Degenerative Diseases

Question 1

ALS

Answer 1

Amyotrophic Lateral Sclerosis

• Most common form of adult onset progressive motor neuron disorder
• physically devastating of the neurodegenerative diseases

Question 2

Define Amyotrophy

Answer 2

peripheral nn changes resulting in muscle fier atrophy

Question 3

Define Lateral (ALS)

Answer 3

refers to the motor neurons in the lat aspect of the spinal cord, brainstem, and cerebral cortex which are involved

Question 4

Define Sclerosis

Answer 4

degeneration and scarring

Question 5

ALS-clinical Manifestations

Answer 5

-Extensor muscles weaker than flexors muscles typically, especially in the hands.
-positive hoffmans & babinskis
-hyper-reflexive DTRs
-Clonus
eye mov, sensory, bowel and bladder fx and cognition are preserved

Question 6

ALS Clinical Manifestations cont

Answer 6

• progressive bulbar palsy
• progressive spinal muscle atrophy
• Respiratory complications
• Oral Motor Complications

Question 7

ALS Diagnosis

Answer 7

• Clinical presentation
• EMG (fibrillation & fasiculations)
• muscle biopsy (denervation atrophy)
• muscle enzymes (CPK levels elevated)
• normal CSF, no changes no pyelogram

Question 8

Symptomatic therapy (ALS medical Management)
**no known method to stop the clinical course of ALS

Answer 8

• anticholinergic drugs-control drilling
• baclofen and diazepam for spasticity
• difficulty in food management, chewing, lip closure, and swallowing, may require modification in the consistency and texture of food and fluids
• ventilator for respiratory failure

Question 9

ALS Prognosis

Answer 9

“relentlessly” progressive

-adult onset type-death within 2-5 yrs (die from complications related to respiratory compromise-pneumonia

Question 10

AD

Answer 10

Progressive dementia, characterized by a slow decline in memory, language, visuospatial skills, personality, and cognition. (Miller et al, 1994)

Question 11

AD-pathophysiology

Answer 11

-Cell death and atrophy of cerebral cortex
-Progressive accumulation of insoluble fibrous
material (amyloid)
-Senile plaques- amyloid material surrounded by cellular debris (fragmented axons, glial cells)
◦ Located mostly in cerebral cortex and hippocampus
-Neurofibrillary tangles- bundles of abnormal filaments within neurons accumulated in a tangled mass in cell bodies
-Decrease in axonal transport of neurotransmitters

Question 12

AD-Clinical Picture(early stages)

Answer 12

◦ Subtle personality changes ( Indifference, impulsivity, irritability, egocentricity)
◦ Inability to learn new information*
◦ Inability to handle money, balance checkbook
◦ Diminished decision making/judgements
◦ Visuospatial deficits (Navigating the environment, cooking, manipulating mechanical objects in the home)
◦ Depression

Question 13

AD- clinical picture (later stages)

Answer 13

◦ Loss of older memories and recall of events ◦ Language deficits
◦ Delusion
◦ Agitation- Sun downing
◦ Difficulty sleeping, eating
◦ Inappropriate sexual behavior
◦ Patient becomes mute and bedridden

Question 14

AD-clinical picture (motor function issues)

Answer 14

◦ Generalized weakness
◦ Stereotypical and rigid movements
◦ Postural reflexes diminish
◦ Increased risks of falls (30% of individuals with AD)
-Decreased perception, postural reflexes, ability to move adequately around objects