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June 1953


Author Affiliations


From the Second Cornell Medical (Neurology) Division, Bellevue Hospital, and the Department of Medicine, Cornell University Medical College.

AMA Arch NeurPsych. 1953;69(6):756-759. doi:10.1001/archneurpsyc.1953.02320300089010

CURRAN,1 at Bellevue Hospital, in 1931 described a family in which five of six siblings had Strümpell-Westphal pseudosclerosis, or progressive hepatolenticular degeneration. Readmission of two of these siblings in 1952 presented us with an opportunity to study again and to review an unusually large family group with this disorder and to observe the natural history of the disease during a 21-year period. Wilson,2 in his discussion of hepatolenticular degeneration, noted only one family with more members afflicted, a family of 14 in which 6 members had the disease. He reported only two patients who lived more than 20 years after the onset of symptoms. One patient lived 41 years, and another was alive at the time of the report, 28 years after the appearance of symptoms.

The clinical evidence for the familial nature of this disease is well established.2 Recent family studies have indicated that even the