11 Motor Neuron Diseases Flashcards
LMN signs in ALS
Muscle weakness Muscle atrophy Fasciculations Hyporeflexia Hypotonicity
UMN signs in ALS
Spasticity
Clonus
Hyper-reflexia
Pathological reflexes
Other symptoms of ALS
Sialorrhea Dysarthria Dysphagia Chronic fatigue Difficulty breathing Urinary changes Pseudobulbar affect
Clinical presentation of ALS
Asymmetric focal distal weakness of one limb (UE>LE>bulbar)
Contiguous progression to adjacent regions
Preservation of sensory function, sexual, bowel, bladder, oculomotor, cardiovascular, cognitive function
ALS prognosis
Average life expectancy is 3 years from onset
Neg prognosis- initial respiratory or bulbar symptoms
Positive prognosis- younger onset, primary lateral sclerosis or spinal muscle atrophy
ALS diagnosis
World Federation of Neurology EI Escorial criteria
Evidence of both UMN and LMN degeneration
Progressive spread of symptoms within region or to other regions
No electrophysiological or pathological evidence, neuroimaging of other diseases
Electromyography in ALS
Needle or surface electrode to detect motor unit activity
Denervation changes- less voluntary motor units, fibrillations and positive sharp waves (small involuntary muscle contractions), fasciculations (spontaneous muscle contractions)
Re-innervation changes- long duration polyphasic units
Nerve conduction velocity in ALS
Surface electrodes to measure speed and strength of neural signals evoked by electrical stimulation
Usually normal in ALS but may be slightly slowed
Meds for ALS
Riluzole- only med approved to treat
Decreases glutamate release
Prolongs survival 2-9 months
Expensive
Exercise for ALS
Moderate intensity may be beneficial in early stage
High intensity exercise is contraindicated
Poliomyelitis definition
Virus causing inflammation of meninges and anterior horn cells with loss of spinal and bulbar motor neurons
Asymmetric, flaccid paralysis occurs more often in legs than arms
Collateral sprouting in polio
Single motor neuron innervates significantly more muscle fibers than before polio
Polio survivors have few, significantly enlarged motor units doing work of many
Post-polio syndrome presentation
In 25-40% of people, 15-40 years after initial infection
Fatigue, joint and muscle pain, weakness, new respiratory difficulties, decreased endurance, cold intolerance
Steady, slow progression of weakness 1-2%/year
Failure of oversized motor units
Factors that lead to post-polio syndrome
Age >10 years
Prior hospitalization for acute illness
Ventilator dependency
Paralytic involvement of all 4 extremities
Rapid return of functional strength following extensive initial involvement
PT management of post-polio syndrome
Energy conservation techniques
Glossopharyngeal breathing, assistive coughing
Weight loss, use of orthoses and AD to decrease stress on joints
Sub-maximal, short-duration strengthening exercises
Stretching
Cardiovascular conditioning
Guillain-Barre syndrome definition
Autoimmune disorder of peripheral nervous system
Affects motor, sensory, and autonomic nerves
CNS is unaffected
Affects Schwann cells- primary demyelination
Some cases get axonal damage = Wallerian degeneration
