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Hypothalamic hamartoma: Is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis
Author(s) -
Scholly Julia,
Valenti MariaPaola,
Staack Anke M.,
Strobl Karl,
Bast Thomas,
Kehrli Pierre,
Steinhoff Bernhard J.,
Hirsch Edouard
Publication year - 2013
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/epi.12456
Subject(s) - epileptogenesis , gelastic seizure , hypothalamic hamartoma , epilepsy , anterior temporal lobectomy , temporal lobe , neuropathology , epilepsy surgery , electroencephalography , lesion , medicine , neuroscience , psychology , precocious puberty , surgery , pathology , disease , hormone
Summary Gelastic seizures associated with hypothalamic hamartomas ( HH s) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography ( EEG ) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration. Surgery in adults provides seizure freedom in only one third of patients. The poor results of epilepsy surgery could be explained by an extrahypothalamic epileptogenic zone. The existence of an independent, secondary epileptogenic area with persistent seizures after resection of the presumably primary lesion supports the concept of a “hypothalamic plus” epilepsy. “Hypothalamic plus” epilepsy could be related to either an extrahypothalamic structural lesion (visible on magnetic resonance imaging [ MRI ] or on neuropathology) or if the former is absent, to a functional alteration with enhanced epileptogenic properties due to a process termed secondary epileptogenesis. We report two patients with gelastic seizures with HH (gelastic seizures isolated or associated with dyscognitive seizures of temporal origin). Both patients underwent two‐step surgery: first an endoscopic resection of the HH , followed at a later time by temporal lobectomy. Both patients became seizure‐free only after the temporal lobectomy. In both cases, neuropathology failed to demonstrate a significant structural lesion in the temporal lobe. To our knowledge, for the first time, these two cases suggest the existence of independent secondary epileptogenesis in humans.