Neuromuscular Health Conditions Flashcards
What is the difference between neuropathy and myopathy?
Neuropathy- soma/axons are involved
- there is distal involvment (more symmetrical than asymmetric)
- stocking-glove pattern
- longest nerves affected first
Myopathy- end plate/mm involved
- proximal symmetrical involvement
- difficulty arising
- overhead activities
What would the difference be between an LMN and an UMN as it pertains to:
Stiffness Tone Reflexes Atrophy Fasciculations sensory disturbances
LMN will have reduced stiffness, UMN is very stiff
LMN decreases tone, UMN increases tone
LMN decreases reflexes, UMN increases reflex
LMN has profound atrophy, UMN has minimal atrophy
LMN has fasciculations, UMN does not
Sensory disturbances are equal for both
What nueromuscular conditions affect the motor neuron (LMN-Anterior horn cell)?
Progressive Spinal Muscular Atrophy
Post-Polio Syndrome
Amyotrophic Lateral Sclerosis
What structure does Guillian Barre and Charco Marie Tooth affect?
Peripheral nerves
What structure does Myasthenia gravis affect?
Motor End Plate
What goes into forming a prognosis for a NM disease?
- making long and short term goals
- describing natural progression of the condition (onset period, progression rate, recovery potential)
- identify contextual factors that would help and/or harm
finally based on these factors you form a prognosis which is the likely time frame for achieving the goals
What does PT intervention for NM conditions depend on?
Depends on the stage of the disease
What PT interventions are applicable for when you want to optimize function for a patient with a NM condition?
Education, encouraging activity and participation, strengthening (mod intensity), aerobic training (mod intensity), flexibility
What PT interventions are applicable for when you want to adapt a patient to their limitations due to a NM condition?
Prevent overuse, prevent respiratory dysfunction (breathing techniques and coughing), prescribe assistive technology (orthotics, assistive devices, w/c) to support participation
What PT interventions are applicable for when you want to maximize quality of life for a patient with a NM condition?
Addressing respiratory dysfunction (postural drainage and assistive coughing), positioning, supporting and training the caregiver
What is Spinal Muscular Atrophy?
What causes it?
Premature death of alpha motor neurons in spinal cord and brainstem which causes weakness and wasting of voluntary muscles (often more severe in legs than arms)
It is a genetic disorder due to a 5q gene deletion
What is the onset, progression, movement system, and prognosis for type 1 spinal muscular atrophy?
Type 1 is acute infantile (werdnig-hoffman)
onset is from birth to 6 months
progression is rapid
common movement system is patient never sit, poor head control/suck/swallow/cry
prognosis is usually fatal within 2 yrs
What is the onset, progression, movement system, and prognosis for type 2 spinal muscular atrophy?
Type 2 is chronic infantile (Dubowitz Disease)
Onset is from 6 months to 18 months
Progression is slower
movement system: delayed motor milestones and is able to sit but not stand or walk
Prognosis is variable
What is the onset, progression, movement system, and prognosis for type 3 spinal muscular atrophy?
Type 3 is chronic juvenile
Onset is after 18 months
Progression is slower
Movement System: most can stand and walk, but have trouble going up/down stairs and can lose ability to walk later on, in w/c develop scoliosis; bulbar dysfunction later in life
Prognosis: typical life expectancy
What is the onset, progression, movement system, and prognosis for type 4 spinal muscular atrophy?
Type 4 happens to adults
onset is in the 30s
Patients lose ability to walk
Will have typical life expectancy
What are the conditions for a medical diagnosis for spinal muscular atrophy?
What are the common impairments in this condition?
- genetic testing (5q deletion)
- EMG (reduced amplitude, fibrillations)
- Muscle biopsy (to detect atrophy)
Impairments:
- severe proximal muscle weakness
- flaccidity
- bulbar dysfunction in more severe cases, as in type 1 or in late progression of type 2 or 3
What are the intervention guidelines for spinal muscular atrophy?
- strength training of proximal muscle groups: shoulder flexors/extensors, elbow flexors/extensors, hip flexors, extensors, and knee extensors
- 2 sets of 15 reps for each muscle group w/ 5 minute rest between sets
- initial intensity should emphasize biomechanics using body weight as resistance and progressed by adding light resistance
- monitor w/ physical exertion (RPE); goal is under “somewhat hard” to “hard”
What is post-polio syndrome?
How does it distribute?
How does a patient recover from polio?
Late manifestation of acute poliomyelitis that degenerates larger motor neuron pools and slowly progresses new muscles weakness in previously affected areas
usually distrbutes asymmetrically and can be distal, proximal or patchy
Sprouting of spared motor units
What needs to be present for a medical diagnosis of post-polio syndrome?
- prior paralytic poliomyelitis
- EMG showing polyphasic motor unit potentials and fibrillations
- muscle biopsy showing muscle fiber atrophy
What is a common movement system diagnosis for post-polio syndrome patients?
difficulty walking and standing secondary to fatigue, pain, or weakness
What are the intervention guidelines for post-polio syndrome?
- lifestyle modification
- energy conservation techniques
- avoid overuse of muscle groups
- non-fatiguing muscle strengthening program can result in strength gains
- be aware of poor body mechanics and malalignment and incorporate postural exercises
- monitor fatigue and pain
What is amyotrophic lateral sclerosis?
an acquired disease that leads to degeneration of the cortical spinal tract (UMN and LMN) and primarily affects anterior horn cells and motor cranial nuclei
presents as progressive asymmetrical weakness starting distally and moving proximally while sensory function stays intact
What does the physical manifestation of ALS look like?
- anterior spinal nerve roots are shrunken/atrophied
- loss of cell bodies
- loss of corticopinal tracts
What is the typical onset of ALS?
