light touch

cauda equina

• LMN

• loss of long nerve roots at or below L1

• incomplete lesion common

• potential for regeneration (up to 1yr)

• RESULT

  • flaccid paralysis with no spinal reflex activity

  • flaccid paralysis of bladder and bowel

conus medullaris

• LMN

• injury of sacral cord and lumbar nerve root

• LOSS:

  • LE motor and sensory loss

  • areflexic bowel and bladder

autonomic nervous system (ANS)

• involuntary muscles (smooth muscle, heart, glands)

• helps maintain homeostasis

• two division:

  • sympathetic: fight or flight, emergency response, incr BP, inhibits peristalsis

  • parasympathetic: converses and restores, slow HR, decr BP, peristalsis

• modulated by brain centers

  • descending autonomic system: hypothalamus, lower brain stem

  • cranial nerves

cerebrospinal fluid

• clear fluid that provides mechanical support (cushion), controls brain excitability by regulating ionic compo and exchange of nutrients

  • normal pressure: 70-180 mm/H2O

  • total volume: 125-150 cc

• hydrocephalus: abnormal accumulation of CSF in ventricles > pressure on brain

brain blood supply (4)

• carotid system

• vertebrobasilar system

• circle of willis

• venous drainage

neurons

• vary in size and complexity

  • cell body, dendrites, axons, myelin, synapses, nodes of ranvier, neuromuscular junction

• disorders:

  • myasthenia gravis: disorder of NMJ

• TYPES:

  • nuclei: compact groups in PNS

  • projection: CNA

  • axon bundles: spinal

  • neuroglia: support, production

  • UMN: motor from brain to brain

  • LMN: motor from brain to body

• nerve fibers:

  • A: large, fast. myelin

    • Alpha: prop, somatic motor

    • Beta: touch, pressure,

    • Gamma: motor

    • Delta: pain, temp, touch

  • B: small, myelin, slow

  • C: smallest, no myelin, slowest

UMN lesion

• location: CNS

• structures: cortex, brainstem, corticospinal tract, spinal cord

• disorders: stroke, TBI, SCI

• sx:

  • hypertonia, velocity-dependent

  • hyperreflexia, clonus, babinski response

  • muscle spasms

  • bilateral, contralateral, ipsilateral weakness or paralysis; impaired or absent voluntary movements

  • disuse atrophy

LMN lesions

• location: peripheral NS

• structures: anterior SC, spinal roots, peripheral nerves, CN

• disorders: Polio, guillain-barre, PNI, peripheral neuropathy

• SX:

  • hypotonia, flaccidity

  • hyporeflexia

  • fasciculations

  • limited distribution of strength

  • neurogenic atrophy, severe wasting

  • weak or absent voluntary movements if nerve impacted

cranial nerves

• 12 pairs

  1. olfactory: sensory, smell

  2. optic: sensory, vision

  3. oculomotor: motor, eyes movement

  4. trochlear: motor, eye movement

  5. trigeminal: both, chewing, head sense

  6. abducens: motor, eye mvmt

  7. facial: both, facial sensation and motor

  8. vestibulocochlear: sensory, hearing & balance

  9. glossopharyngeal: both, swallowing, head sense, taste, lungs, parotid

  10. Vagus: both, voice, swallowing, taste, heart, vessels, lungs, bowels

  11. accessory: motor, trap (neck)

  12. hypoglossal: motor, tongue

dermatomes and myotomes

• dermatomes: sensory distribution along the skin

• myotomes: nerve distribution for skeletal muscles

spinal reflexes

• what

• types (5)

• involuntary response to stimuli

  • basic, specific, predictable

• provide basis for unconscious motor function and basic defense mechanisms

• types:

  • stretch (myotatic)

    • stimulation with muscle stretch

    • maintain muscle tone, support agonist muscle, provide feedback about muscle length

  • inverse (myotatic) stretch:

    • stimulation with muscle contraction

    • provide agonist inhibition, dim force of agonist contraction

  • gamma reflex loop

    • stretch reflex forms part of loop

    • allow muscle tension to come under control > regulating level of tension in muscle

  • flexor (withdrawal)

    • stim with cutaneous sensory stim to large flexor muscles

    • protective withdrawal to remove body part from harmful stimuli

  • crossed extension

    • stim with noxious stim and reciprocal action of antagonist (flexors on one side excited > extensors on same side inhibited)

