Sachen: Anterior Horn Cell Disease Flashcards
what comes in through the dorsal root ganglion?
- sensory info
- motor stuff comes out through the anterior
-What is motor neuron diseas
-disorders thatcause degeneration of the motor neurons in the spianl cord with or w/o similar lesion in the lower brainstem motor nuclei and/ or the BETz cells of the brain and associated long tracts
How is motor neuron disease characterized clinically?
-by progressive wasting and weakness of the affecte muscle without accompanying sensory, cerbellar, or mental changes
Motor neuron disease ddx
- idiopathic
- toxins: heavy metals
- infections: polio, post-polio syndrome, west nilve virus
- metabolic: alpha glucosidase (acid maltase) deficiency, remote effect of cancer
- familial- rare
- multiple radiculopathies
- pompe’s disease*
what is Pompe disease
- genetic… manifests in children usually
- it’s treatable
- look for maltase… you probably won’t find it
What are the adult motor neuron diseases?
- ALS: amyotrophic lateral sclerosis
- PBP: progressive bulbar disease
- SMA: spinal muscle atrophy
- PLS: primary lateral sclerosis
- atypical ALS
What does amyothrophic latral sclerosis mean?
- A- withous
- Myo- muscle
- trophic- nourishment
- lateral- side of the spinal cord
- sclerosis- hardening or scaring
- Lou Gehrig’s disease
ALS
- no definite risk factors related to occupation
- mixed upper and lower motor neuron signs
- may also be bulbar involvement of the upper or lower motor neuron type
pathophys of ALS
- degeneration of the anterior horn cells and lateral and ventral corticospinal tracts
- etiology unkown
What kind of cells degenerate in ALS?
-pyramidal Betz cells, anterior horn cells, brainstem motor nuclei of the lower cranial nerves, and corticospinal and corticobulbar tracts… alone or in combo
clinical presentation of ALS
- gait disorder, limb weakness, speech or swallowing difficulty are common initial complaints
- unexplained weight loss, cramps, and fasciculations
- tongue atrophy and fasciculations commonly seen
Diagnostic testing in ALS
- EMG: widespread denervation and reinnervation
- CPK: normal or slightly increased
- CSF: normal
- Imaging sudies (brain, spine)…. normal
- Muscle biopsy- only needed in confusing cases
What are the important “rule of thumb” diagnositc negatives
- no sensory symtoms
- normal metation
- no EOM involvement
- bowel or bladder sx not prominent
- ducbiti rare
prognosis of ALS
- progressive without remissions, relapses, or stable plateus… screwed
- death from resp failure, pnneumonia, pulm embolus
- mean duration of sx 4 years
- tx is supportive: feeding tubes, ventilatory support….
what medication could we use to prolong life by 3-6 months?
- Riluzol
- a glutamate inhibitor
- super expensive
