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').
D001479:Basal Ganglia / Q000473:pathology; D001933:Brain Stem / Q000473:pathology; D001996:Bronchopneumonia / Q000209:etiology; D003593:Cytoplasm; D003937:Diagnosis, Differential; D005727:Ganglia, Spinal / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000473:pathology*; D008297:Male; D009046:Motor Neurons; D009133:Muscular Atrophy / Q000151:congenital / Q000175:diagnosis / Q000209:etiology / Q000473:pathology*; D009468:Neuromuscular Diseases / Q000473:pathology; D013118:Spinal Cord Diseases / Q000175:diagnosis