Amyotrophic lateral sclerosis (ALS) Flashcards Preview

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Flashcards in Amyotrophic lateral sclerosis (ALS) Deck (20):
1

Amyotrhopic lateral sclerosis name meaning

amyotrophic (atrophys of fiber)
Lateral (lateral column of spinal cord)
sclerosis (inflm causing damage, hardening)

2

ALS is a __________ neuron disorder

Motor neuron disorder, (AFFECTS MOTOR NEURONS ONLY)

3

ALS higher incidence

in middle aged males (2x), therefore we can create expectation autoimmunity not involved

4

ALS is

acute, progressive, death in 2-5yrs
-respiratory complications most frequent cause of death

5

what is most frequent cause of death in ALS

-respiratory complications

6

in ALS there is issues

with innervation to muscle, which causes motor function problems

7

when word ends in ase

it is an enzyme

8

Etiology of ALS

Sporadic (90-95%):
-SOD1 gene on chr 21 (aprox 5%)

Familial (5-10%):
-SOD1 (20%)
-other genes (80%) complex trait (polygenic & enviromental)

other components as well (viral trigger, autoimmune?)

9

SOD1 gene

superoxide dismutase 1 gene:
gene makes enzyme that breaks down free radicals)
if gene intact enzyme will work, if mutated accumulation of free radicals

10

Patho of ALS

-degeneration of motor neurons especially in:
anterior horn cells of spinal cord
motor nuclei in brain stem
upper motor neurons in cerebral cortex (connect brain to spinal cord)

-mechanism unclear: proposed to be via free radicals &/or glutamate toxicity (unclear why free radicals specifically targeting motor neurons)

11

what do the upper motor neurons in cerebral cortex do

connect brain to spinal cord

12

glutamate

neurotransmitter in excess can cause neurotoxicity

13

manifestations of ALS

-muscle atrophy, weaknesss, fasciculations (muscle twitches)
-dysarthria (impaired speech, need to utilize muscles to speak, muscles associated w speech atrophy, problems)
-acute com
-intact cognition & sensory function

14

what are fasciculations

muscle twitches

15

what is dysarthria, what is dysphagia

dysarthria: impaired speech, need to utilize muscles to speak, muscles associated w speech atrophy, problems with speech)
dysphagia: cannot swallow d/t muscle atrophy causing aspiration (not able to cough because muscles atrophied)

16

what are some acute complications form ALS

dysphagia & aspiration

17

what is intact with ALS

intact cognition & sensory function

18

Diagnosing ALS

-history, clinical presentation & physical exam
-electromyography

19

what is electromyography

detects abnormal muscle & neuron functions
can look at muscle function & neuron function or both, from electrical stimulus

20

treatment of ALS

-largely supportive (ex. Ensure flu shot, pneumnococcal vaccines, issues wiht ingestion of food d/t dysphagia< PEG tube- nutrition goes through stomach via abdominal wall)
-supportive care can turn into palliative care
-riluzole (antiglutamate neuro protection):
somehow prevents glutamate toxicity, does not know action, limited success