    • coordinates reciprocal limb ax (gait)

CVA

• disease of cerebral vasculature in which there is failure of blood and oxygen to the brain, causing brain death

• types:

  • transient ischemic attack (TIA):

    • mini stroke, transitory stroke that lasts for few minutes; warning stroke

    • sx usually disappear within the hour (may persist 24hr)

      • numbness, weakness to face, arm, or leg (one side); confusion, slurred words, vision difficulty, LOB

  • ischemic stroke

    • most common due to embolism or thrombosis of cranial arteries

  • hemorrhagic stroke

    • bleed secondary to hypertension or aneurysm or AVM

• SX:

  • abrupt unilateral signs (weakness, vision loss, sensory changes)

    • contralateral manifestation

  • specific sx determined by site of stroke

  • FAST

    • F = face drooping

    • A = arm weakness

    • S = speech difficulty

    • T = time to call 911

• diagnosis: cerebrovascular imaging & diagnostic testing

• medical care: antithrombotic, thrombolytic therapy (t-PA)

stroke sx by artery

• middle cerebral (MCA): contralateral hemiplegia, hemianesthesia, hemianopsia

  • aphasia, unilateral neglect, bilateral apraxia (left MCA) or left apraxia (right MCA)

• internal carotid (ICA): sx similar to MCA

• anterior cerebral (ACA): contralateral hemiplegia, grasp reflex, incontinence, confusion, apathy

• posterior cerebral (PCA): homonymous hemianopsia, thalamic pain, hemisensory loss

• vertebrobasilar: dysarthria, dysphagia, emotional instability

left vs right hemisphere functions

LEFT:

• movements of right side

• process sensory info from right

• visual reception from right

• visual verbal processing

• bilateral motor praxis and auditory reception

• verbal memory, speech, process verbal auditory

RIGHT

• movement of left side

• sensory info from left

• vision on left

• visual spatial, nonverbal memory, attention, emotional lability, interpreting abstract info, nonverbal auditory

• left motor praxis

traumatic brain injury (TBI)

• open or closed trauma from fractures, trauma to blood vessels, nerves, or meninges, hemorrhage, edema, etc.

  • open: damage from penetration of skull (bullet)

  • closed:

    • rapid acceleration or deceleration

    • blunt external force to head

• SX:

  • concussion (loss of consciousness)

  • cerebral contusion/laceration/edema

  • hemiplegia, abnormal reflexes, fixed pupils

  • coma, decorticate or decerebrate rigidity

Glasgow Coma Scale

• 15-point scale of levels of consciousness done at initial eval

• DOMAINS

  • eye opening

  • verbal response

  • motor response

• indicates class of brain injury (mild, moderate, severe)

• admin right after initial injury

• SCORE: 3 domain criteria will be scored and totaled

  • 8 or less = severe TBI

  • 9-12 = moderate TBI

  • >13 = mild TBI

decorticate rigidity

• UE in spastic flexion w/IR and add

• LE in spastic extension w/IR and add

• corticospinal tracts

• associated with better outcomes

decerebrate rigidity

• brainstem and extrapyramidal tracts

  • poorer prognosis

• both UE/LE in spastic extension, add, IR

• wrists and fingers flexed

• ankles plantar flexed with feet inverted

• trunk extended

• head retracts

Rancho Los Amigos Levels of Cognitive Functioning Scale

• clinical tool used to rate how people with brain injury are recovering

• 10 levels

Rancho level I

No response: Total assistance

• no response to ALL stimulus

• total assist

Rancho Level II

Generalized Response: Total assistance

• GENERALIZED response to pain through gross bodily movement

• vocalization = inconsistent

• total assist

Rancho Level III

Localized Response: Total assistance

• LOCALIZED response to discomfort stimuli inconsistently when localized with commands

• withdrawal or vocalization to painful stim

• turn towards or away from auditory

• follows moving object passed in visual field

• discomfort > pulls at restraints and tubes

• inconsistently responds to simple commands

• total assist

Rancho Level IV

Confused/Agitated: Maximum assistance

• alert and in heightened state of anxiety

• purposeful attempts to remove restraints, tube, lines

• may perform motor ax > sitting, reaching, walking (no purpose)

