Thursday, November 10, 2016

Anti-N-methyl-D-aspartate receptor encephalitis after herpes simplex virus-associated encephalitis

Schein F, Gagneux-Brunon A, Antoine JC, Lavernhe S, Pillet S, Paul S, Frésard A, Boutet C, Grange R, Cazorla C, Lucht F, Botelho-Nevers E. Anti-N-methyl-D-aspartate receptor encephalitis after Herpes simplex virus-associated encephalitis: an emerging disease with diagnosis and therapeutic
challenges. Infection. 2016 Nov 8. [Epub ahead of print]

Morbidity and mortality of Herpes simplex virus encephalitis (HSE) remain high. Relapses of neurological signs may occur after initial clinical improvement under acyclovir treatment.
We report here a case of post-HSE anti-N-methyl-d-aspartate receptor-mediated encephalitis in an adult and perform a systematic search on PubMed to identify other cases in adults.
We identified 11 previously published cases, to discuss diagnostic and therapeutic management. Symptoms in adults are often inappropriate behaviors, confusion and agitation. Diagnosis of anti-NMDA-R encephalitis after HSE is often delayed. Treatment consists in steroids, plasma exchange, and rituximab. Prognosis is often favorable.
Anti-NMDA-R antibodies should be searched in cerebrospinal fluid of patients with unexpected evolution of HS

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[The article below contains the 2 pediatric cases of the 11 previously published cases referred to in the article above.]

Armangue T, Moris G, Cantarín-Extremera V, Conde CE, Rostasy K, Erro ME, Portilla-Cuenca JC, Turón-Viñas E, Málaga I, Muñoz-Cabello B, Torres-Torres C, Llufriu S, González-Gutiérrez-Solana L, González G, Casado-Naranjo I, Rosenfeld M, Graus F, Dalmau J; Spanish Prospective Multicentric Study of Autoimmunity in Herpes Simplex Encephalitis.. Autoimmune post-herpes simplex encephalitis of adults and teenagers. Neurology. 2015 Nov 17;85(20):1736-43.

To report 14 patients with immune-mediated relapsing symptoms post-herpes simplex encephalitis (HSE) and to compare the clinical and immunologic features of the teenage and adult group with those of young children.
Prospective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously.
Among the teenage and adult group (8 patients, median age 40 years, range 13-69; 5 male), 3 had an acute symptom presentation suggesting a viral relapse, and 5 a presentation contiguous with HSE suggesting a recrudescence of previous deficits. Seven patients developed severe psychiatric/behavioral symptoms disrupting all social interactions, and one refractory status epilepticus. Blepharospasm occurred in one patient. Five patients had CSF antibodies against NMDA receptor (NMDAR) and 3 against unknown neuronal cell surface proteins. In 5/6 patients, the brain MRI showed new areas of contrast enhancement that decreased after immunotherapy and clinical improvement. Immunotherapy was useful in 7/7 patients, sometimes with impressive recoveries, returning to their baseline HSE residual deficits. Compared with the 6 younger children (median age 13 months, range 6-20, all with NMDAR antibodies), the teenagers and adults were less likely to develop choreoathetosis (0/8 vs 6/6, p < 0.01) and decreased level of consciousness (2/8 vs 6/6, p < 0.01) and had longer delays in diagnosis and treatment (interval relapse/antibody testing 85 days, range 17-296, vs 4 days, range 0-33, p = 0.037).

In teenagers and adults, the immune-mediated relapsing syndrome post-HSE is different from that known in young children as choreoathetosis post-HSE and is underrecognized. Prompt diagnosis is important because immunotherapy can be highly effective.

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