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NCS > Motor Neuron Diseases > Flashcards

Motor Neuron Diseases Flashcards

1
Q

What is the most common adult onset motor neuron disease?

A

ALS

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2
Q

Does ALS affect UMN or LMN?

A

BOTH - it affects both UMN and LMN

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3
Q

Who is most often affected by ALS? Age of onset?

A
  • White males and non-hispanics

- Typical age of onset is 55-75

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4
Q

What is the initial presentation of ALS?

A
  • Asymmetrical, focal, distal weakness of one limb

* *Most commonly the UE > LE > bulbar

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5
Q

What does the progression of ALS look like?

A

It has a “contiguous progression” meaning it spreads to anatomically adjacent regions
**RUE&raquo_space; LUE; RLE&raquo_space; LLE

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6
Q

What is the clinical presentation of ALS?

A
  • UMN signs (spasticity, clonus, hyper reflexia, and pathological reflexes)
  • LMN signs (muscle weakness, muscle atrophy, fasciculations, hypo reflexia, hypotonicity)
  • Other symptoms: sialorrhea, dysarthria, dysphagia, pseudobulbar affect, chronic fatigue, difficulties breathing, urinary changes
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7
Q

What functions and motor neurons are typically NOT affected by ALS

A
  • Sensory
  • Bowel and bladder
  • Cognition
  • Sexual function
  • Cardiovascular
  • Oculomotor
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8
Q

What is the life expectancy of ALS?

A

Average is 3 years from onset

**20% survive 5 years, 10% survive 10 years, and 5% survive 20 years

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9
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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10
Q

What is the cause of ALS?

A

UNKNOWN, but it is associated with several genetic mutations

**90% is sporadic and 10% is hereditary, but no difference in clinical presentation

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11
Q

What criteria is used to Dx ALS?

A
  • Presence of UMN signs, LMNS, and spread of sx within a region or to other regions
  • Absence of other disease processes - electrodiagnostic testing
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12
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
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13
Q

What medication is use to treat ALS? How does it work? Pros and cons?

A

Riluzole: It decreases the release of glutamate.

- It increases life expectancy by 2-9 months - It’s very expensive

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14
Q

Exercise intensity recommendations for people with ALS

A
  • High intensity exercise is CONTRAINDICATED in all stages of ALS
  • Moderate intensity exercise may be beneficial in EARLY stages only
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15
Q

Use of AD and DME for persons with ALS

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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16
Q

What causes polio?

A

Virus poliomyelitis that causes inflammation to the meninges and anterior horn cell with loss of spinal and bulbar motor neurons

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17
Q

What is the clinical presentation of polio?

A

Asymmetrical, flaccid paralysis, usually LE > UE

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18
Q

What is post polio syndrome?

A

New onset of symptoms occurring 15-40 years post infection

19
Q

What are symptoms of post polio syndrome?

A
  • Fatigue
  • New respiratory difficulties
  • Cold intolerance
  • Weakness
  • Joint and muscle pain
  • Decreased endurance
20
Q

What is the progression like in post polio syndrome?

A

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

21
Q

Why do they think post polio syndrome occurs?

A

Failure of the oversized motor units that developed due to collateral sprouting

22
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
23
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
24
Q

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

A

25-40%

25
Q

Does GBS affect UMN or LMN?

A

LMN, CNS is unaffected.

26
Q

What is the mechanism of GBS?

A
  • Autoimmune disorder of the PNS
  • Inflammatory process affects the Schwann cells causing de-myelination of the axon
  • Axon can remain intact or in some cases axonal damage occurs
27
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
28
Q

Who is most affected by GBS?

A
  • Men > women

- Ages 30-50 yr old

29
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
30
Q

How fast/slow does GBS progress?

A

Onset to peak disability ranges from hours to week

31
Q

What type of disease is GBS

A

Autoimmune disorder of the PNS

32
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 effective - actually delays recovery
33
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
34
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
35
Q

What structures does Myasthenia Gravis affect?

A
  • Oculomotor
  • Facial
  • Mastication
  • Speech and swallowing muscles
36
Q

What is the medical management for myasthenia gravis?

A

Corticosteroids or immunosuppressants

37
Q

What are the rehab implications for people with myasthenia gravis?

A
  • Energy conservation

- Risk for falls with decreased bone density

38
Q

Who does myasthenia gravis usually affect?

A

Younger females and older males

39
Q

What are some trophic changes that can occur with peripheral neuropathy?

A

Shiny skin and brittle nails

40
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
41
Q

Peripheral nerve anatomy

A
  • Epineurium - entire nerve
  • Perineurium - around fasciculations
  • Endoneurium - around axons
42
Q

Seddon Classification

A

Class I - neurapraxia
Class II - axonotmesis
Class III - neurotmesis

43
Q

Sunderland Classificatrion

A

Grade I - neurapraxia, complete/fast recovery
Grade II - axonotmesis, full recovery
Grade III - disruption of axon/endoneruium, variable recovery
Grade IV - disruption of axon, endoneurium and perineurium with no recovery, surgery required
Grade V - nerve disection, surgery

44
Q

Charcot-Marie-Tooth Disease

A
  • Genetic condition that affects motor/sensory nerves
  • Associated with leg weakness and foot deformities
  • Progressive, no treatment available

NCS (19 decks)

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