The phenotype of bilateral hippocampal sclerosis and its management in “real life” clinical settings

Sen, Arjune, Dugan, Patricia, Perucca, Piero, Costello, Daniel, Choi, Hyunmi, Bazil, Carl, Radtke, Rod, Andrade, Danielle, Depondt, Chantal, Heavin, Sinead, Adcock, Jane, Pickrell, W. Owen, McGinty, Ronan N., Nascimento, Fábio, Smith, Philip ORCID: https://orcid.org/0000-0003-4250-2562, Rees, Mark I., Kwan, Patrick, O'Brien, Terence J., Goldstein, David and Delanty, Norman 2018. The phenotype of bilateral hippocampal sclerosis and its management in “real life” clinical settings. Epilepsia 59 (7) , pp. 1410-1420. 10.1111/epi.14436

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Abstract

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.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine
Publisher:	Wiley
ISSN:	0013-9580
Date of Acceptance:	8 May 2018
Last Modified:	09 Nov 2022 10:19
URI:	https://orca.cardiff.ac.uk/id/eprint/139075

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