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September 1970

Familial Hemiplegic Migraine, Retinal Degeneration, Deafness, and Nystagmus

Author Affiliations

Charleston, SC
From the Division of Neurology and the Department of Pediatrics (Dr. Young), Medical University of South Carolina, Charleston, SC.

Arch Neurol. 1970;23(3):201-209. doi:10.1001/archneur.1970.00480270011002

HEMIPLEGIC is defined as "vascular headache featured by sensory and motor phenomena which persist during and (for a brief time) after the headache."1 The definition implies that the patient is well prior to an attack and that he recovers completely. Complicated migraine designates cases in which neurologic abnormality persists without recovery. Common migraine indicates vascular headache without striking prodromes, and it may be unilateral or bilateral. Familial occurrence of hemiplegic migraine has been reported infrequently since Clarke2 described a family in which 11 relatives from four generations were affected. Retinitis pigmentosa associated with migraine was described by Friedman3 and again by Connor.4 The retinal abnormality was unilateral and was thought to be due to central vein occlusion in Friedman's case. Connor summarized 18 cases of complicated migraine in which permanent or long-lasting retinal or brain damage occurred. Thirteen of the 18 cases had

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