Amyotrophic Lateral Sclerosis

Question 1

What is specifically unique about ALS?

Answer 1

ALS results in UMN and LMN impairments

Question 2

Amyotrophic Lateral Sclerosis

Answer 2

Chronic degenerative disease of the motor neurons in the brian, brainstem, and spinal cord
Senosry neurons are spared

Trophic - refers to mm atrophy, fasciculations & weakness = LMN
Lateral Sclerosis - refers to hardening on palpation of the lateral column of the SC

Affecting SC & more specifically the Corticospinal tract = UMN

Question 3

Progessive degeneration and loss of motor neurons… Name the structures

(4 + 5)

Answer 3

1. UMN in motor cortex
2. Corticospinal tracts
3. Brainstem nuclei of CN V (trigeminal, VII (facial), IX (hypopharyngeal), X (vagus), XII (hypoglossal)
4. LMN in anterior horn cells of spinal cord

Question 4

Areas that are generally spared…

Answer 4

1. Sensory system
2. Spinocerebellar tracts
3. Brainstem nuceli of CN III (oculomotor), IV (trochlear), and VI (abducens) = all CN that control the eye movements
4. Anterior horn cells for S2 (innervated striated mm of pelvic floor)

Question 5

ALS characteristics

(5)

Answer 5

1. Clnical manifestations depend on extent as well as the location ALS affects
2. UMN & LMN - flaccidity, spasticity, weakness
3. Asymmetrical distributions - both areas on either side may be affected differently
4. DISTAL -> proximal - see this first & then progresses proximally
5. CAUDAL -> rostral spread w/in SC

Question 6

What is the cardinal sign of ALS?

Answer 6

WEAKNESS

Question 7

Bulbar Pathology - Impairments

(6)

Answer 7

1. “Mixed palsy” common
   UMN (spasticity) & LMN (flaccid) components
2. Bulbar mm weakness
   mm of mouth, throat, and lips that are responsible for speech & swallowing
   Generally from impairments in CN 9-12
3. Dysphagia
   INC risk of aspiration
   INC wt loss & cachexia - weakness/wasting of the body d/t an illness
4. Dysarthria
   Initial symptoms: inability to project voice and problems with enunciation (nunciation refers to how clearly and distinctly a particular individual forms the sounds that make up a word)
   Late stages: pt can become anarthric (a total inability to articulate speech in the absence of any deficit both of auditory comprehension and of written language)
5. Sialorrhea
   Absense of spontaneous swallowing to clear excess saliva
   Weakness of oral mm making it difficult to clos elips and prevent saliva from leaking out
6. Pseudobulbar affect
   Emotional liability - poor emotional control - sudden swings & emotions that are not appropriate for the context

Question 8

What type of ALS is more likely to have cognitive impairments?

Answer 8

Bulbar-onset ALS

Frontotemporal demnetia (FTD)
- cognitve decline, executive functioning impairment, difficulty with planning & organization, & personality/ behavioral chnages (irritability social disinhibition)