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Chapter: Medicine Study Notes : Neuro-sensory

Other CNS Degenerative Diseases

Most common form for progressive Motor Neurone Disease

Other CNS Degenerative Diseases

 

Amyotrophic Lateral Sclerosis (ALS)

 

·        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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Medicine Study Notes : Neuro-sensory : Other CNS Degenerative Diseases |


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