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April 1985

Visual Evoked Response Abnormality in Myoclonus Epilepsy With Large PupilsOccurrence in a Family With Acorpuscular Myoclonus Epilepsy

Author Affiliations

From the Departments of Neurology (Drs Hess, Meienberg, Muff, and Ludin) and Ophthalmology (Dr Verones), University of Bern (Switzerland).

Arch Neurol. 1985;42(4):359-361. doi:10.1001/archneur.1985.04060040069014

• Flash and pattern evoked responses were recorded from three siblings with myoclonus epilepsy who all had strikingly large pupils in daylight. Comparison with the visual evoked responses (VERs) of 15 normal and eight epileptic control subjects (including one with myoclonus epilepsy but normal pupils) disclosed a substantial reduction of the amplitude of the flash response as compared with the normal pattern response in these three patients. It is suggested that the VER constellation and the pupillary abnormality, together with the normal electroretinogram and diffusely distributed relative scotomas, were due to a ganglion cell loss in the retina.