Amytrophic Lateral Sclerosis (ALS)

Question 1

ALS is also known as ____.

Answer 1

Lou Gehrig’s Disease

Question 2

What is ALS?

Answer 2

Combination of UMN and LMN symptoms

Characterized by degenerative scarring or sclerosis in the lateral aspects of the spinal cord, brainstem and cortex

Question 3

Peripheral nerve involvement lead to atrophy of what 2 structures?

Answer 3

1. Ventral root atrophy

2. Muscle atrophy

Question 4

____ % of ALS occurs with unknown etiology.

Answer 4

90%

Question 5

ALS is more common in which sex? What is the mean age of onset?

Answer 5

Males > Females

Mean age of onset: 57

Question 6

The presence of what 4 impairments rules out a diagnosis of ALS?

Answer 6

1. Cognitive
2. Oculomotor
3. Bowel and Bladder
4. Sensory

Question 7

What is the pathogenesis behind ALS?

Answer 7

1. Demyelination and gliosis occurs in the corticospinal
   and corticobulbar tracts
2. Death of motor neurons in the brainstem and spinal
   cord result in denervation and subsequent atrophy

Question 8

What is the most common early sign of ALS?

Answer 8

Focal striated muscle weakness that

progresses to include the entire body

Question 9

Clinical presentation of NCVs and EMGs in patients with ALS

Answer 9

NCVs are typically normal

EMGs consistent with LMN disease

Question 10

What is the typical early clinical presentation of ALS?

Answer 10

1. Most patients unaware of changes until some functional limitation draws attention to the problem
2. Physical exam, especially MMT, will show much
   greater weakness than the patient reports

Question 11

____% of motor neurons in affected area may have been lost before the beginning of symptoms

Answer 11

80%

Question 12

What is the typical pattern of onset in ALS?

Answer 12

UE, LE and Bublar primarily affected

A significant feature is asymmetry of weakness with the sparing of muscle fibers even in highly atrophied muscles

Question 13

What areas of the brain and functions are spared in patients with ALS?

Answer 13

Cerebellum is unaffected

Occulomotor and bowel and bladder function are spared

Question 14

What are the outcomes associated with ALS?

Answer 14

1. Paralysis of all spinal
   musculature and all muscles innervated by cranial nerves
2. Persons with bulbar onset tend to have a more rapid
   progression than those with extremity onset
3. Death usually results from respiratory failure ~4-5 years
   after onset

Question 15

What supplements/medications are used to treat ALS?

Answer 15

1. Riluzole (Rilutek), a glutamate antagonist that slows excitotoxic
   cell death, has been used with some success
2. Antioxidants: May limit further injury to motor neurons, protecting against cell death
3. Neurotrophic factors: May aid in the recovery of injured, but still viable, motor neurons

• A combination of these therapies is the current practice

Question 16

How is ALS medically managed? (4)

Answer 16

1. Muscle spasm can be treated with PROM or
with antispasmodics
2. Maintenance of adequate nutrition
3. Artificial ventilation
4. End of life care at late stages