The phenotype of bilateral hippocampal sclerosis and its management in “real life” clinical settings
par Sen, Arjune;Dugan, Patricia;Perucca, Piero;Costello, Daniel;Choi, Hyunmi;Bazil, Carl C.W.;Radtke, Rodney;Andrade, Danielle;Depondt, Chantal ;Heavin, Sinead;Adcock, Jane;Pickrell, William Owen;McGinty, Ronan;Nascimento, Fábio;Smith, Philip;Rees, Mark M.I.;Kwan, Patrick;O'Brien, Terence John;Goldstein, David;Delanty, Norman
Référence Epilepsia, 59, 7, page (1410-1420)
Publication Publié, 2018-07
Référence Epilepsia, 59, 7, page (1410-1420)
Publication Publié, 2018-07
Article révisé par les pairs
Résumé : | Objective: There is little detailed phenotypic characterization of bilateral hippocampal sclerosis (HS). We therefore conducted a multicenter review of people with pharmacoresistant epilepsy and bilateral HS to better determine their clinical characteristics. Methods: Databases from 11 EPIGEN centers were searched. For identified cases, clinicians reviewed the medical notes, imaging, and electroencephalographic (EEG), video-EEG, and neuropsychometric data. Data were irretrievably anonymized, and a single database was populated to capture all phenotypic information. These data were compared with phenotyped cases of unilateral HS from the same centers. Results: In total, 96 patients with pharmacoresistant epilepsy and bilateral HS were identified (43 female, 53 male; age range = 8-80 years). Twenty-five percent had experienced febrile convulsions, and 27% of patients had experienced status epilepticus. The mean number of previously tried antiepileptic drugs was 5.32, and the average number of currently prescribed medications was 2.99; 44.8% of patients had cognitive difficulties, and 47.9% had psychiatric comorbidity; 35.4% (34/96) of patients continued with long-term medical therapy alone, another 4 being seizure-free on medication. Sixteen patients proceeded to, or were awaiting, neurostimulation, and 11 underwent surgical resection. One patient was rendered seizure-free postresection, with an improvement in seizures for 3 other cases. By comparison, of 201 patients with unilateral HS, a significantly higher number (44.3%) had febrile convulsions and only 11.4% had experienced status epilepticus. Importantly, 41.8% (84/201) of patients with unilateral HS had focal aware seizures, whereas such seizures were less frequently observed in people with bilateral HS, and were never observed exclusively (P =.002; Fisher's exact test). Significance: The current work describes the phenotypic spectrum of people with pharmacoresistant epilepsy and bilateral HS, highlights salient clinical differences from patients with unilateral HS, and provides a large platform from which to develop further studies, both epidemiological and genomic, to better understand etiopathogenesis and optimal treatment regimes in this condition. |