NeuroDz 2

Question 1

MC degenerative dz of motor neuron system

Answer 1

ALS

Question 2

amyotrophy

Answer 2

atrophy of muscle fibers

Question 3

lateral sclerosis

Answer 3

changes in lateral columns of spinal cord as UMN axons degenerate and are replaced by scarring

Question 4

risk factor for ALS

Answer 4

smoking

Question 5

epidemiology of ALS

Answer 5

can begin young, highest risk 75-80

caucasians, M/F almost equal

Question 6

ALS affects ____ neurons at _____ levels of the ____ network supplying _____ regions of the body

Answer 6

ALS affects motor neurons at 2+ levels of the motor neuron network supplying multiple regions of the body

Question 7

neuron systems ALS affects (3 different areas affected)

Answer 7

1. LMN
2. corticospinal UMN
3. prefrontal motor neurons

Question 8

ALS effect on LMN

Answer 8

progressive weakness, wasting

loss of reflexes/muscle tone

Question 9

ALS effect on corticospinal UMN

Answer 9

precentral gyrus

brisk reflexes and spasticity in upper

Question 10

ALS and prefrontal motor neurons

Answer 10

plan and orchestrate UMN and LMNs

results in cognitive impairment

Question 11

areas NOT affected by ALS

Answer 11

Sphincter control
EOMS
sensory function

Question 12

clinical picture of ALS

Answer 12

insidious loss of function or slowly progressive painless weakness in 1+ region of body

w/o sensation change and w/o another cause

Question 13

emotional and cognitive changes in ALS

Answer 13

involuntary laughing/crying
loss of executive function
behavioral changes

Question 14

if LE involvement ALS clinical

Answer 14

slapping gait
tripping/stumbling w/running or walking
foot drop

Question 15

if UE involved, ALS clinical pic

Answer 15

difficulties with FINE more tasks (buttons, keys)

atrophy of hand muscles

Question 16

natural history of ALS

Answer 16

fatal, survival 3 yrs from time of apparent muscle weakness

Question 17

ALS tx

Answer 17

supportive, multimodal

muscle spasms can be tx with antispasticity agents

riluzole/Rilutek

Question 18

riluzole brand + indication

Answer 18

Rilutek

prolongs tracheostomy in ALS pts by 2-3 months

Question 19

supportive care ALS

Answer 19

assisted respirations

PEG tube

Question 20

CRPS is characterized by

Answer 20

pain, swelling, vasomotor dysfunction of extremity

pain is constant and disproportionate to injury

Question 21

CRPS epidemiology

Answer 21

MC b/t 30-60yrs

no racial, gender differences

Question 22

inciting effects of CRPS

Answer 22

trauma (penetrating or non penetrating)

surgery (carpal tunnel release, dentistry)

systemic illness (MI, CVA, pan coasts tumor)

local disease (tissue ischemia)

spontaneous

Question 23

stages of CRPS

Answer 23

acute
subacute
chronic

Question 24

acute CRPS

Answer 24

3 mo

burning pain that limits function of extremity

swelling (cool to tough, limits movement)

demineralization (due to disuse)

Question 25

subacute CRPS

Answer 25

persistent severe pain and fixed edema skin is cyanotic, blue, pale, dry loss of function due to increased pain and fibrosis of the joints (chronic inflammation_

Question 26

chronic CRPS

Answer 26

1 yr after onset pain is more variable edema subsides over time, leaving fibrosis of the joint loss of function and stiffness

Question 27

tx options for CRPS

Answer 27

prednisone NSAIDS synthetic blockage IV Calcitonin

Question 28

prednisone CRPS

Answer 28

``` daily taper (2-4 wks) improves mobility, pain, and stiffness ``` best in acute/subacute stage

Question 29

Sympathetic blockade CRPS

Answer 29

lidocaine or bupivacaine +/- epi blocks nerve impulse and allows movement of the extremity to prevent chronic fibrosis and inflammation

Question 30

peripheral neuropathy

Answer 30

disorders that affect peripheral nerves, MC hands/feet/lower legs cause pain, numbness, tingling, burning, weakness

Question 31

etiologies of peripheral neuropathy

Answer 31

1. vitamine deficiencies 2. causative meds 3. comorbitidies 4. diabetes

Question 32

diabetes peripheral neuropathy types

Answer 32

painful DM (LE painful to touch) distal symmetric (stocking glove) autonomic neuropathy (gastroparesis, urinary retention, ED, orthostatic HoTN) isolated neuropathy (one area, Motor> sensory)

Question 33

comorbidies that cause peripheral neuropathy

Answer 33

alcohol intake celiac dz CKD, HIV, amyloidosis

Question 34

medications that cause peripheral neuropathy

Answer 34

chemo drugs abx (metronidazole, fluorquinalones, isoniazid) statins phenytoin

Question 35

peripheral neuropathy study of choice

Answer 35

EMG/NCS

Question 36

mc inherited neuro disorder of neuropathy without metabolic cause

Answer 36

CMT

Question 37

natural hx of CMT

Answer 37

first 20 yrs = tripping over feet, freq. falls, clumsiness foot drop and stoppage gait distal weakness, difficulty with motor tasks

Question 38

s/s of CMT

Answer 38

wasting of muscles of legs (stork legs) pes cavus, loss of DTRs, gait ataxia pain/temp sensations intact