Rehab 2.4 - Neuromuscular disorders

• motor neuron disease (amyotrophic lateral sclerosis)

• anterior poliomyelitis/polio syndrome

which neuromuscular disease(s) involves the anterior horn cell

PD

PD or MS:

rigidity

MS

PD or MS:

spasticity

PD

PD or MS:

akinesia, bradykinesia, hypokinesia

MS

PD or MS:

ataxia/chorea

MS

PD or MS:

hypotonia

both

PD or MS:

postural instability

both

PD or MS:

dysmetria/discoordination

flexors; extensors

with parkinsons disease you should stretch the (flexors or extensors) and strength the (flexors or extensors)

inflammatory neuropathies (guillain barre)

which neuromuscular disease(s) involves the peripheral nerve

myasthenia gravis

which neuromuscular disease(s) involves the neuromuscular junction

• muscular dystrophies

• inflammatory myopathies dermatomyositis/polymyositis

which neuromuscular disease(s) involves the muscle

Amyotrophic Lateral Sclerosis

_______________ NMD is the degeneration of the motor nerve cells and replaces them with scar tissue (sclerosis)

caucasian men mid to late 50s

what population is most susceptible to ALS (ethnicity, gender, age)

• disease-causing mutations

• clusters

• family history

• gender (male > female)

• age

what are the 5 known risk factors for ALS

• sporadic (most common, encapsulates both limb and bulbar onset)

• familial

what are the two classification of ALS? which is the most common?

glutamate (excites cells to the point of degeneration/death → “neurotoxicity”)

ALS has excessive amounts of the __________ neurotransmitter

• Autonomic nervous system

• basal ganglion

• cerebellum

• frontotemporal

• oculomotor

• sensory systems

ALS is a multi-system health condition that encompasses….

• upper

• corticospinal

• anterior

• 5, 7, 9, 10, 12

ALS is a progressive degeneration of (upper or lower) motor neurons.

This effects:

• ___________ tract of the motor cortex

• (anterior or posterior) horn cells in the spinal cord

• ___________ cranial nuclei in the brainstem

MN is trying to innervate a denervated MS → MN compenstates with reinnervation and becomes enlarged but eventually reinnervation cannot compensate for the rate or degeneration → impairment develops

why does the motor unit enlarge with ALS

foot drop

what would you see with LE issues with ALS

fine motor

what would you see with UE issues with ALS

tongue, lips, voice

what would you see with bulbar issues with ALS

head droop (weak extensors)

what would you see with cervical issues with ALS

distal; proximal (this is the main difference between ALS and muscular dystrophy)

(proximal or distal) weakness is more than (proximal or distal) with ALS

dysfunction

with UMN loss in ALS, is there more significant dysfunction or weakness in the LMN

• spasticity

• hyperreflexia

• clonus

• muscle weakness

what are the 4 UMN signs/sx of ALS

• spastic or flaccid bulbar plasy

• dysarthria

• dysphagia (aspiration precautions)

• sialorrhea (drooling, could be the first bulbar sign)

what are the 4 bulbar signs of ALS

• LMN signs (weakness seen in foot drop/fine motor/tongue/lips/voice/cervical extensors, distal weakness more than prox; atrophy; fasciculations; muscle cramping; fatigue; additional LMN signs like hyporeflexia and hypotonia)

• UMN signs (spasticity, hyperreflexia, clonus, muscle weakness)

• Bulbar signs (spastic or flaccid, dysarthria, dysphagia, sialorrhea)

• respiratory (decrease VC, DOE/orthopnea, hypoxia)

• cognitive (frontotemporal pattern, decrease executive function, limb-onset)

• pseudobulbar affect (poor emotional control, emotional lability)

• pain (secondary due to muscle issues)

what are the 6 signs/sx of ALS

• UMN signs

• LMN signs

• regional progression OR to other regions

in order to diagnosis someone with ALS, what are the 3 “must have” signs

• riluzole (inhibits glutamate and slows progression)

