Other CNS Degenerative Diseases
· Most common form for progressive Motor Neurone Disease
·
Affects upper motor neurones (ie
layer five of the motor cortex) and lower motor neurones (anterior horn cells)
·
1 – 3 per 100,000 per year
·
5 – 10% autosomal dominant, rest
sporadic
·
Cause unknown
· Clinical presentation:
o Lower motor neurons: fasciculations, progressive wasting of muscles,
bulbar involvement ® difficulty chewing etc, weakness of respiratory muscles
o Upper motor neurons: spasticity, muscle stiffness, hyperreflexia
o Sensory, bowel, bladder, ocular movements and cognitive functions
relatively preserved
o Median survival 3 years
·
Pathology:
o Neurons shrink and accumulate lipofuscin
o No macrophage or inflammatory response
o Disappearance of axons in Corticospinal and corticobulbar tracts ®
astrogliosis ® lateral sclerosis
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