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Classification

Five varieties have been distinguished on clinical grounds.

A. Progressive Bulbar Palsy: Bulbar involvement predominates owing to disease processesaffecting primarily the motor nuclei of the cranial nerves.

B. Pseudobulbar Palsy: Bulbar involvement predominates in this variety also, but it is due to bilateral corticobulbar disease and thus reflects upper motor neuron dysfunction.

C. Progressive Spinal Muscular Atrophy: This is characterized primarily by a lower motor neuron deficit in the limbs due to degeneration of the anterior horn cells in the spinal cord.

D. Primary Lateral Sclerosis: There is a purely upper motor neuron deficit in the limbs.

E. Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease): A mixed upper and lower motor neuron deficit is found in the limbs. This disorder is sometimes associated with dementia or parkinsonism.

 

 

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