Antibody-mediated status epilepticus: a retrospective multicenter survey.
BACKGROUND
In recent years, an increasing number of auto-antibodies (AB) have been detected in the CSF and serum of patients with new onset epilepsy. Some of these patients develop convulsive or nonconvulsive status epilepticus (AB-SE), necessitating intensive medical care and administration of multiple antiepileptic and immunomodulatory treatments of uncertain effectiveness.


OBJECTIVE
In this retrospective multicenter survey we aimed to determine the spectrum of gravity, the duration and the prognosis of the disorder. In addition, we sought to identify the antibodies associated with this condition, as well as determine whether there is a most effective treatment regime.


METHODS
12 European Neurology University Clinics, with extensive experience in the treatment of SE patients, were sent a detailed questionnaire regarding symptoms and treatment of AB-SE patients. Seven centers responded positively, providing a total of 13 patients above the age of 16.


RESULTS
AB-SE affects mainly women (12/13, 92%) with a variable age at onset (17-69 years, median: 25 years). The duration of the disease is also variable (10 days to 12 years, median: 2 months). Only the 3 oldest patients died (55-69 years). Most patients were diagnosed with anti NMDAR encephalitis (8/13) and had oligoclonal bands in the CSF (9/13). No specific treatment regimen (antiepileptic, immunomodulatory) was found to be clearly superior. Most of the surviving 10 patients (77%) recovered completely or nearly so within 2 years of index poststatus.


CONCLUSIONS
AB-SE is a severe but potentially reversible condition. Long duration does not seem to imply fatal outcome; however, age older than 50 years at time of onset appears to be a risk factor for death. There was no evidence for an optimal antiepileptic or immunomodulatory treatment. A prospective multicenter study is warranted in order to stratify the optimal treatment algorithm, determine clear risk factors of unfavorable outcome and long-term prognosis.
Department of Neurology, University Hospital, Geneva, Switzerland. franz-josef.holzer@hcuge.ch
Franz Josef
FJ
Holzer
Andrea O
AO
Rossetti
Anne-Chantal
AC
Heritier-Barras
Dominik
D
Zumsteg
Robert
R
Roebling
Roman
R
Huber
Holger
H
Lerche
Ines C
IC
Kiphuth
Jürgen
J
Bardutzky
Christian G
CG
Bien
Mathias
M
Tröger
Gaby
G
Schoch
Harald
H
Prüss
Margitta
M
Seeck
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012640:Seizures / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D013226:Status Epilepticus / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D055815:Young Adult
D001323:Autoantibodies