• radicava/radicut (dec free radicals)

what are the two common medications for ALS

4

huntington’s disease is an abnormal expansion of a gene in chromosome ______

• psychological (depression, memory impairment, anxiety, sexual dysfunction)

• motoric problems (slow and clumsy → loss of full motor control)

• chorea (postural control is rapid, jerky, involuntary)

• oculomotor (saccadic latency - delayed eye movement to a target)

• gait impairments (variable velocity, dec stride length, non-rhythmical cadence)

• dystonia

• bradykinesia

• clonus

• ataxia

• dysarthria

• dysphagia

• cognitive impairments (dementia)

what are the signs/sx of huntington’s disease

??????????????

athetosis

____________ is slow, involuntary writhing, twitching movements

hemiballismus

____________ is large amplitude sudden, violend, failing motions of the extremtiies on one side

chorea

_________ is involuntary, rapid, irregular, jerky movements

dystonia

___________ is sustained involuntary contractions

hyperkinesia

huntington’s disease leads to (hypokinesia or hyperkinesia)

• antidopaminergic drugs

• neuroleptic drugs (slows movements)

what are the two common medications for huntington’s disease

• limb weakness

• facial drooping

• trouble swallowing or talking

• difficulty with eye movements/drooping eyelids

• respiratory distress (weakness - vent support)

5 symptoms of acute flaccid myelitis

polio

________ type of acute flaccid myelitis is person to person transmission

west nile

________ type of acute flaccid myelitis is transmitted via animales; ex. mosquito

• flu-like

• in some cases…

  • paresthesia

  • meningitis

  • paralysis

what are the sigs and sx of poliomyelitis

• 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

what are the 5 risk factors for developing post-polio syndrome

25-40

post-polio syndrome occurs in _____-_____% of those patients that were affected by the polio virus 15-40 years after original infection

• aging (loss of MN)

• sprouts constantly undergoing degeneration (leads to giant MN that can’t keep up with ACh demands to initiate contraction)

what are the two possible causes of post-polio syndrome

guillain barre syndrome

_________ is a NMD that causes general neuromuscular paralysis where there is profound weakness in the ascending tracts and descending recovery

ascending

guillain barre syndrome has weakness with the (ascending or descending) tracts

acute

is the onset of GBS acute or insidious

foot drop and/or decrease grip strength

what are the signs of the abrupt onset of GBS

• abrupt onset (foot drop/dec grip)

• autonomic dysfunction

• sensory changes (numbness or tingling)

• decreased deep tendon reflex

• muscle weakness (symmetrical, begins in LE, flaccid ascending weakness, facial and respiratory weakness)

what are the signs and sx of GBS

• acute = 1-3 weeks

  • max paralysis reached

  • ends when no new signs/sx develop or deterioration occurs

• plateau = several days - 2 weeks

• recovery = 6-18 months

  • regeneration and remyelination

  • motor return begins proximally and progresses distally

what are the stages of GBS and it’s associated timefrmae

• plasmapheresis (abnormal antibodies removed)

• steroids

• gamma globulin

• other immunomodulatina agents

• psychological support

what are the 5 ways to medically treat GBS

but at least you know it’s a nerve issue ;)

so many signs and sx

yay!!!

(fyi it’s a neuromuscular junction disorder… something to do with ACh

remember…. shaab said no myasthenias gravis on the test

• prevent weakness

• avoid overuse weakness (ALS)

• supportive care for weakness (orthotics, wheelchair)

what are the 3 PT management strategies for ALS and huntington’s disease

• pain management

• restore or increase ROM

• strength

what are the 3 PT management strategies for post-polio syndrome

• muscles >3/5 → strengthen with non-fatiguing exercises

• muscles <3/5 → rest/splints, range and balance

• discontinue if increase pain, weakness, or fatigue

what are the 3 strengthening principles/guidelines for post-polio syndrome

• fatigue management (could cause relapse)

• gradual progressive program is essential for recovery (ex. exercises throughout the day instead of all at once)

what are the 2 PT management strategies for GBS