Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis.
OBJECTIVE
To describe the characteristics of a previously overlooked devastating epileptic encephalopathy that presents as intractable bilateral perisylvian epilepsy starting with prolonged status epilepticus (SE) in normally developing school-aged children.


METHODS
Retrospective study over 7 years of all normally developing children admitted in our institution for a prolonged SE following non-specific febrile illness with at least one seizure recorded on EEG.


RESULTS
Fourteen children were included at a median age of 7.5 years (4-11) (median follow-up of 4 years (1-7)). Intractable SE lasted 4-60 days (median 30). CSF cell count was normal in five cases and moderately increased in the others. During SE, seizures were recorded in 11 patients and involved temporal lobes in 7; the other 4 patients exhibited perisylvian clinical features with secondary generalization. Intractable epilepsy followed SE in all cases without any latent period. Persisting seizures were recorded in 10 patients and involved temporo-perisylvian regions in 8, frontal regions in 2; 3 others had perisylvian ictal semiology. Spiking was bilateral in 10 cases. MRI showed bilateral hippocampal hypersignal and/or atrophy in 10 cases (extended to the neocortex in 3). All children had major cognitive sequelae. When feasible (six patients), detailed neuropsychology suggested fronto-temporal impairment.


CONCLUSIONS
Among so called grey matter encephalitis patients, we identified a recognizable pattern we propose to call Devastating Epileptic encephalopathy in School-age Children (DESC) that begins with prolonged SE triggered by fever of unknown cause, and persists as intractable perisylvian epilepsy with severe cognitive deterioration.
Mikaeloff
Y
Y
Neuropediatric Department, University Hospital, Bicêtre, France. yann.mikaeloff@tnn.aphp.fr
Jambaqué
I
I
Hertz-Pannier
L
L
Zamfirescu
A
A
Adamsbaum
C
C
Plouin
P
P
Dulac
O
O
Chiron
C
C
D000927:Anticonvulsants / Q000627:therapeutic use; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003071:Cognition / Q000502:physiology*; D004351:Drug Resistance; D004569:Electroencephalography; D004660:Encephalitis / Q000134:cerebrospinal fluid / Q000209:etiology* / Q000503:physiopathology; D004827:Epilepsy / Q000188:drug therapy / Q000209:etiology*; D005335:Fever of Unknown Origin / Q000150:complications; D006801:Humans; D008279:Magnetic Resonance Imaging; D009483:Neuropsychological Tests; D011379:Prognosis; D012189:Retrospective Studies; D013226:Status Epilepticus / Q000150:complications*
D000927:Anticonvulsants; D002556:Cerebrospinal Fluid Proteins