• very brief sustained attention, absent STM, aggressive, mood swings, no cooperation

Rancho Level V

Confused, Inappropriate, Non-agitated: Maximal A

• alert, not agitated, wanders, not oriented, frequent brief sustained attention

• impaired memory, absent goal-direct problem solving, unable learn new info

• requires frequent cues and structure

Rancho Level VI

Confused, Appropriate: Mod A

• inconsistently oriented, more memory depth, vague recognition of familiar

• emerging awareness of appropriate

• MOD a for problem solving

• max a for new learning, supervision for old

• consistently follows simple directions

Rancho Level VII

Automatic, appropriate: Min A for Daily

• consistently oriented to person & place

• able to attend to familiar w no distractions

• min a for new learning, carryover of learning

• able to carry out routine and modify plan

• superficial awareness of condition

• unrealistic planning for future, overestimates abilities, oppositional, no thought of consequences

• unaware of others feelings or inappropriate social behaviors

Rancho Level VIII

Purposeful, appropriate: stand by

• consistently oriented

• indp attend to familiar

• able to recall, use memory aids for daily

• initiates and carries out daily routines and other roles with stand by a

• no assist for new learning

• aware of impairments, thinks of consequences, acknowledges others

• depressed, irritable, self-centered, depressed

• aware of inappropriate social behaviors and corrects with min a

Rancho Level IX

Purposeful, Appropriate: stand by on request

• indp multi-task at least 2 hrs

• assistive memory devices

• thinks about consequence with a when requested, accurately estimates abilities

• acknowledges others with stand by

• depression, easily irritable, self monitor appropriateness

Rancho Level X

Purposeful, Appropriate: Mod I

• multi task with periodic breaks

• manage assistive devices indp

• indp carries out familiar and unfamiliar tasks

• anticipates limitations, indp thinks of consequences, accurately estimates abilities

• recognize and appropriately respond to others

• mental health aligning with "typical" > occasional depression, irritable when sick or fatigued, etc.

mild TBI

• a bump or jolt to either the head or the body that causes the brain to move rapidly inside the skull

• may result in loss of consciousness 0−30mins

  • post traumatic amnesia (PTA) no longer than 24hr

• imaging often show no damage

• sx:

  • clumsy, slow, mood or behavior changes, vomiting, headache, dizziness, sensitivity, confusion

• severe concussion: seek emergency services

  • drowsiness or inability to wake, loss con longer than 30s, pupils differ in size, headache gets worse, slurred speech

post-concussion syndrome

• set of sx that may continue for weeks, months, or year after concussion

• sx:

  • headache, fatigue, cognitive impairments, dizziness, depression, anxiety, impaired balance, sensory sensitivities, emotional problems

spinal cord injury (SCI)

• nontraumatic or traumatic compression, shearing, or contusion to spinal cord

• sx: vary depending on severity

  • spinal shock: 4-8wks, all reflex activity is obliterated BLOI > flaccid paralysis for time being

  • sensory deficits, loss bowel/bladder, loss temp control, decreased resp, sexual dysfunction, muscle tone changes

ASIA impairment scale

GRADE A: Complete, no motor or sensory

GRADE B: Sensory Incomplete, sensory but no motor function

GRADE C: Motor Incomplete, motor function is preserved below the neurological level, and the majority of key muscle groups have a muscle grade of less than or equal to 3/5

GRADE D: Motor Incomplete , motor function is preserved below the neurological level, and the majority of key muscle groups have a muscle grade of greater than or equal to 3/5

GRADE E: Normal; sensory and motor

C1-C3 SCI

• possible movements:

  • neck flex, etx, and rotation

  • limited movement of head and neck

• functional capabilities:

  • ventilator dependent, unable to clear secretions (cough)

  • difficult or limited communication > AAC

  • total assist for ADLs and IADLs

  • total assist for bed mobility

  • indp use power chair with head or mouth controls

C4 SCI

• PHYSICAL ABILITIES:

  • head & neck control

  • shoulder shrug (scapular elevation due to traps)

• FUNCTIONAL GOALS

  • may require vent; assisted cough

  • operate power chair using head control, mouth stick, sip & puff or chin control

  • total assist for ADLs and IADLs, possible indp with AT for eating

  • total assist for bed mobility

C5 SCI

PHYSICAL ABILITIES:

