A family with a stereotyped unilateral or bilateral transient visual loss, that recurred many times daily and was associated with childhood epilepsy and familial hemiplegic migraine, is reported from University Hospitals, Geneva, Switzerland. The index case was a 43-year-old man who had 3 to 10 daily episodes since infancy, commonly provoked by sudden changes of light intensity or by pressure on the eyelids, and followed by a 30 sec refractory period when amaurosis could not be reprovoked. Illumination of the affected eye in unilateral amaurosis failed to induce pupillary responses, whereas the response to illumination of the unaffected eye was normal, with both direct and consensual pupillary constriction. Between episodes, pupillary responses and the neurologic exam, including MRI and angiography, were normal. The patient also developed familial hemiplegic migraine and partial epilepsy with secondary generalization in adolescence. No relation was observed between the episodic amaurosis, the migraine or seizures. Only the seizures were responsive to AEDs. Relatives affected by the episodic amaurosis, named “elicited repetitive daily blindness (ERDB),’ included a cousin, and two daughters, all affected also by familial hemiplegic migraine and partial seizures in two. Genetic linkage to CACNA1A was excuded, and inheritance is segregated as monogenic, autosomal dominant with variable expression. [1]

COMMENT. This benign familial syndrome of episodic repetitive daily blindness (ERDB), beginning in childhood and later associated with familial hemiplegic migraine and epilepsy, appears to be localized to an intermittent defect in the eye that is non-progressive but repetitive in to adult life. Ischemic causes, usually invoked in amaurosis fugax, are possible in ERDB but are considered unlikely because of the rapid reversibility and benign course.