Background: In recent years, an increasing number of autoantibodies
(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 (ABSE),
necessitating intensive medical care and administration
of multiple antiepileptic and immunomodulatory treatments
of uncertain effectiveness. Objectives: 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.
Conclusion: 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