Familial Hemiplegic Migraine, Retinal Degeneration, Deafness, and Nystagmus

Abstract

HEMIPLEGIC is defined as "vascular headache featured by sensory and motor phenomena which persist during and (for a brief time) after the headache."¹The 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 Clarke²described a family in which 11 relatives from four generations were affected. Retinitis pigmentosa associated with migraine was described by Friedman³and again by Connor.⁴The 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