Neuromuscular Disorders

PD deficits and PT tx for them

deficits: bradykinesia/akinesia/hypokinesia, rigidity, festinating gait, freezing of gait, postural instability, dysmetria/dyscoordination

stretch flexors, strengthen extensors, speed of movement, increase amplitude of movement, initiation and termination, balance

MS deficits and PT tx

spasticity, hypotonia, ataxia/chorea, postural instability, dysmetria/discoordination, fatigue

regain control of movement, decrease amplitude of movement, execution, energy conservation, balance

anterior horn cell disorders

motor neuron disease - amyotrophic lateral sclerosis

anterior poliomyelitis/polio syndrome

peripheral nerve disorder

inflammatory neuropathies (Guillain Barre)

neuromuscular junction disorder

myasthenia gravis

muscle disorder

muscular dystrophies

inflammatory myopathies (dermatomysitis/polymositis)

amyotrophic lateral sclerosis (Lou Gehrig’s Disease)

raid progressive disorder with degeneration of motor nerve cells, replaces with scar tissue

onset of ALS? often affects? prognosis?

mid to late 50s

men 60%, mostly caucasian

death within 3-5 years, younger is better prognosis

ALS risk factors

age, family history, male gender, disease causing mutations, clusters

sporadic vs familial ALS

sporadic - most common form, limb onset and bulbar onset (middle aged women)

familial - genetic dominant inheritance, 5-10% of US cases

etiology of ALS

unknown

buildup of free radicals, autoimmune, excessive glutamate, lack of neurotrophic factors

does ALS affect UMN or LMN

both

how does ALS spread?

caudal to rostral, cervical to bulbar

what is the pathophysiology of ALS?

MN degeneration - results in enlarged motor units to compensate for denervated motor neurons

innervation preserves strength/function initially but eventually rate of degeneration exceeds reinnervation

signs and sx ALS (UMN, LMN, Bulbar signs)

LMN: weakness (focal, asymmetrical, foot drop, UE fine motor, Bulbar tongue, lips, voice, head drop, distal weakness more than proximal), atrophy, fasciculation, muscle cramping, fatigue, hyporeflexia

UMN: spasticity - contracture, hyperreflexia, clonus, muscle weakness

Bulbar: spastic or flaccid bulbar plasy, dysarthria - anarthria, dysphagia, sialorrhea

what are other s/sx of ALS?

respiratory, pain, cognitive impairments, emotional control

what sx must one have to have ALS?

LMN signs

UMN signs

regional progression to other regions

medical management for ALS

no cure for ALS

Riluzole - inhibits glutamate, modestly slows progression

Radicava/Radicut - decreases free radicals

sx-based tx - reduce fatigue, ease muscle cramps, control spasticity, reduce excess saliva and phlegm

Huntington’s disease epidemiology

does not skip generation, 50% chance of inheriting gene

15-20 yr lifespan after sx

s/sx of Huntington’s

first psychological sx then motor problems

chorea, oculomotor, gait impairment, athetosis, hemiballismus, dystonia, dysarthria, dysphagia, cognitive impairments

Huntington’s etiology

damage to globus pallidus - loss of indirect pathway leading to hyperkinesia

frontal cortex damage

mx management of Huntington’s

antidopaminergic drugs

neuroleptic drugs

what is acute flaccid myelitis?

not polio, but similar sx

unknown etiology, sx based tx and early dx is important

AFM sx

limb weakness, facial drooping, trouble swallowing or talking, difficulty with eye movements/drooping eyelids, respiratory distress (vent support)

when did scientists id polio virus?

1908

when were polio immunizations released?

1955 - inactive, injectable vaccine

1950s - live attenuated oral vaccine

how is polio transmitted? where does it live? sx?

person to person transmission

throat and intestinal tract

flu-like sx

acute polio hx

fever, stiff neck/back, increased protein in spinal fluid

Bulbar polio - affects swallowing and respiration

what increases the chance of post-polio syndrome

long hospitalization as a child

older than 12 at time of onset

required mech vent

all 4 extremities were involved

rapid recovery after extensive involvement

what are possible causes of post polio syndrome?

sprouts may be constantly undergoing degeneration only to be reinnervated by another MN causing giant MNs. when body is unable to keep up with the Ach demands to initiate contraction

s/sx of PPS

new weakness in affected muscles - atrophy

joint/general aches - overuse syndromes, new respiratory problems such as sleep apnea, weight gain

fatigue

pain

Guillain Barre Syndrome causes

general neuromuscular paralysis

what % of population return to normal?

75%

etiology of GBS

unknown possibly autoimmune

what is GBS pathology?

inflammation and demyelination of nerves in sensory, motor, autonomic and cranial

s/sx of GBS

abrupt onset (begins with foot drop/decreased grip) and has a rapid progression to debility

autonomic dysfunction

sensory changes - numbness/tingling

decreased deep tendon reflexes

muscle weakness - symmetrical, usually begins in LE, flaccid ascending weakness, evolving from hours up to 10 days, facial and respiratory muscle weakness is common

stages of GBS

acute (1-3 weeks) - max paralysis reached, ends when no progression/deterioration

plateau (several days to 2 weeks)

recovery phase (8-18 mo) - regeneration (1 in/mo), motor return begins proximally and progresses distally

mx management of GBS

repeated plasmapheresis, high dose steroids, gamma globulin, immunomodulating agents, pain management

myasthenia gravis affects more?

women in 20-30s

etiology of MG

acquired AI disorder

deficiency of Ach receptors in NMJ - loss up fo 80% of receptors

increased amount of acetylcholinesterase

thymus gland is often abnormal

s/sx of MG

weakness and fatigability of muscles - oculomotor, oropharyngeal, progresses to trunk and limb

sx that fluctuate throughout the day - worse after activity and end of day, better with rest

what is the first sx of MG

oculomotor (ptosis or diplopia)

stages of MG

usually progressive

active - sx fluctuate over short period of time, progressively severe for several years

inactive - strength fluctuations occur but are attributable to fatigue, illness, other factors

burn-out - weakness becomes fixed, severely involved muscles are frequently atrophic

mx management of MG

drugs to deactivate acetylcholinesterase

high dose steroids, gamma globulin, IVIG

thymectomy

plasmapheresis

PT management of general neuro disease

treat sx

maintain function

maintain QoL

use techniques to manage UMN and LMN s/sx, be creative

ther ex - strengthening, stretch, ROM (contracture)

balance - posture, functional, related to pt needs

functional mobility training - bed mob, transfer, gait (minimize energy, saftey)

orthotics

splinting

pain

PT management for ALS and Huntington’s

prevent weakness

avoid overuse weakness (ALS)

supportive care for weakness - orthotics (cervical collars, AFO), WC

PT management for PPS

pain management

restore or increase ROM

strengthening - muscles <3/5 place on rest, range and brace; muscles >3/5 can be strengthened with non-fatiguing exercises

stop if pain, weakness, fatigue

set firm limits on numbe rof reps and frequency of sessions

PT management of GBS

fatigue - may cause relapse so be careful in endurance training

gradual progressive program is essential to recovery

PT management for MG

most live normal live, rarely referred to PT

POC - light activities, short duration, few reps