Adult epileptic spasms: a clinical and video-polygraphic study

To analyse the video-polygraphic features of two patients with adult epileptic spasms (ES). Two patients with epileptic encephalopathy related to different cortical malformation; epileptic spasms had been present since the age of 18 and 25 years respectively. The patients underwent a clinical and neurophysiological examination (awake and sleep video-polygraphic monitoring). In patient 1, epileptic spasms were characterized by loss of contact and a drooping forward of the head and upper extremities. ES were in clusters, of brief duration, lasting for 20'''-1''; all events occurred during eating. Awake and sleep interictal EEG disclosed asynchronous paroxysmal abnormalities in the temporo-parieto-occipital regions. The ictal EEG showed a diffuse slow wave complex with intermingled fast activity, prevalent on both anterior regions; on polygraphic recordings, the prevalence of the muscle activation, in crescendo-decrescendo, was in the neck and the right side. In patient 2, ES were characterized by loss of awareness, head deviation to the left, jerks of the head and left limbs. ES occurred in cluster, and were of brief duration (< 1"), usually on awakening. Interictal EEG showed epileptiform abnormalities in the right fronto-centro-temporal regions; abnormalities present in the awake EEG were enhanced during NREM sleep. The ictal EEG showed a diffuse, slow wave complex with intermingled fast activity, prevalent in the right regions, with concomitant muscle activation, in crescendo-decrescendo, on the left deltoid and tibialis anterior. Our two cases of adult ES were characterized by distinct clinical and polygraphic features, which were of brief duration, mild intensity, rare frequency per minute, and with preservation of cluster organization. In one patient, ES were induced by eating (reflex periodic spasms triggered by eating). A firm diagnosis of adult ES can be reached using video-polygraphic monitoring.