Analysis of relapses in anti-NMDAR encephalitis.
ABSTRACT The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series of anti-NMDAR encephalitis.
We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously.
A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode.
Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.
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ABSTRACT: OPINION STATEMENT: Patients with limbic encephalitis and related cortical syndromes develop neurologic and psychiatric symptoms due to autoimmune damage of limbic and extralimbic brain areas. Some patients develop limbic encephalitis as a paraneoplastic manifestation of systemic cancer, where immune tolerance is thought to be lost. Serum and cerebrospinal antibodies against neuronal antigens can be detected in most patients. According to the type of antibodies found, patients may be classified into two broad groups, with implications for neurological and cancer diagnosis, treatment and prognosis. Patients with antibodies to intracellular neuronal antigens (onconeuronal antibodies: Hu, Ma2, amphiphysin, CV2/CRMP5) almost invariably have an underlying neoplasm (lung, testis, breast, etc.), often require aggressive combined antineoplastic and immunomodulatory treatment and their prognosis is usually poor. Patients with antibodies to cell-membrane antigens may present with classical limbic encephalitis (anti-LGI1 antibodies, other) or with a more diffuse stereotyped encephalitis, combining psychiatric symptoms, seizures, dyskinesias, catatonia and central hypoventilation (NMDAR-encephalitis). These patients may or may not have an associated tumor (thymoma, ovarian teratoma, etc.), are likely to improve with immunomodulatory treatment (and tumor removal for paraneoplastic cases) and have, overall, a better prognosis. Immunotherapy in patients with limbic encephalitis may be escalated according to the severity of neurological symptoms, the associated antibodies and the predicted success of antineoplastic treatment. First-line immunotherapies include high dose steroids, intravenous immunoglobulins and plasma exchange. Second-line drugs include Rituximab and Cyclophosphamide. For some patients, maintenance treatment with immunosuppressants might be required to prevent deterioration or relapses. Supportive treatment includes antiepileptic drugs, psychopharmacologic agents and scrutiny and prophylaxis of opportunistic infections.Current Treatment Options in Neurology 12/2012; · 1.94 Impact Factor
Article: Antibodies in epilepsy.[Show abstract] [Hide abstract]
ABSTRACT: Antibody mediated limbic encephalitis is an increasingly recognized cause of seizures in cryptogenic epilepsy. Autoimmune encephalitis and epilepsy have been linked to both neuronal intracellular antibodies (GAD65, ANNA-1, and Ma) and neuronal cell surface antibodies (VGKC complex, NMDAR, AMPA, GABA-B, and GluR5). This article outlines the latest data on these various antibodies with a focus on their association with acute seizures in limbic encephalitis and likely increased risk for chronic epilepsy. There is mounting evidence that these antibodies may play a role in acute onset and chronic seizures in the general epilepsy population without manifesting typical limbic encephalitis symptoms. This review will discuss the data supporting early recognition and treatment options, beyond typical antiepileptic medications, necessary to improve outcomes in this epilepsy subgroup.Current Neurology and Neuroscience Reports 05/2013; 13(5):348. · 3.78 Impact Factor
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ABSTRACT: L’encéphalite aiguë reste une cause fréquente d’admission en réanimation pédiatrique. Bien que la majorité soit d’origine infectieuse, les encéphalites non infectieuses restent mal connues, leur diagnostic et leur traitement souvent retardés. Nous présentons ici les principales causes d’encéphalites aiguës non infectieuses comme l’encéphalomyélite aiguë disséminée, l’encéphalite liée au syndrome d’activation macrophagique, l’encéphalopathie d’Hashimoto, les encéphalites antirécepteurs de l’acide N-méthyl-D-aspartique (NMDA) et les fever-infection related epilepsy syndrome (FIRES)/devastating epilepsy in school-age children (DESC) syndrome.Réanimation 22(2).