• head & neck control, shoulder shrug (scapular elevation)

• some shoulder control

• elbow flexors (biceps, brachialis) able to

  • bend elbows & turn palms up

FUNCTIONAL GOALS:

• with DME can be independent with eating & grooming

• with caregiver assistance may be able to perform UE dressing & some bathing

• total assist for mobility

• power w/c

• assisted cough

• AE: universal cuff

C6 SCI

PHYSICAL ABILITIES:

• movement of head, neck, shoulders, arms & wrist

• ADD: turn palms up & down & extend wrists

• C6 wrist extensors: extensor carpi radialis longus & brevis)

• tenodesis grasp!!

FUNCTIONAL GOALS:

• DME, can be independent with eating & grooming & UE dressing

• may need assist with LE dressing

• some assist for transfers (slide board)

• ultra light manual w/c or power for ease in community/uneven distances

• AE: universal cuff, dressing stick, leg lifter, long-handled sponge

C7 SCI

PHYSICAL ABILITIES:

• movement of head, neck, shoulder, arms, & wrist

• elbow extensors (triceps)

FUNCTIONAL GOALS:

• may need some or no assistance for ADLs and functional mobility

• independent transfers

• independent with manual w/c

C8 SCI

PHYSICAL ABILITIES:

• movement of head, neck, shoulders, arms, ELBOWS!!, wrist, HANDS!!

• added strength & precision of hands & fingers

• C8: finger flexors (flexor digitorum profundus) to middle finger)

• T1: small finger abductors (abductor digiti minimi)

FUNCTIONAL GOALS:

• may need some or no assistance for ADLs and functional mobility

• independent transfers

• independent with manual w/c

T2-T12 SCI

PHYSICAL ABILITIES:

• normal function head, neck, shoulders, arms, hands, fingers

• increased use of rib & chest muscles

• T10-12: more improvements in trunk control due to increased ab strength

FUNCTIONAL GOALS:

• independent!! in self care (bowel & bladder), w/c push ups for pressure relief, bed mobility & transfers

• manual w/c

• load and unload w/c for driving with hand controls

L1-S5 SCI

FUNCTIONAL ABILITIES

• gradual gain of hip, knee, foot movements

• depending on level of injury, some degree of voluntary bladder, bowel, & sexual function returns

FUNCTIONAL GOALS

• ambulate with specialized leg braces & walking device

• functionality of ambulation depends on strength & movement in LEs

• may use w/c for community mobility

SCI complications

• respiratory complications! (pneumonia)

• decubitus ulcer

• orthostatic hypotension

  • rapid decr in BP when changing positions

• DVT

• autonomic dysreflexia: abnormal response to noxious stim resulting in extreme incr in BP, headache, sweating

  • usually pain or irritant BLOI

  • medical emergency

• UTI

• heterotopic ossification

cerebral palsy

• caused by malformation of brain structures and/or injury to brain structures during prenatal, perinatal, or postnatal periods (until 2yrs)

  • four P’s (premature)

• common causes

  • premature birth w/injury to brain

  • low birth weight

  • maternal infection or infant infection

  • hypoxia!!

• non progressive

• comorbidities:

  • respiratory problems, seizure disorders, scoliosis, contractures, pressure sores

• cognitive and language skills vary

• early indicators:

  • hypotonia, spasticity, head lag, persistent primitive reflexes, no protective responses, clonus, global delay

• types:

  • spastic, dyskinetic, ataxic, mixed

spastic CP

• most common (88%)

• increased muscle tone (hypertonia)

• muscle spasticity: velocity-dependent resistance to stretch; stiffness upon movement

  • combo of hypo and hyper tone in different muscles

• restricts voluntary movement, present at rest

• white matter, pyramidal(UMN)

• TYPES: hemiplegia, diplegia (preemie), quadriplegia (most severe)

dyskinetic CP

• asphyxiation at birth (hypoxic ischemic encephalopathy)

• abnormal muscle tone which may involve whole body

  • decr tone during sleep

• changing pattern of muscle tone

• involuntary movements often present

• TYPES:

