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]

Abstract

INTRODUCTION:

Morbidity and mortality of Herpes simplex virus encephalitis (HSE) remain high. Relapses of neurological signs may occur after initial clinical improvement under acyclovir treatment.

METHODS:

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.

RESULTS:

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.

CONCLUSION:

Anti-NMDA-R antibodies should be searched in cerebrospinal fluid of patients with unexpected evolution of HS

Courtesy of:   https://www.mdlinx.com/neurology/medical-news-article/2016/11/10/herpes-simplex-virusencephalitisanti-nmda-receptor-antibodies/6939046/?category=latest&page_id=3

[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.

Abstract

OBJECTIVE:

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.

METHODS:

Prospective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously.

RESULTS:

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).

CONCLUSION:

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.