Clinical and pathological findings in an unusual infantile motor neurone disease.

An unusual motor neurone disease with a characteristic denervation atrophy of muscle is described in an infant. The striking change is a vacuolization of the cytoplasm of the affected ganglion cells in many motor nuclei of spinal cord and brain-stem, and in the claustrum. To our knowledge this disease has not yet been described in man and similar pathological changes have been found only in a certain strain of mice (`wobbler').