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October 1971

The Chediak-Higashi-Steinbrinck Syndrome: Presentation of Three Cases With Features Resembling Spinocerebellar Degeneration

Author Affiliations

From the departments of neurology and internal medicine, Lahey Clinic Foundation, Boston. Dr. Sheramata is now with the Boston City Hospital.

Arch Neurol. 1971;25(4):289-294. doi:10.1001/archneur.1971.00490040015001

Three patients with Chediak-Higashi-Steinbrinck syndrome and clinical pictures resembling a spinocerebellar degeneration are older than any patients previously reported. Including this report, six of 38 cases in the English literature have presented evidence of similar neurologic pictures. Pathologic evidence of reported cases and animal work support the conclusion that a form of spinocerebellar degeneration is, to some extent, a regular part of the varied clinical and pathologic manifestations comprising this syndrome.

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