Motor Neuron Disease

Question 1

what medication is used for ALS?

Answer 1

Riluzole, which inhibits glutamate release

Question 2

what diseases result from degeneration of anterior horn cells

Answer 2

ALS, poliomyelitis and post-polio syndrome

Question 3

prognosis of ALS

Answer 3

50% die within 3 years, 30% live for 5 years, 10% live for 10 years

Question 4

what MND are pseudobulbar signs seen in?

Answer 4

ALS

Question 5

which MND is due to both upper and lower motor neuron lesion

Answer 5

Amyotrophic lateral sclerosis

Question 6

how long after initial poliomyelitis diagnosis is required before having diagnosis of post poliomyelitis with return of symptoms?

Answer 6

15 years

Question 7

which muscle in SMA is least affected

Answer 7

facial muscles

Question 8

wheelchair in SMA 2 and 3 is by what age?

Answer 8

SMA 2 - by 2-3 years old

SMA 3 - by 30 years old

Question 9

snaps and cmaps of ALS and polio

Answer 9

ALS and poli is normal SNAP and CMAP. post-polio syndrome shows abnormal CMAP

Question 10

what tongue finding is seen in SMA

Answer 10

tongue fasciculations

Question 11

what virus is involved with poliomyelitis

Answer 11

picornavirus

Question 12

which SMA is characterized by frog legged position

Answer 12

SMA 1

Question 13

what is seen in SFEMG for ALS

Answer 13

increase jitter

Question 14

which SMA is fatal

Answer 14

1 and 2

Question 15

which SMA has best prognosis

Answer 15

SMA 3 - normal life expectancy. SMA 2 - death by 10 years

Question 16

when are patients diagnosed with ALS usually wheelchair bound

Answer 16

12-18 months after dx

Question 17

pseudobulbar signs

Answer 17

difficulty chewing, swallowing, and with speech, along with emotional outburst such as crying and laughing

Question 18

which MND is due to lower motor neuron lesion

Answer 18

spinal muscle atrophy
poliomyelitis
post-polio syndrome

Question 19

Genetics of SMA

Answer 19

All are autosomal recessive except SMA 3 which may be dominant

Question 20

which SMA do you see gower’s sign

Answer 20

SMA 3

Question 21

which MND is due to upper motor neuron lesion

Answer 21

primary lateral sclerosis and hereditary spastic paraplegia

Question 22

how many muscles has to be evaluated in EMG for ALS

Answer 22

2 muscles from 2 different nerve roots in 3 different body regions

Question 23

blood findings of SMA

Answer 23

increased CPK

Question 24

What are the three SMA alternate names?

Answer 24

SMA 1 = werdnig-hoffman diseaes
SMA 2 = chronic werdnig-hoffman
SMA 3 = kugelberg-welander

Question 25

first signs seen in ALS

Answer 25

asymmetric atrophy, weakness, fasciculations

Question 26

what MND is sensation spared in?

Answer 26

ALS and poliomyelitis

Question 27

prognosis of ALS and age of onset

Answer 27

younger is better

Question 28

sitting/standing pattern and SMA

Answer 28

SMA 1 - never sits independently
SMA 2 - independent sitting
SMA 3 - independent standing

Question 29

which SMA has worst prognosis

Answer 29

SMA 1 - death by 2-3 years

Question 30

which SMA do you find normal CMAP

Answer 30

SMA 3