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In order Cuccurullo > Ch 5 - EDX: Motor Neuron Disease (MND) > Flashcards

Ch 5 - EDX: Motor Neuron Disease (MND) Flashcards

1
Q

What is affected in motor neuron disease?

A

Progressive degeneration of the motor neurons in the spinal cord, brainstem, or motor cortex

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2
Q

What are examples of lower motor neuron diseases?

A

Spinal muscle atrophy (SMA)
Poliomyelitis/Post-polio
syndrome

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3
Q

What are examples of lower and upper motor neuron diseases?

A

Amyotrophic lateral sclerosis

ALS

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4
Q

What are examples of upper motor neuron diseases?

A

Primary lateral sclerosis (PLS)

Hereditary spastic paraplegia

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5
Q

What are signs of lower motor neuron diseases?

A

Atrophy
Flaccidity
Hyporeflexia
Fasciculations

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6
Q

What are signs of upper motor neuron diseases?

A

Weakness
Spasticity
Hyperreflexia
Upgoing plantar response

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7
Q

What should be evaluated on EDX in motor neuron disease?

A

Evaluation of at least 1 upper and 1 lower limb

Start with most severely affected muscles

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8
Q

What should be ruled out on EDX of motor neuron disease?

A

Treatable neuropathies such as multifocal motor neuropathy

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9
Q

What is seen on SNAP in motor neuron disease?

A

Typically normal
Can be ABN with hereditary spastic paraplegia and
spinobulbar muscular atrophy

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10
Q

What is seen on CMAP in motor neuron disease?

A

ABN in weak muscles with asymmetry side to side
Dec CV
Prolonged latencies

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11
Q

What is seen on EMG in motor neuron disease?

A

Active denervation with reinnervation must be found in 3 of 4 body segments (craniobulbar, cervical, thoracic, and lumbosacral). At least 2 muscles with different innervations should be abnormal.

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12
Q

Which motor neuron diseases will have Fibs and + sharp waves on EMG?

A
  • SMA Type I
  • SMA Type II
  • SMA Type III
  • Amyotrophic lateral sclerosis (ALS)
  • Poliomyelitis
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13
Q

Which motor neuron diseases will have fasiculations on EMG?

A
  • ALS
  • Poliomyelitis
  • Post-polio syndrome
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14
Q

Which motor neuron diseases will have complex repetitive discharges on EMG?

A

• SMA Type III

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15
Q

Where should a monopolar needle be placed during EMG to evaluate the diaphragm?

A

Inserted through the eighth or ninth intercostal space at the anterior axillary line

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16
Q

What is evaluated on EMG during expiration?

A

MUAP of the intercostal muscles is recruited during expiration and is higher in amplitude
than the diaphragm

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17
Q

What is evaluated on EMG during inspiration?

A

Diaphragm recruitment

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18
Q

What is the inheritance and onset of SMA Type I (Werding-Hoffman disease)?

A

Autosomal recessive

3 to 6 months

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19
Q

What is the course of SMA Type I (Werding-Hoffman disease)?

A

Rapid, fatal respiratory failure
Death by 2 to 3 yo
Worst prognosis

20
Q

What is the clinical presentation of SMA Type I (Werding-Hoffman disease)?

A 
• Floppy baby/ hypotonia
• Unable to reach
milestones
• Progressive weakness
• Absent MSR
• Difficulty feeding
• Weak cry
• Frog-legged position
• Tongue fasciculations
• Paradoxical breathing
• Never sits independently
21
Q

What is spared in SMA Type I (Werding-Hoffman disease)?

A
  • Facial muscle affected least
  • Extraocular muscles intact
  • Sphincter muscles are spared
22
Q

What is seen on labs and muscle biopsy in all SMA types?

A

Increase CPK levels

Hyper/atrophic fibers

23
Q

What is the inheritance pattern and onset of SMA Type II (Chronic Werding-Hoffman disease)?

