ALS

Question 1

what is ALS

Answer 1

• adult onset
• prgoressive NM disease
• muscle wasting
• sclerosis
• UMN and LMN

-most physically devastinging disease

Question 2

what are some characteristics of ALS

Answer 2

-difficulty wtih language comprehension
attention
planning
abstract reasoning

Question 3

what is the diagnosis of ALS

Answer 3

UMN/LMN in 3 regions

EMG

Question 4

what is the prognosis

Answer 4

earlier onset (younger than 50) has a longer course
-adult sporadic onset results in death 2-5 years from pneumoina and inability to eat

Question 5

what is staging 0

Answer 5

kings= presymptomatic

MITOS: functional involvement/disease onset

Question 6

what is staging 1

Answer 6

kings= invovlement of 1 clinical region

MITOS: loss of independence in 1 functional domain

Question 7

what is staging 2

Answer 7

kings= invovlement of 2 clinicl regions

MITOS: loss of independence in 2 domains

Question 8

what is staging 3

Answer 8

kings= inovlvement in 3 clinicla regions

MITOS: loss of independence in 3 domains

Question 9

what is staging 4

Answer 9

kings= substantial respiratory or nutritional failure
MITOS= loss of independence in 4 domains

Question 10

what is staging 5

Answer 10

both= death

Question 11

what does exam history include

Answer 11

1. location of symptom onset
2. year of onset
3. date of diagnosis
4. previous trauma
5. falls secondary to weakness
6. laminectomies
7. carpal tunnel

Question 12

what is bulbar ALS

Answer 12

• inability to hold eye closed against pressure
• dysarthria
• inability to swallow
• decreased tongue movement
• droolikng
• fasciultations

Question 13

what are LMN symptoms

Answer 13

flaccid

fasciculations of tongue

Question 14

what are UMN symtoms

Answer 14

lack of dexterity
spasticity
decrased strength
hyperreflexiaq

Question 15

what is preserved throuhgout the course of ALS

Answer 15

eye movements
sensory
bowel and bladder function

Question 16

what is PT for early stages

Answer 16

• aerobic and moderate exercise
• AD
• discourage exercises with hypertrophy goal
• gentle ressitance
• ROM and stretching

Question 17

what are some risk factors

Answer 17

• older age
• low BMI
• smoking
• professional athletes
• repeated head injuries

Question 18

what is the incidence

Answer 18

2x in white
male more than female
-increases after age 40
-peaks age 65-70

Question 19

what is the pathology

Answer 19

• dysfunction of astrocytic excitatory amino transporter

- reduced uptake of gluteamate

Question 20

what is amyotrophy

Answer 20

loss of brainstem CN nuceli and anterior horn cells in spinal cord

Question 21

what is lateral sclerosis

Answer 21

loss of cortical motor cells causing corticospinal tract dysfunction

Question 22

what is the el escorial critera

Answer 22

1. evidence of LMN degeneration
2. evidence of UMN degeneration
3. progression of motor syndrome

Question 23

what are negative prognositc factors

Answer 23

1. bulbar onset
2. weight loss
3. cognitive impairment
4. impaired respiratory function