Rehab II - Neuromuscular Disorders (from Jillian)

this disease is characterized by rigidity, postural instability, festinating & freezing of gait, and bradykinesia

PD

this disease is characterized by spasticity, hypotonia, ataxia, dysmetria, and fatigue

MS

to treat this disease, we want to focus on decreasing amplitude of movement, execution, balance, and energy conservation

MS

to treat this disease, we want to focus on increasing speed & amplitude of movements as well as the initiaion and termination of movements

PD

2 Progressive Neuromuscular Disorders

PD & MS

what part of nervous system does anterior poliomyelitis/polio affect?

anterior horn cell

what part of nervous system does guilliain barre and other inflammatory neuropathies affect?

peripheral nerve

what part of nervous system does inflammatory myopathies such as dermatomysitis/polymyositis affect?

muscle

interventions to focus on with PD patients

ü Stretch (Flexors)

ü Strength (Extensors)

ü Speed of movement

ü Incr amplitude of movement

ü Initiation & Termination

ü Balance

for patients with PD, we want to stretch ___ and strengthen ___

flexors // extensors

interventions to focus on with MS patients

ü Regain control of movement

ü Decr amplitude of movement

ü Execution

ü Energy Conservation

ü Balance

rapidly progressive disorder in which there is degeneration of the motor nerve cells and they are replaced with scar tissue (sclerosis)

Amyotrophic Lateral Sclerosis (ALS) / lou gehrig's

with ALS, where does degeneraiton of the nerve start?

anterior horn cell

onset age & demographic of ALS

mid to late 50's (but can effect people at any age) caucasian men

what do most patients with ALS die of?

respiratory failure

which type of ALS has a better prognosis: limb-onset or bulbar (CN involvement)?

limb-onset

T/F: the longer time between symptom onset and diagnosis means better prognosis for ALS

TRUE

what are 2 factors that make prognosis for ALS worse?

-dementia/cognitive impairments

-psychological distress

most common classification of ALS is ___

sporadic (includes both limb-onset and bulbar) = no genetic component

which is more common in women: limb or bulbar onset ALS?

bulbar-onset

is the cause of ALS known?

NO, but theory that there is build up of free radicals, excessive glutamate, or lack of neurotrophic factors (CNTF)

does ALS have a genetic component?

yes - 5-10% of cases

etiology of ALS is unknown, but theory is that there is a buildup of ____ due to a defective gene that usually destroys them

free radicals

ALS may be caused by excessive ___ leading to excitotoxicity and neurodegeneration

glutamate

this disease has an unknown etiology but may be autoimmune, may be caused by buildup of free radicals, excessive glutamate, or lack of neurotrophic factors

ALS

In ALS, what preserves strength/function initially but then results in enlarged motor units?

Healthy MN innervates denervated MS -- reinnervation can compensate for degeneration until ~50% of the motor unit is lost and then impairment develops

multi-system health condition wherein there is a progressive degeneration of UMNs in motor cortex, spinal cord, and brainstem

ALS

T/F: spread in ALS occurs locally within a region (Lumbosacral SC) before moving to other regions (caudal --> rostral; cervical --> bulbar)

TRUE

signs and symtoms of LMN in ALS

-weakness

-atrophy

-fasciculations

-muscle cramping

-fatigue

foot drop & head droop are ___ weaknesses in ALS

LMN

what signs of weakness (LMN) would we see in ALS?

-focal/asymmetrical

-foot drop

-head droop

atrophy, fasciculations, muscle cramping, and fatigue are all ___ symptoms of ALS

LMN

which is weaker in ALS LMN: distal or proximal?

distal > proximal (BIG DIFFERENCE BTWEEN ALS AND MUSCULAR DYSTROPHY)

T/F: ALS has both UMN & LMN symptoms

TRUE (but LMN worse)

what are bulbar LMN weakness in ALS?

weakness in tongue, lips, voice

in ALS, more significant dysfunction than weakness caused by (UMN/LMN) loss

LMN!

UMN signs in ALS

-spasticity - contracture

-Hyperreflexia

-Clonus

-Muscle weakness

spasticity, clonus, hyperreflexia are ___ signs and symptoms of ALS

UMN

what is one of the first signs of bulbar onset ALS?

sialorrhea (drooling)

why might ALS patients be on aspiration precautions?

dysphagia common symptom

respiratory impairments of ALS due to loss of muscle strength

-Decr VC

-Fatigue

-DOE/Orthopnea

-Hypoxia (causes a headache in morning)

cognitive impairments of frontotemporal pattern and decreased executive function are mroe common in ___-onset ALS

LIMB

ALS patients have psuedobulbar affect - what does this mean?

poor emotional control or no emotions shown

how do we diagnose ALS?

rule out everything else ; must have LMN & UMN signs

studies to diagnose ALS

uEMG

uNCV

uMuscle & Nerve biopsies

uNeuro imaging

is there a cure for ALS?

NO, just disease-modifying agents

what may disease-modifying agents for ALS focus on?

-inhibiting glutamate

-decrease free radicals

___ is used to treat ALS by inhibiting glutamate and modestly slows progression by increasing 2-3 mos survival

riluzole

___ is used to treat ALS by decreasing free radicals; more effective early

radicava

for medical management of ALS, what symtpoms are we trying to manage?

-reduce fatigue

-ease muscle cramps

-control spasticity

-reduce excess saliva

disease that is due to an abnormal expansion of a gene in chromosome 4; gene does not skip a generation

Huntington's Disease

age of onset for Huntington's Disease

30s-40s; juvenile form possible

which typically has a longer lifespan: ALS or Huntington's Disease?

huntingtons (15-20 years)

Huntington's Disease starts with ___ symptoms such as...

psychological: depression, anxiety, memory impairment, sexual dysfunction

Huntington's Disease develops into ___ problems such as....

motoric: slow and clumsy

what disease is defined by chorea: rapid, jerky, involuntary movements?

