Brain on Fire - Anti-NMDA Receptor Encephalitis

Short Attention Span Summary

Brain on Fire
Anti-NMDA receptor encephalitis is an emerging condition that is being recognized more often.  This is a fairly large case series of the ICU management of 77 patients.  First, a brief primer on what it is.  It's an neuroautoimmune disease associated with teratomas in 60% and is more common in young women.  It presents with 4-5 days of fever, malaise, poor concentration, nausea, diarrhea, vomiting, or headache.  The illness progresses to psychosis or seizures.  Then unresponsiveness follows and may present as catatonia or mutism, often accompanied by autonomic dysregulation.  It looks like a person with encephalitis, with more psychiatric overtones.  The diagnosis is made with CSF anti-NMDAR antibody testing.  It's treated with supportive critical care and immunotherapy.

In this study, patients were treated with steroids, IVIG, or plasmapheresis, and less often, cyclophosphamide or rituximab.  At 6-month follow up, 57% had a good neurological outcome.  The prognosis was better in those treated early with combined immunotherapy.

Spoon Feed
Early treatment of anti-NMDA receptor encephalitis improved recovery.  But first, you have to make the diagnosis.  Corey Slovis always says, "the eye doesn't see what the mind doesn't know."  ALiEM has a brief, excellent summary.  Also, the book Brain on Fire* gives a patient's first-hand account of her experience with this disease.


Abstract

Am J Respir Crit Care Med. 2017 Feb 15;195(4):491-499. doi: 10.1164/rccm.201603-0507OC.

Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Adult Patients Requiring Intensive Care.

de Montmollin E1, Demeret S2, Brulé N3, Conrad M4, Dailler F5, Lerolle N6, Navellou JC7, Schwebel C8, Alves M9, Cour M10, Engrand N11, Tonnelier JM12, Maury E13, Ruckly S14, Picard G15, Rogemond V15, Magalhaes É16, Sharshar T17, Timsit JF16,14, Honnorat J15,18, Sonneville R16,19; ENCEPHALITICA Study Group ‡.

Author information:

11 Polyvalent Intensive Care Unit, Centre Hospitalier de Saint-Denis, Saint-Denis, France.

22 Neurologic Intensive Care Unit, Hôpital Pitié-Salpêtrière.

33 Medical Intensive Care Unit, Centre Hospitalier Universitaire de Nantes, Nantes, France.

44 Medical Intensive Care Unit, Centre Hospitalier Universitaire de Nancy, Nancy, France.

55 Neurologic Intensive Care Unit, Hôpital Pierre Wertheimer, Groupement Hospitalier Est, Hospices Civiles de Lyon, Lyon, France.

66 Department of Medical Intensive Care and Hyperbaric Medicine, Centre Hospitalier Universitaire d'Angers, Angers, France.

77 Medical Intensive Care Unit, Centre Hospitalier Universitaire Jean Minjoz, Besançon, France.

88 Medical Intensive Care Unit, Hôpital Universitaire Albert Michallon, Grenoble, France.

99 Polyvalent Intensive Care Unit, Centre Hospitalier Intercommunal de Poissy-Saint-Germain-en-Laye, Poissy, France.

1010 Medical Intensive Care Unit, Groupement Hospitalier Edouard Herriot, Hospices Civiles de Lyon, Lyon, France.

1111 Neurologic Intensive Care Unit, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France.

1212 Medical Intensive Care Unit, Hôpital de la Cavale Blanche, Centre Hospitalier Universitaire Régional de Brest, Brest, France.

1313 Medical Intensive Care Unit, Hôpital Saint-Antoine.

1414 Unité Mixte de Recherche (UMR) 1137, Infection Antimicrobials Modelling Evolution Team 5, DeSCID: Decision SCiences in Infectious Diseases, Control and Care, Institut National de la Santé et de la Recherche Médicale (INSERM), and.

1515 French National Reference Centre for Paraneoplastic Neurologic Syndromes, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; and.

1616 Medical and Infectious Diseases Intensive Care Unit, Hôpital Bichat-Claude-Bernard, and.

1717 Polyvalent Intensive Care Unit, Hôpital Raymond Poincaré, Assistance Publique-Hôpitaux de Paris, Paris, France.

1818 Institut NeuroMyoGene, INSERM U1217/Centre National de la Recherche Scientifique (CNRS), UMR 5310, Lyon, France.

1919 INSERM U1148, Université Paris Diderot, Sorbonne Paris Cité, Paris, France.

Abstract

RATIONALE:

Encephalitis caused by anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is the leading cause of immune-mediated encephalitis. There are limited data on intensive care unit (ICU) management of these patients.

OBJECTIVES:

To identify prognostic factors of good neurologic outcome in patients admitted to an ICU with anti-NMDAR encephalitis.

METHODS:

This was an observational multicenter study of all consecutive adult patients diagnosed with anti-NMDAR encephalitis at the French National Reference Centre, admitted to an ICU between 2008 and 2014. The primary outcome was a good neurologic outcome at 6 months after ICU admission, defined by a modified Rankin Scale score of 0-2.

MEASUREMENTS AND MAIN RESULTS:

Seventy-seven patients were included from 52 ICUs. First-line immunotherapy consisted of steroids (n = 61/74; 82%), intravenous immunoglobulins (n = 71/74; 96%), and plasmapheresis (n = 17/74; 23%). Forty-five (61%) patients received second-line immunotherapy (cyclophosphamide, rituximab, or both). At 6 months, 57% of patients had a good neurologic outcome. Independent factors of good neurologic outcome were early (≤8 d after ICU admission) immunotherapy (odds ratio, 16.16; 95% confidence interval, 3.32-78.64; for combined first-line immunotherapy with steroids and intravenous immunoglobulins vs. late immunotherapy), and a low white blood cell count on the first cerebrospinal examination (odds ratio, 9.83 for <5 vs. >50 cells/mm3; 95% confidence interval, 1.07-90.65). Presence of nonneurologic organ failures at ICU admission and occurrence of status epilepticus during ICU stay were not associated with neurologic outcome.

CONCLUSIONS:

The prognosis of adult patients with anti-NMDAR encephalitis requiring intensive care is good, especially when immunotherapy is initiated early, advocating for prompt diagnosis and early aggressive treatment.

PMID: 27552490 [PubMed - in process]

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