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NCS Exam > Motor Neuron Diseases > Flashcards

Motor Neuron Diseases Flashcards

1
Q

how many people who had polio will develop post polio syndrome?

A

25-40%

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2
Q

What is the most mild type of peripheral nerve damage? what class/grade is that?

A

Neuropraxia (class 1 and grade 1): local conduction block or demyelination. Small part of the nerve. Complete and fast recovery is expected

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3
Q

What does Myasthenia Gravis affect?

A
  • Oculomotor
  • Facial
  • Mastication
  • Speech and swallowing muscles
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4
Q

what is post polio syndrome

A

new onset of symptoms occurring 15-40 years post infection

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5
Q

what is the clinical presentation of polio?

A

asymmetrical, flaccid paralysis, usually LE > UE

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6
Q

what is the life expectancy of ALS?

A

the average is 3 years from onset. 20% survive 5 years, 10% survive 10 years, and 5% survive 20 years

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7
Q

who is most affected by GBS?

A

men > women

ages 30-50 yr old

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8
Q

what are some autonomic symptoms that can occur with peripheral neuropathy

A
  • abnormal sweating
  • changes in BP regulation (OH)
  • abnormal HR
  • bowel and bladder changes
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9
Q

What are the layers of connective tissue surrounding the peripheral spinal nerve?

A

-outer layer - in (E-P-E): epineurium, perineurium, endoneurium.

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10
Q

Better vs worse prognostic factors for ALS?

A 
Better= younger onset and having primary lateral sclerosis or spinal muscle atrophy vs classic ALS
Worse = initial symptoms involving the bulbar muscles or respiratory system
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11
Q

why do they think post polio syndrome occurs?

A

theorized that it is due to failure of the oversized motor units that developed due to collateral sprouting

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12
Q

Does GBS affect UMN or LMN?

A

LMN. CNS is unaffected.

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13
Q

what is the mechanism of GBS?

A

it is an autoimmune disorder of the PNS. the inflammatory process affects the schwann cells causing de-myelination of the axon. Axon can remain intact or in some cases axonal damage occurs

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14
Q

Physical therapy management of persons with post polio syndrome (interventions, focus of treatment, etc)

A

energy conservation technique, submaximal short duration strength training, cardiovascular conditioning, stretching, decrease stress on joints, respiratory techniques,
Education: fatigue and over work weakness are common, need to educate on submax exercise and energy conservation

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15
Q

Exercise intensity recommendations for people with ALS

A

high intensity exercise is contra-indicated in all stages of ALS. Moderate intensity exercise may be beneficial in EARLY stages only.

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16
Q

use of AD and DME for persons with ADL

A

manual w/c is often not recommended/ordered due to progressive nature of disease. Power w/c is order early and can be adapted

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17
Q

what is the clinical presentation of GBS?

A
  • Progressive symmetrical weakness of the limbs and face. Legs to arm, to face
  • Paresthesias in the hands and feet in a glove and stocking distribution
  • Back pain
  • Diminished/absent DTR
  • CN are affected in ~50% of all cases
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18
Q

What does the progression of ALS look like?

A

it has a “contiguous Progression” meaning it spreads to anatomically adjacent regions

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19
Q

what is the progression like in post polio syndrome?

A

slow and steady progression: 1-2% increase in weakness per year

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20
Q

what factors increase the likelihood of developing post polio syndrome?

A

> 10 years old, requiring hospitalization for the illness, paralytic involvement of all four limbs, vent dependent, rapid return of function following extensive involvement

21
Q

What medication is use to treat ALS? how does it work? pros and cons?

A

Riluzole is the only drug approved by the FDA to treat ALS. It decreases the release of glutamate. It increases life expectancy by 2-9 months. It’s very expensive

22
Q

what are the rehab implications for people with myasthenia gravis?

A
  • energy conservation

- risk for falls with decreased bone density

23
Q

PT/exercise considerations for patients with GBS?

A
  • Watch for overwork weakness due to DOMS 1-5 days post activity and Reduction in maximum force production that gradually recovers
  • Train fast twitch muscle contractions
  • Monitor vitals during aerobic exercise due to autonomic dysfunction
24
Q

type of inherited peripheral neuropathy and symptoms?

A

Charcot marie tooth disease - associated with leg weakness and foot deformities. slow progressive condition affecting motor and sensory nerves. Onset is adolescence to mid adult.

