Motor Neuron disorders Flashcards
bulbar means involvement of ______
and involves CN: (4)
brain stem (medulla oblongata)
9, 10, 11, 12
Motor Neuron Diseases:
- LMN lesion
- U and LMN lesion
- UMN lesion
- spinal muscle atrophy (SMA)
- poliomyelitis
- postpolio syndrome
- Amyotrophic lateral sclerosis
- Primary lateral sclerosis
____ is spinal anterior horn cell loss, LMN with little to no bulbar involvement
spinal muscle atrophy (Primary muscular atrophy)
____ is degeneration of bulbar nuclei, not c/w LMN or UMN signs
adult onset progressive bulbar palsy
____ is a UMN spastic paraparesis without cerebellar dysfunction
primary lateral sclerosis
Primary musculature atrophy:
- Weakness and atrophy without _____
- ____ motor neuron lesions
- male vs female
- symmetric?
- presents with:
- hyperreflexia
- lower
- Male:femal 3.5:1
- symmetric
- wasting of intrinsic hand muscles (can’t open jars)
_____ occurs when weakness and motor wasting predominate in the muscles innervated by the motor nuclei of the lower brainstem. Will see weakness of muscles of the jaw, face, tongue, pharynx, and larynx.
adult progressive bulbar palsy
in Adult progressive bulbar palsy no \_\_\_\_ signs no \_\_\_\_\_ cell problems male \_\_\_\_ female Onset age \_\_\_\_\_
UMN
AH cell problems (no somatic problems in the limbs)
=
60-70s
in the differential of those people with ALS who present in their 70s but these people have it localized to the brainstem nuclei, not in the limbs
PLS is degeneration of _____ tract.
no _____ signs
no _____ signs
Mean age onset:
corticospinal tract
bulbar
LMN
53.4 yoa
spastic paraparesis, no sensory abnormalities, no cerebellar dysfunction, normal intellect, no LMN signs.
Rare, non-familial disease of unknown etiology
Amyotropic Lateral Sclerosis - incidence - some areas of higher incidence (3) - prevalence Age onset Age of death Disease duration male:female
I: 0.4-1.5/100,000 sweden, guam, kiwi peninsula of Japan P: 4-6/100,000 Age 52-66 Death 62 years Usual disease duration: 3 years - early onset 45 mo - late onset 25 mo - 3:1
etiology of ALS:
___% are familial
Gene defect?
Abnormal _______
if familial, age of onset:
male:female
Duration of illness: ____
speculative; most are idiopathic
5% are AD familial
Long arm Ch 21
form of enzyme superoxide dismutase
45-48
1: 1
2. 5 years
Initial symptoms ALS
____ % with arm symptoms
____% with bulbar symptoms
____% with LE symptoms
40-60%
25-30%
20%
no sensory symptoms
ALS:
Painless ____
symmetric?
If presenting with bulbar symptoms, will have ____ and ____.
weakness
asymmetric progresses to symmetric
dysarthria and dysphagia
_____ is seen with weakness, atrophy, fasciculations in different part of body wehre they have weakness, increased reflexes, increased tone, clonus, plantar responses are upgoing. Increased jaw jerk reflex, increase SNOUT because of hyperreflexia of bulbar muscles
ALS
____ and ____ are typically spared in ALS
EOM and bowel/bladder function (sphincter muscles)
in ALS, ____ and ____ suggest worse prognosis
older onset and bulbar presenting symptoms
Early ALS will have
_____ sensory NCS
_____ motor NCS
___ shows up pathology first
normal
normal
onset latency
simple rule for diagnosing ALS on EMG/NCS
abnormal spontaneous activity in 3 limbs or 2 limbs with bulbar findings.
fasciculations (LMN findings) in the same distribution, result of loss of anterior horn cells.
as ALS progresses, what happens to motor NCS
- CMAP amplitude gets smaller (death of AH cells and motor units)
(gets less than 30% of the mean) - slowed CV (fastest fibers are dead)
(typically never less than 70% of the mean)
______ paraspinals are the best muscles to test
why?
less overlap and less DJD