Huntington's Disease

Saccadic latency is an occulomotor problem in what disease?

huntingtons - delayed eye movement to a target

gait impariements in Huntington's Disease

-variable veolcity

-decreased stride length

-non-rhythmical cadence

slow, involuntary writhing, twitching movements, common in HD & CP

athetosis

large amplitude, sudden, violent, flailing motions of the extremities on one side, common in HD

Hemiballismus

involuntary, rapid, irregular, jerky movements, common in HD

chorea

sustained inoluntary contractions including abnormal postures/twisting motions, common in HD

dystonia

signs and symptoms of this disease include bradykinesia, dystonia, clonus, ataxia, dysarthria, dysphagia, and cognitive impairments

HD

HD is casued by damage to what structure in the brain?

globus pallidus -- loss of indirect pathway causing hyperkinesia because there is no "brake"

medical management for HD (2)

-antidopaminergic drugs

-neuroleptic drugs (slows down movements)

which has a more genetic component: ALS or HD?

HD (50% chance of getting it)

this disease affects the body's nervous system (SC) causing paralysis spread either person to person (polio) or via west nile virus

Acute flaccid myelitis (AFM)

T/F: in AFM, we focus on symptom-based treatment wherein early diagnosis is important

TRUE

symptoms of Acute flaccid myelitis (AFM)/polio

-Limb weakness

-Facial drooping

-Trouble swallowing or talking

-Difficulty with eye movements/Drooping eyelids

-Respiratory distress (weakness)

what pathology presents with limb weakness, facial drooping, trouble swallowing or talking, difficlty with eye movements, and respiratory distress?

Acute flaccid myelitis (AFM)

T/F: patents with Acute flaccid myelitis (AFM) may need vent support

TRUE - respirtatory weakness

this disease lives in throat and the intestinal tract and is spread by person to person transmission; flu-like symptoms

poliomyelitis

a small portion of people infected with ___ will have paresthesia, meningitis, or paralysis

poliomyelitis

this disease occcurs in approximately 25%-40% of those patients that were affected by the polio virus 15-40 years after original infection

Post-Polio Syndrome

increased chance of Post-Polio Syndrome if...

-Long hospitalization as a child

-Older than 12 at the time of onset

-Required mechanical ventilation

-All four extremities were involved

-Rapid recovery after extensive involvement

if you were older than 12 when you got polio virus, required mechanical ventilation, all 4 extremeties were involved, and you had a rapid recovery, you are (more/less) likely to get post-polio syndrome

MORE

possible causes of post-polio syndrome (2)

-normal aging process (loss of motor units)

-degeneration (giant motor neurons)

signs & symptoms of post-polio

-weakness & atrophy in affected muscles

-joint/general aches

-respiratory issues

-weight gain

-fatigue

-pain

atrophy, joint aches, respirtaory problems such as sleep apnea, weight gain, physical & mental fatigue, and pain are symptoms of

post-polio syndrome

Post-Polio Personality

-Type A

-Feelings of invincibility/independence

-Denial/anger facing disease previously overcome

-Reluctance to show new weakness

-Distrust of medical centers/professionals

-Hatred of braces/assistive devices

this disease causes general neuromuscular paralysis characterized by ascending, profound weakness and descending recovery

Guillain Barre Syndrome (AIDP)

T/F: 75% of guillan barre syndrome patients return to normal

TRUE - others have complaints of disabling weakness or sensory symptoms 2 years after onset

do we know the cause of guillan barre?

NO - theory is autoimmune

T/F: 2/3 of patients have a history fever with a respiratory or GI issue 1-3 weeks prior to the onset guillian barre

TRUE

pathology of guillan barre includes...

inflammation and demyelination

what does guillan barre usually start with?

foot drop or decreased grip but rapidly progresses to debility

muscle weakness of guillan barre

-Symmetrical

-Usually begins in the lower extremities

-Flaccid ascending weakness

-Evolving from hours up to 10 days

-Facial and respiratory muscle weakness is common (may need vent)

is ventillation necessary in GBS patients?

sometimes - due to facial and respiratory muscle weakness

what disease has autnomic dysfunction, sensory changes such as numbness/tingling, decreased deep tendon reflexes, symetrical flaccid ascending muscle weakness?

GBS

in this stage of guillan barre, maximum paralysis is reached and it ends when no new signs/symptoms develop or deterioration occurs

acute (1-3) weeks

in this stage of guillan barre, there is regeneration and remyelination and motor return begins proximally and progresses distally

recovery phase (6-18 mos)

in GBS, motor return begins ___ and progresses ____

proximally // distally

stages of guillan barre

-acute (1-3 weeks)

-plateau (days-weeks)

-recovery (6-18 months)

does GBS have sensory symptoms?

yes, numbness/tingling and decr deep tendon reflexes

for what disease would medical manamgenet include repeated plasmapheresis wherin abnormal antibodues are removed from the blood?

GBS

PT Management: ALS & Huntington's Disease

-Prevent weakness

-Avoid overuse weakness (ALS)

-Supportive care for weakness (orthotics, wheelchairs)

speech therapy is essential for these two diseases

ALS & HD

PT Management: post-polio syndrome

-Pain management

-Restore or increase ROM

PT management: strength principles for post-polio syndrome

-muscles less than 3/5, place them on rest (splints), range and brace

-muscles greater than 3/5, can be strengthened with non-fatiguing exercises

for muscles less than 3/5 in post polio syndrome, what do we do?

rest (splints), range and brace