25
Q

What is the most common adult onset motor neuron disease?

A

ALS

26
Q

What functions and motor neurons are typically NOT affected by ALS

A

typically the following are spared: sensory, bowel and bladder, cognition, sexual function, cardiovascular, oculomotor

27
Q

Axonotmesis: class/grades

A

Class 2. Grade 2 = axonal damage. Full recovery. Grade 3 = axonal and endoneurium. Varied recovery. Grade 4= axonal, endoneurium, and perineurium, no recovery expected, needs surgery

28
Q

medical management of GBS- what is recommended? What is NOT recommended?

A
  1. ) (IVIg) intravenous immunoglobulin therapy is recommended over plasma exchange therapy because its easier to administer and is associated with fewer complications.
  2. ) Corticosteriods have not shown to be affective. Actually delays recovery
29
Q

Does ALS affect UMN or LMN?

A

Both. It affects both UMN and LMN

30
Q

Neurotmesis

A

Class 3 and Grade 5: complete transection of nerve including epineurium. No recovery, Needs surgery

31
Q

what type of disease is GBS

A

an autoimmune disorder of the PNS

32
Q

what is the clinical presentation of ALS?

A

you will see UMN signs (spasticity, clonus, hyper reflexia, and pathological reflexes) and LMN signs (muscle weakness, muscle atrophy, fasciculations, hypo reflexia, hypotonicity). Other symptoms: sialorrhea, dysarthria, dysphagia, pseudobulbar affect, chronic fatigue, difficulties breathing, urinary changes.

33
Q

What is the cause of ALS?

A

the cause is unknown, but it is associated with several genetic mutations. 90% is sporadic and 10% is hereditary, but no difference in clinical presentation

34
Q

what is the medical management for myasthenia gravis?

A

corticosteroids or immunosuppressants

35
Q

what is the initial presentation of ALS?

A

ALS initially presents as asymmetrical, focal, distal weakness of one limb. Most commonly the UE > LE > bulbar

36
Q

What criteria is used to Dx ALS?

A

The world Federation of Neurology El Escorial Criteria. Presence of UMN signs, LMNS, and spread of sx within a region or to other regions. Absence of other disease processes. Use of electrodiagnostic testing

37
Q

what are the two systems for classifying peripheral nerve damage?

A

seddon (class 1-2) and sunderland (Grades 1-5)

38
Q

what causes polio?

A

polio is caused by the virus poliomyelitis that causes inflammation to the meninges and anterior horn cell with loss of spinal and bulbar motor neurons

39
Q

What causes GBS?

A
  • following non specific viral syndrome (IE mild upper respiratiory infection)
  • also associated with viral infections, Campylobacter jejuni bacterial infection, vaccinations or recent surgery, lymphoma, systemic lupus, AIDS
40
Q

who is most often affected by ASL? Age of onset?

A

mostly affects white males and non-hispanics. Typical age of onset is 55-75 but can occur form late teens to very old

41
Q

what are symptoms of post polio syndrome

A

fatigue, new respiratory difficulties, cold intolerance, weakness, joint and muscle pain, decreased endurance

42
Q

who does myasthenia gravis usually affect?

A

younger females and older males

43
Q

what are some trophic changes that can occur with peripheral neuropathy

A

shiny skin and brittle nails

44
Q

how fast/slow does GBS progress?

A

onset to peak disability ranges from hours to week

45
Q

How has peripheral neuropathy affect muscles?

A

causes weakness due to denervation of muscles and disuse atrophy

46
Q

types of acquired peripheral nerve neuropathy

A
  1. Trauma 2. systemic disease 3. infection/autoimmune.
  2. ) Trauma (traction, laceration, compression, repetition), 2.) Systemic disease (DM, vit decencies, alcohol, vascular damage, cancer/tumor, hormonal imbalances, toxins) 3.) Infections / autoimmune disorders (HIV, Lyme disease, viral/bacterial infections, CIDP)
47
Q

What do you expect to see on electrodiagnostic testing with ALS

A

EMG: reduction in the number of motor units, fibrillations and fasciculations.
Nerve conduction tests: usually normal but might be slightly slowed

48
Q

what is the hallmark sign of Myasthenia Gravis?

A

fluctuating fatigue and strength. Weakness/fatigue increase during activity and improves with rest. Environmental factors can also affect symptoms

NCS Exam (13 decks)

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