  • chorea

    • rapid, random, jerky movements

  • athetoid

    • slow, writhing, involuntary movements; wrist & feet

  • choreoathetoid

    • combo of slow and rapid

  • dystonic

    • involuntary muscle contraction with repetitive movements and abnormal posture

ataxic CP

• cerebellum involvement

• balance and coordination issues

• abnormal voluntary movement

• difficulty with trunk/limb position in space

• often walk with unsteady gait, with a wide based gait

• difficulty with precise coordination (overshooting)

Gross Motor Functional Classification System (GMFCS)

1. walks without limitations

2. walks with limitations

3. walks using hand-held mobility device

4. self-mobility with limitations; may use powered mobility

5. transported in manual wheelchair

Manual Ability Classification System (MACS)

1. handles objects easily and successfully

2. handles most objects but with somewhat reduced quality and/or speed of achievement

3. handles objects with difficulty; needs help to prepare and/or modify activities

4. handles limited selection of easily managed objects in adapted situations

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

CP distribution

• quadriplegia

  • all 4 limbs

• diplegia

  • all four limbs; LEs more severely affected than UEs

• hemiplegia

  • one side affected; UE usually more involved

• triplegia

  • three limbs; usually UEs + LE

• monoplegia

  • only one limb; usually UE

chorea

• brief, purposeless, involuntary movements of distal extremities and face

dyskinesias

• involuntary, nonrepetitive, but occasionally stereotyped movements affecting distal, proximal, and axial musculature

myoclonus

• brief and rapid contraction of muscle or group of muscle

parkinson’s disease

• long-term, slowly progressive movement disorder

• may live 20-30 years, onset 40+

• progressive loss of voluntary and involuntary motor function

• CAUSE: hereditary > environmental

  • death of neurons of the substantia nigra (SN), specifically pars compacta (PC) portion, in the basal ganglia (BG), resulting in decreased production of neurotransmitter dopamine

  • lewey bodies contribute to PD

• cardinal signs: TRAP

  • Tremor

  • Rigidity

  • Akinesia

  • Postural instability

• TX:

  • pharma for dopamine

    • side effects > more as disease progresses, involuntary movements, become resistant

stages of parkinson’s

1. unilateral tremor, rigidity, akinesia, minimal or no functional impairment

2. bilateral tremor, rigidity or akinesia, w/or w/out axial signs, indp with ADLs, no balance impairments

3. worsening of sx

   • first sign of impaired righting reflexes, onset of disability in ADLs, can be indp

4. requires help with some or all ADLs, unable live alone w/out assistance, able to walk and stand unaided

5. unable to stand, walk, or perform ADLs, w/c and max a

neural tube defects

• complex interaction of environmental and genetic factors, which contribute to malformation of brain, vertebrae, and spinal cord

  • lack of brain development, malformation of skull, malformation of vertebral arches

  • anencephaly: more severe malformation of skull and brain; no neural development above brainstem

  • encephalocele: malformation of the skull where portion of brain protrudes

  • spina bifida: most common

• lack of folic acid may induce

• onset: prenatal (within 26 days conception)

• outcome: spontaneous abortion is common, infancy mortality high, better outcomes with frontal malformation

spina bifida

• malformation of vertebral arches

• types

  • occulta: bony malformation with separation of vertebral arches, no external manifestations

    • most common

    • occult spinal dysraphism (OSD): external manifestation (red birthmark, patch hair, dermal sinus, dimple)

    • sx: usually none, maybe slight instability, mild gait alterations, bowel/bladder problems

  • cystica: exposed pouch of spinal cord and meninges

    • meningocele: protrusion of sac through spine containing spinal fluid and meninges; no spinal cord protrusion

      • sx: slight instability, minimal functional impairments (no spinal cord protrusion)

    • myelomeningocele: protrusion of spinal fluid, meninges, and spinal cord (most common in lumbar)

      • sx: sensory and motor deficits occurring BLOL, paralysis BLOL, bowel/bladder incontinence (S2-S4)

• tx: shunts, neonatal sac repair, therapy

neurogenic bowel

• absence of innervation (bladder, urethra, and bowel)

• bowel and bladder dysfunction present in all children with meningomyelocele

INTERVENTIONS:

- Clean Intermittent Catheterization

- Bowel Management

tethered cord syndrome

• end of spinal cord adheres to one of lower vertebra > spinal cord stretched > damaged and interferes with blood supply to spinal cord.

