Near reflex accommodation spasm: unusual presentation of generalized photosensitive epilepsy

We report on a 9 year old girl admitted with prolonged and recurrent episodes of near reflex accommodation spasm (NRAS), which was the sole clinical manifestation of primary generalized photosensitive epilepsy. Four days prior to admission, the patient started to experience intermittent episodes of adduction of both eyes and miosis, namely near reflex accommodation spasm, which apparently became fixed three hours prior to admission, with no other perturbations. On examination she was fully alert showing a fixed NRAS, but otherwise unremarkable neurological examination. Time locked with NRAS, the EEG demonstrated generalized spike/wave discharges, also induced with photic stimuli, compatible with generalized photosensitive epilepsy. Assuming an epileptic etiology for the diagnosed NRAS, she was started on a small dose of valproate and became asymptomatic within the first day of treatment, along with EEG normalization. She remained asymptomatic within seven months when the valproic acid was erroneously discontinued. After a few days she began experiencing short lived episodes of NRAS, which could also be induced by photic stimulation and a concurrent EEG again demonstrated generalized epileptiform discharges. Searching the literature, this is the first report associating generalized photosensitive epilepsy with NRAS, and hence complete resolution of NRAS in response to antiepileptic therapy along with normalization of the EEG. An epileptic disorder should be therefore taken into account in any patient with new onset NRAS, especially when other epileptic phenomena are absent.