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April 1962

Progressive Bulbar Paralysis in Childhood (Fazio-Londe's Disease): Report of a Case with Pathologic Evidence of Nuclear Atrophy

Author Affiliations

From the Departments of Neurology, Pediatrics, and Pathology, Wayne State University College of Medicine, and the Children's Hospital of Michigan.

Arch Neurol. 1962;6(4):317-323. doi:10.1001/archneur.1962.00450220059009

Bailey, Buchanan, and Bucy1 have said: "Whenever in our experience a child has developed in a chronic manner multiple palsies of the bulbar nerves, the cause has always been a neoplasm." To this rule there seem to be very few exceptions.

We have had the opportunity of studying a child with progressive dissolution of the motor functions of the brain stem and of confirming by pathologic examination that there was, indeed, degeneration of the corresponding motor nuclei. This was the most striking manifestation of a progressive muscular atrophy. The following presentation is, to the best of our knowledge, the first clinical and pathological study of progressive nuclear amyotrophy chiefly affecting the brain stem in a child (progressive bulbar paralysis of childhood, Fazio-Londe's disease).

Report of Case  A white girl, the only child of healthy young parents, was born on April 7, 1956. The father had been hospitalized in early