• may go undiagnosed until sx occur

• sx:

  • visible signs > hairy patch, dimple, red mark

  • bowel/bladder difficulties, gait disturbances, feet deformities, low back pain, scoliosis

chiari type II malformation

• more severe form

• displacement of brainstem and cerebellum into spinal canal causing compression on spinal cord, cerebellum, and brain stem

• common in meningomyelocele

• sx:

  • trouble breathing, swallowing problems, weakness, arching of head backward

ventriculo-peritoneal shunt (VP shunt)

• most common type shunt for hydrocephalus

• divert CSF from ventricles to abdomen to be better absorbed

• known to fail!!! early recognition is key

COMPLICATIONS:

• blocked or infected

  • sx: extreme head growth, regression, change in UE fxn, decline in cog fxn, impaired vision, seizures, LOB

• by 2-3 yrs half of shunts have failed and been replaced

muscular dystrophies

• group of degenerative disorders resulting in muscle weakness and decreased muscle mass due to hereditary disease process

• absent muscle protein dystrophin

  • blood test or biopsy to ID

• onset: anytime, avg age > 5yrs

• progression: may be rapid & fatal or stable (earlier the appearance of sx > more severe and rapid)

• types

  • Duchenne’s (DMD)

    • fast

  • Becker’s (BMD)

    • slow

  • limb-girdle (LGMD)

    • onset late life, affects pelvis and shoulders, slow

  • facioscapulohumeral (FSHD)

    • early adolescence, slow

  • myotonic (DM)

  • emery-dreifuss (EDMD)

  • distal muscular dystrophy

  • oculopharyngeal muscular dystrophy

duchenne’s MD (DMD)

• most common

• boys!

• detected btw 3-5yrs, survival rates > rarely beyond 20yrs

• sx:

  • pseudohypertrophy (enlarged calf muscles)

  • weakness at proximal joints > sig fxnal mobility impairment

    • trendelenburg’s waddle

    • difficulty getting up from floor > Gower’s

  • muscle weakness in all voluntary (incl. heart and diaphragm)

  • behavioral/learning difficulties

becker’s MD

• slower to progress than DMD, less severe, and less predictable

• survival into late adulthood

• sx:

  • loss motor fxn hips, thighs, pelvic, and shoulders

  • enlarged calves

  • cardiac system involvement

spinal muscular atrophy

• weakness of voluntary muscles of shoulders, hips, thighs, and upper back resulting in spinal curvature

• muscles for breathing and swallowing can be affected

• age of diagnosis and onset determines severity and functional deficits

myasthenia gravis

• disease caused by autoimmune attack on acetylcholine receptors at NMJ

• initiating event unknown

• onset & prognosis: any age, most often young women and older men, prognosis varies but usually progressive

  • can be congenital

• sx:

  • ptosis, diplopia, muscle fatigue, dysphagia, and proximal limb weakness

  • sensation and deep tendon reflexes intact

  • sx fluctuate, relapsing periods

  • mysathenic crisis: progressive weakness, tachycardia, tachypnea, dysphagia, impaired speech, anxiety, decr respiratory fxn > seek emergency services!!

charcot-marie tooth disease

• involves peripheral nerves marked by progressive weakness, primarily in peroneal and distal leg muscles

• occurs in teen years or earlier

myopathies

• sx similar to dystrophies but myopathies progress slowly with better prognosis

• weakness of face, neck, and limbs

progressive supranuclear palsy (PSP)

• rare brain disease causing

  • progressive problems with gait and balance

  • complex eye movements

  • cognitive impairments

• classic sign: inability to coordinate eye movements causing blurry vision

• other sx: altered mood, depression, apathy, progressive dementia

  • can be confused w/parkinsons however PDP is more rapid with NO tremors

huntington’s chorea

• autosomal dominant disorder

• onset & prognosis: insidious at middle age > progressive until end of life

• sx:

  • choreiform movements and progressive ID

    • chorea movements (rapid, jerky, unpredictable)

  • psychiatric disturbances

  • impaired walking and balance

cerebellar/spinocerebellar disorders

• lesions

• degenerations

• disorders

  • ataxia, dysmetria (under-overshoot mvmts), dysdiadochokinesia (inability perform rapid mvmt sequences), hypotonia, movmt decomp, intention tremors, nystagmus

• cerebellar lesions:

