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April 1978

Sensory Neuropathy With Onion-Bulb Formation: Report of a Case With Onset in Infancy

Author Affiliations

From the Departments of Pathology (Neuropathology) (Drs Koto, Horoupian, and Suzuki) and Pediatrics (Dr Torch), the Saul R. Korey Department of Neurology (Dr Spiro), and the Rose F. Kennedy Center for Research in Mental Retardation and Human Development (Drs Koto, Horoupian, and Suzuki), Albert Einstein College of Medicine, Bronx, NY.

Am J Dis Child. 1978;132(4):379-381. doi:10.1001/archpedi.1978.02120290051009

• A 27-month-old girl suffered from severe sensory neuropathy with minimal motor dysfunction. The CSF protein level was increased and nerve conduction was severely impaired. Sural nerve biopsy specimen showed increased endoneurial connective tissue. An onion-bulb pattern with concentric interdigitations of Schwann cell cytoplasmic processes and redundant basal laminae were prominent features under electron microscopy. Degree of myelination in individual fiber was far less than expected. Although the clinical manifestations of onion-bulb neuropathy with onset in infancy have been reported to resemble infantile progressive spinal muscular atrophy, the present case demonstrates that the condition can also appear as severe sensory ataxia.

(Am J Dis Child 132:379-381, 1978)

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