11 Motor Neuron Diseases Flashcards

1
Q

LMN signs in ALS

A
Muscle weakness
Muscle atrophy
Fasciculations
Hyporeflexia
Hypotonicity
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2
Q

UMN signs in ALS

A

Spasticity
Clonus
Hyper-reflexia
Pathological reflexes

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3
Q

Other symptoms of ALS

A
Sialorrhea
Dysarthria
Dysphagia
Chronic fatigue
Difficulty breathing
Urinary changes
Pseudobulbar affect
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4
Q

Clinical presentation of ALS

A

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

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5
Q

ALS prognosis

A

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

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6
Q

ALS diagnosis

A

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

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7
Q

Electromyography in ALS

A

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

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8
Q

Nerve conduction velocity in ALS

A

Surface electrodes to measure speed and strength of neural signals evoked by electrical stimulation
Usually normal in ALS but may be slightly slowed

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9
Q

Meds for ALS

A

Riluzole- only med approved to treat
Decreases glutamate release
Prolongs survival 2-9 months
Expensive

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10
Q

Exercise for ALS

A

Moderate intensity may be beneficial in early stage

High intensity exercise is contraindicated

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11
Q

Poliomyelitis definition

A

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

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12
Q

Collateral sprouting in polio

A

Single motor neuron innervates significantly more muscle fibers than before polio
Polio survivors have few, significantly enlarged motor units doing work of many

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13
Q

Post-polio syndrome presentation

A

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

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14
Q

Factors that lead to post-polio syndrome

A

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

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15
Q

PT management of post-polio syndrome

A

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

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16
Q

Guillain-Barre syndrome definition

A

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

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17
Q

GBS etiology

A

Follows non-specific viral syndrome
Campylobacter jejuni bacterial infection
Vaccinations or recent surgery
Lymphoma, lupus, AIDS

18
Q

GBS clinical presentation

A
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
19
Q

GBS long-term impact

A

20% severe permanent disability
44% reduces leisure and social activities 3-6 years after
62% ongoing detrimental impact 3-6 years after

20
Q

Medical management of GBS

A

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

21
Q

PT management of GBS considerations

A

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

22
Q

Myasthenia gravis definition

A

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

23
Q

Management of myasthenia gravis

A

Med- corticosteroids or immunosuppressants

Energy conservation techniques

24
Q

Sensory symptoms of peripheral neuropathy

A

Decreased or increased sensation

Neuropathic pain

25
Motor symptoms of peripheral neuropathy
Weakness | Muscle atrophy
26
Autonomic symptoms of peripheral neuropathy
Abnormal sweating Bowel and bladder changes BP regulation changes Abnormal heart rate
27
Trophic symptoms of peripheral neuropathy
Shiny skin | Brittle nails
28
Peripheral nerve anatomy
Epineurium- outermost Perineurium- bundles of nerves Endoneurium- surround axons and myelin sheaths
29
Overall classification of peripheral neuropathy
``` Seddon class 1 = neuropraxia Seddon class 2 = axonotmesis Seddon class 3 = neurotmesis ``` Sunderland- Grades 1-5
30
Neuropraxia/Seddon class 1/ grade 1
Local conduction block or demyelination | Complete, fast recovery expected
31
Axonotmesis/Seddon class 2/ grade 2
Disruption of axon with regeneration and full recovery
32
Axonotmesis/Seddon class 2/grade 3
Disruption of axon and endoneurium with variable recovery
33
Axonotmesis/Seddon class 2/ grade 4
Disruption of axon, endoneurium, and perineurium with no recovery Requires surgery Epineurium intact
34
Neurotmesis/Seddon class 3/ grade 5
Complete nerve transection No recovery Surgery required
35
Evaluation of peripheral neuropathy
Nerve conduction studies and electromyography | Sensory and strength testing
36
Trauma causing peripheral neuropathy
Stretch injuries most common- traction force Lacerations Compression Repetitive stress
37
Systemic disease causing peripheral neuropathy
``` Diabetes mellitus- most common cause Vitamin deficiencies and alcoholism Vascular damage Cancer/tumor Hormonal imbalances Toxin exposure ```
38
Infections/autoimmune disorders causing peripheral neuropathy
AIDP/CIDP Viral/bacterial infections HIV Lyme
39
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 ```
40
Primary lateral sclerosis
Corticospinal tract involvement | UMN signs without LMN involvement
41
Progressive spinal muscular atrophy
LMN involvement only without UMN