  • strokes, tumor deposits, MS, alcoholism

• spinocerebellar degenerations:

  • progressive ataxia due to degeneration of cerebellum, brain stem, spinal cord, peripheral n, and basal ganglia

  • types: spinal ataxias, cerebellar ataxias, multiple system degeneration

    • friedrichs ataxia: onset in childhood causing gait unsteadiness, UE ataxia, tremors, areflexia, scoliosis

Amyotrophic lateral sclerosis (ALS)

• motor neuron disease of unknown etiology

• more prevalent in men than women

• average onset at 57, prognosis 2-5yrs

• sx:

  • muscle weakness & atrophy. distally & asymmetrically

    • begins at hands

  • cramps precede weakness

  • spasticity, hyperactive deep tendon reflexes, dysarthria, dysphagia

  • sensory systems, eyes, urinary

• medication management (2 FDA approved options)

• tx focused on secondary complications (spasticity)

brachial plexus disorder

• secondary to traction during birth, invasion of metastatic cancer, post-radiation tx, or traction injury

  • common in shoulder dystocia at birth

• sx:

  • mixed motor and sensory disorders corresponding to limb

  • rostral (medial/proximal) injuries = shoulder dysfunction

  • caudal (lateral/distal) injuries = hand dysfunction

• common injuries:

  • Erb’s palsy: paralysis of upper Brachial plaxus w/ C5-6 nerves

    • shoulder paralysis, arm cannot be raised, weak elbow flexion, weak scapula

      • 6mos > contractures may form

    • waiters tip position: arm straight and wrist fully bent

  • Klumpke’s palsy: paralysis of lower BP w/ C7-8 and T1 nerves

    • rare compared to Erbs

    • paralysis of hand and wrist with ipsilateral horners (miosis, ptosis, and facial anhidrosis)

    • limp hand and immobile fingers

horners syndrome

• miosis

• ptosis

• facial anhidrosis

peripheral neuropathy

• multiple or single nerve affected due to variety causes

  • trauma, pressure paralysis, forcible overextension of joint, hemorrhage, diabetes M, lyme disease, vascular disease, malnutrition, alcohol abuse

• sx:

  • sensory, motor, reflex, and vasomotor sx

    • pain, weakness, paresthesia at affected nerve area

• tx focuses on underlying cause not neuropathy itself

guillian-barre syndrome

• unknown cause

• autoimmune disease that occurs after infection, surgery, or immunization (little evidence)

• acute and rapidly progressing demyelination of peripheral nerves and spinal nerve roots

• onset & prognosis: any age, 2-4wk recovery, most ambulatory in 6mos, half have mild neurological deficits

• sx:

  • symmetric muscular weakness and mild distal sensory loss

    • distal to proximal, gradual loss

  • minor sensory signs (painful extremities)

  • deep tendon reflexes lost (sphincters spared)

  • respiratory failure and dysphagia in some cases

post-polio syndrome (PPS)

• some motor neurons infected by polio recover, after years of stability > break down and cause muscle weakness

• onset & prognosis: 15yrs after polio, slow progress w/good prognosis

• sx:

  • new weakness, easily fatigued, muscle pain, joint pain, cold intolerance, atrophy

multiple sclerosis (MS)

• slowly progressive CNS disease characterized by patches of demyelination in brain and spinal cord

• onset, prevalence, prognosis

  • most often btw 20-30yrs (diagnosed in 30’s)

  • prognosis variable

• diagnosed by sx criteria (maybe MRI for lesions)

• multiple and varied neurologic sx with insidious onset

  • fatigue,

  • paresthesias of extremities, trunk, or face

  • weakness or clumsiness in leg or hand

  • visual distrubances

  • emotional disturbances

  • balance loss or vertigo

  • bowel/bladder dysfunction

  • cognitive changes (apathy, memory loss, inattention)

  • spasticity, incr reflexes, ataxia, tremors, pain

  • sexual dysfunction

• courses:

  • relapsing remitting

  • secondary progressive: slow progress with plateau periods

  • primary progressive: no remission or flare-ups > steady progression

  • progressive relapsing: steady progression despite relapse/remission (keep getting worse episodes)

fast pain

• A delta fibers

• localization and discrimination of pain

slow pain

• C fibers

• diffuse arousal (protective reactions), affective, and motivational aspects of pain