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

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Arjune Sen Patricia Dugan Piero Perucca Daniel Costello Hyunmi Choi Carl Bazil Rod Radtke Danielle Andrade Chantal Depondt Sinead Heavin Jane Adcock W. Owen Pickrell Ronan N. McGinty Fábio Nascimento Philip Smith Mark I. Rees Patrick Kwan Terence J. O’Brien David Goldstein Norman Delanty

https://doi.org/10.1111/epi.14436

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).

Skills

Posted on

25/09/2020