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
GBS etiology
Follows non-specific viral syndrome
Campylobacter jejuni bacterial infection
Vaccinations or recent surgery
Lymphoma, lupus, AIDS
GBS clinical presentation
Progressive symmetrical weakness of limbs and face (legs to arms to face) Glove and stocking parasthesias Back pain Diminished/absent reflexes Cranial nerves in 50% of cases
GBS long-term impact
20% severe permanent disability
44% reduces leisure and social activities 3-6 years after
62% ongoing detrimental impact 3-6 years after
Medical management of GBS
Plasma exchange or intravenous immunoglobulin shorten course and severity
IVIg preferred- easier to administer, fewer complications
Oral corticosteroids delay recovery
Intravenous corticosteroids do not affect long-term outcomes
PT management of GBS considerations
Overwork weakness- DOMS 1-5 days post-activity
Closely monitor aerobic training- dysautonomia common
Train fast-twitch muscle fibers
Avoid intense eccentric contractions
Include multi-joint exercises
Train muscles outside cardinal planes for functional movement
Myasthenia gravis definition
Chronic autoimmune neuromuscular disease with varying degrees of skeletal muscle weakness
Fluctuating fatigue and strength is hallmark
Warm weather, infection, stress effects symptoms
Often affects oculomotor, facial, mastication and speech/swallowing muscles
Management of myasthenia gravis
Med- corticosteroids or immunosuppressants
Energy conservation techniques
Sensory symptoms of peripheral neuropathy
Decreased or increased sensation
Neuropathic pain
Motor symptoms of peripheral neuropathy
Weakness
Muscle atrophy
Autonomic symptoms of peripheral neuropathy
Abnormal sweating
Bowel and bladder changes
BP regulation changes
Abnormal heart rate
Trophic symptoms of peripheral neuropathy
Shiny skin
Brittle nails
Peripheral nerve anatomy
Epineurium- outermost
Perineurium- bundles of nerves
Endoneurium- surround axons and myelin sheaths
Overall classification of peripheral neuropathy
Seddon class 1 = neuropraxia Seddon class 2 = axonotmesis Seddon class 3 = neurotmesis
Sunderland- Grades 1-5
Neuropraxia/Seddon class 1/ grade 1
Local conduction block or demyelination
Complete, fast recovery expected
Axonotmesis/Seddon class 2/ grade 2
Disruption of axon with regeneration and full recovery
Axonotmesis/Seddon class 2/grade 3
Disruption of axon and endoneurium with variable recovery
Axonotmesis/Seddon class 2/ grade 4
Disruption of axon, endoneurium, and perineurium with no recovery
Requires surgery
Epineurium intact
Neurotmesis/Seddon class 3/ grade 5
Complete nerve transection
No recovery
Surgery required
Evaluation of peripheral neuropathy
Nerve conduction studies and electromyography
Sensory and strength testing
Trauma causing peripheral neuropathy
Stretch injuries most common- traction force
Lacerations
Compression
Repetitive stress
Systemic disease causing peripheral neuropathy
Diabetes mellitus- most common cause Vitamin deficiencies and alcoholism Vascular damage Cancer/tumor Hormonal imbalances Toxin exposure
Infections/autoimmune disorders causing peripheral neuropathy
AIDP/CIDP
Viral/bacterial infections
HIV
Lyme
Inherited peripheral neuropathy
Charcot-Marie-Tooth disease Affects motor and sensory nerves Frequently associated with leg and foot deformities Progressive condition (slow) Onset from adolescence to mid-adulthood
Primary lateral sclerosis
Corticospinal tract involvement
UMN signs without LMN involvement
Progressive spinal muscular atrophy
LMN involvement only without UMN
