Motor Neuron Disease Flashcards
what medication is used for ALS?
Riluzole, which inhibits glutamate release
what diseases result from degeneration of anterior horn cells
ALS, poliomyelitis and post-polio syndrome
prognosis of ALS
50% die within 3 years, 30% live for 5 years, 10% live for 10 years
what MND are pseudobulbar signs seen in?
ALS
which MND is due to both upper and lower motor neuron lesion
Amyotrophic lateral sclerosis
how long after initial poliomyelitis diagnosis is required before having diagnosis of post poliomyelitis with return of symptoms?
15 years
which muscle in SMA is least affected
facial muscles
wheelchair in SMA 2 and 3 is by what age?
SMA 2 - by 2-3 years old
SMA 3 - by 30 years old
snaps and cmaps of ALS and polio
ALS and poli is normal SNAP and CMAP. post-polio syndrome shows abnormal CMAP
what tongue finding is seen in SMA
tongue fasciculations
what virus is involved with poliomyelitis
picornavirus
which SMA is characterized by frog legged position
SMA 1
what is seen in SFEMG for ALS
increase jitter
which SMA is fatal
1 and 2
which SMA has best prognosis
SMA 3 - normal life expectancy. SMA 2 - death by 10 years
when are patients diagnosed with ALS usually wheelchair bound
12-18 months after dx
pseudobulbar signs
difficulty chewing, swallowing, and with speech, along with emotional outburst such as crying and laughing
which MND is due to lower motor neuron lesion
spinal muscle atrophy
poliomyelitis
post-polio syndrome
Genetics of SMA
All are autosomal recessive except SMA 3 which may be dominant
which SMA do you see gower’s sign
SMA 3
which MND is due to upper motor neuron lesion
primary lateral sclerosis and hereditary spastic paraplegia
how many muscles has to be evaluated in EMG for ALS
2 muscles from 2 different nerve roots in 3 different body regions
blood findings of SMA
increased CPK
What are the three SMA alternate names?
SMA 1 = werdnig-hoffman diseaes
SMA 2 = chronic werdnig-hoffman
SMA 3 = kugelberg-welander
first signs seen in ALS
asymmetric atrophy, weakness, fasciculations
what MND is sensation spared in?
ALS and poliomyelitis
prognosis of ALS and age of onset
younger is better
sitting/standing pattern and SMA
SMA 1 - never sits independently
SMA 2 - independent sitting
SMA 3 - independent standing
which SMA has worst prognosis
SMA 1 - death by 2-3 years
which SMA do you find normal CMAP
SMA 3