A

Autosomal recessive

2 to 12 months

24
Q

What is the course of SMA Type II (Chronic Werding-Hoffman disease)?

A

Slower, fatal respiratory failure
WC by 2-3yo
Death ~10 yo

25
Q

What is the clinical presentation of SMA Type II (Chronic Werding-Hoffman disease)?

A 
• Floppy baby/ hypotonia
• Gradual progressive limb weakness; upper > lower
• Absent MSR
• Face least affected
• Kyphoscoliosis
• Equinus deformity of the feet
• ± Tongue fasciculations
• Progressive pulmonary
involvement
26
Q

What is the expected function of SMA Type II (Chronic Werding-Hoffman disease)?

A

• Independent sitting
• Assistive devices for
standing and walking
• WC by 2-3 yo

27
Q

What is the inheritance pattern and onset of SMA Type III (Kugelberg-Welander disease)?

A

Autosomal recessive/dominant

2 to 15 years

28
Q

What is the course of SMA Type III (Kugelberg-Welander disease)?

A

Slow
Normal life expectancy
Wheelchair by 30 years of age

29
Q

What is the clinical presentation of SMA Type III (Kugelberg-Welander disease)?

A 
• Symmetric weakness:
lower limb then upper
limb
• Abnormal MSR
• ± Gowers’ sign
• ± Calf
pseudohypertrophy
• ± Dysphagia
• ± Dysarthria
• Tongue
fasciculations—late
onset
30
Q

What is the expected function of SMA Type III (Kugelberg-Welander disease)?

A

• Normal intelligence
• Independent
standing/walking
• WC by 30 yo

31
Q

What is the pathology of ALS?

A

Degeneration of the

anterior horn cell

32
Q

What is the onset of ALS?

A

Most commonly in men after the sixth decade

33
Q

What are the first signs of ALS?

A

Asymmetric atrophy
Weakness
Fasciculations

34
Q

What are the pseudobulbar signs of ALS?

A

Difficulty chewing, swallowing, speech along with unprovoked emotional
outbursts

35
Q

What is typically spared in ALS?

A
  • Bowel and bladder
  • Sensation
  • Extraocular muscles
36
Q

What is the prognosis of ALS?

A

50% die w/in 3 years
30% live for 5 years
10% live for 10 years
WC by 12-18 months

37
Q

What are predictors of survival of ALS?

A

Younger is better
Severity of onset
Pulmonary function
ABN sniff test=poor survival

38
Q

What is the anatomic pathology of Poliomyelitis?

A

Degeneration of the anterior horn cell

39
Q

What is the etiology of Poliomyelitis?

A

Picornavirus orally enters the body and spreads via lymphoid system leading to orphaned muscle fibers

40
Q

What are signs of initial infection with Poliomyelitis?

A 
Fever
Malaise
Sore throat
Vomiting
Headache
Back and neck pain/ stiffness
41
Q

What is the clinical presentation of Poliomyelitis?

A
  • Weakness
  • Absent MSR
  • Bulbar palsies
  • Sensation spared
  • Autonomic dysfunction can occur
42
Q

What is the prognosis of Poliomyelitis?

A

– 25%: Severe disability
– 25%: Mild disability
– 50%: Complete recovery

43
Q

What is the mortality rate of Poliomyelitis?

A

1%-4% chance in children

10% chance in adults with bulbar and respiratory involvement

44
Q

What is the anatomic pathology of Post Poliomyelitis syndrome?

A

Loss of the anterior horn cell

45
Q

What is the etiology of Post Poliomyelitis syndrome?

A

Death of the motor neuron due to aging

Burnout of motor unit from increased metabolic demand

46
Q

What is the Halstead-Ross criteria of Post Poliomyelitis syndrome?

A
  1. Hx of a previous dx
  2. Recovery of function
  3. Stability for ~15 years
  4. Return of sx
  5. No other medical problems to explain new sx (weakness, atrophy)

In order Cuccurullo (91 decks)

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