How fatal is ALS?
adulthood (median age is 50y.o) with rapid progression of probable and definite ALS
50% fatal in 3.5 years
What are the UMN and LMN signs of Suspected ALS?
What is a Probable ALS classification?
What is a Definite ALS classification?
Suspected ALS:
-LMN or UMN signs only in 1 or more regions
Probable ALS
-LMN and UMN in 2 regions
Definite ALS
-LMN and UMN in 3 regions
What is the prognosis for ALS?
What medications can be used for ALs?
Devastating, rapidly progressive neurodegeneration of AHC, LMN, and UMN with highly predictable clinical course
- patients rarely survive past 3.5-4 yrs after diagnosis
- 50% survival is around 1 year for bulbar onset
Medications
- Riluzole (prolong survival by 2-3 months)
- New agents: new meds, stem cell clinical trials
What are the intervention guidelines for ALS?
-old controversial idea: vigorous activity is a risk factor for motor neuron loss however animal tests have not found this correlation
there is a positive effect of moderate exercises in individuals with ALS
monitor post exercise fatigue to monitor intensity
What is Guillain-Barre’ Syndrome (GBS)?
an acute inflammatory demyelinating polynueropathy with an onset in adulthood and is initially rapidly progressive (within 24 hours to 4 weeks) but recovery occurs
What are the clinical features for the GBS variant:
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
- Demyelinating Polynueropathy (myelin more than axons)
- Sensorimotor GBS (progressive areflexic weakness, mild sensory changes, autonomic dysfunction, some CN deficits)
What are the clinical features for the GBS variant:
Miller Fisher Syndrome (MFS)
Normal CFV
Sensory Changes
Opthalmoplegia, ataxia, areflexia
What are the clinical features for the GBS variant:
Acute Motor Axonal Neuropathy (AMAN)
- Pure motor GBS (motor involvement only)
- Axonal Polynueropathy (motor axon involvement-not mylein)
- CN Rarely affected
What are the clinical features for the GBS variant:
Acute Sensorimotor Axonal Nueropathy (AMSAN)
- Severe variant of AMAN but with sensory deficits
- Axonal Polynueropathy (sensory and motor axons)
- Primarily is Asia, Central and South America
What is the pathology of GBS?
What does recovery look like?
auto immune response usually happens within 2 weeks after infections
- antibodies made by the body to get rid of c.jejuni virus but the c.jejuni virus looks a lot like myelin and so the body attacks myelin and schwann cells
- this affects nerve roots and peripheral nerves causing axonal degeneration and demyelination
Recovery happens proximally to distally-remyelination is faster than regeneration
What are the 3 phase of GBS?
Acute (progressive, ascending, symmetrical weakness and sensory loss)
Plateau Phase
Recovery Phase
What are the classic features of GBS?
LE weakness ) UE wekaness > (these three are the most common) Areflexia ) paresthesia sensory loss pain respiratory failure sphincter involvement (rare)
What is needed for a medical diagnosis of GBS?
- lumbar puncture to assess presence of elevated albumin
- NCV to asses delays in latency suggesting demyelination
- EMG to see if there is axonal degeneration
What is the medical management for GBS?
- admit to ICU
- respiratory changes treated w/ tracheostomy and vent
- psycho-emotional support
- immunomodulation (shortens disease duration)
What is the prognosis for GBS?
When does recovery mainly occur?
recovery takes months or years but 15% of patients will see full recovery
-most will have some residual weakness (67%) and only a few (8%) will die in the acute phase
recovery mainly occurs during first year post onset but here will still be detectable motor and sensory impairments at the 2 years mark in 50% of patients
What are the intervention guidelines for GBS?
- avoid fatigue
- after plateau phase and recovery has begone you should add short periods of non-fatiguing exercise and increase activity if patient improves
- exercise should promote functional activities such as gait and balance
What is Charcot Marie Tooth?
What is the common onset?
Hereditary motor and sensory neuropathy impacting myelin or axons with a symmetrical distal “stocking glove” distribution of weakness and sensory loss
onset is early childhood and is initially rapidly progressive but recovery occurs
What are the common impairments associated with Charcot Marie Tooth?
- Paresthesia (cutaneous and proprioceptive loss)
- Muscle Weakness (symmetrical w/ distal involvement, weak TA, weak peroneii
- Pes Cavus (elevation of medial longitudinal arch, progressing to clawing of toes)
What are the intervention guidelines for Charcot Marie Tooth?
Exercise-ROM, strengthening, blance training, task orientated
Orthoses-AFO
Gait: assistive device may be necessary
Adaptive home equipment
What is myasthenia gravis?
an autoimmune disorder where antibodies bind to the Ach receptors that decreases number and defective functioning of Ach receptor sites at neuromuscular junctions
It is a painless disorder of strength occurring during sustained or repetitive muscle contraction: loss of muscle power occurs in association w/ continuous effort
How common is myasthenia gravis?
relatively uncommon and effects women more than men (2:1)
however this switches as the population ages once patients are in their 60-70s males become more dominant
What are the clinical signs of myasthenia gravis?
What is treatment like for this condition?
ptosis, diplopia, facial weakness, dysarthria, dysphagia, neck mm weakness, weak grip
mestinon (cholinesterase inhibitor), corticosteroids, thymectomy
What is the prognosis for myasthenia gravis?
with treatment there can be significant improvement to the muscle weakness
however sometimes the severe weakness can cause respiratory failure which requires immediate medical care
What are the intervention guidelines for myasthenia gravis?
PT typically not involved since muscle weakness gets worse as the muscles are used repeatedly
however stable patients with MG may exercise using the following guidelines:
- exercise at your best time of the day
- exercise at peak dose of meds
- exercise large prixmal muscles for short periods of time
- Do NOT exceed moderate intensity
- postural exercises
- breathing exercises
