MY MYSTERIOUS LOST MONTH OF MADNESS: a case of Anti-nmdar Encephalitis Acute Psychosis

My mysterious lost month of madness

I was a happy 24-year-old suddenly stricken by paranoia & seizures. Was I going crazy?

By SUSANNAH CAHALAN
To read Susannah's article go here.




Susannah's story was featured on MSNBC's Medical Mysteries Series.

Dr. Souhel Najjar is the Neurologist who determined the underlying cause to Susannah's psychosis was anti-NMDAR Encephalitis.

Street: 223 East 34th Street
City/state/zip: New York NY 10016
Country: US
Email: Souhel.Najjar@nyumc.org
Phone: +1 646 558 0807
Fax: +1 646 358 7166

ANTI-NMDA RECEPTOR ENCEPHALITIS

Epileptic Disord. 2009 Sep;11(3):267-9. Epub 2009 Aug 28.

Anti-NMDA receptor encephalitis: a video case report.
Labate A, Irani SR, Vincent A, Gambardella A, Piane EL, Cianci V, Aguglia U.

Institute of Neurology, University Magna Graecia, Catanzaro, Italy. a.labate@isn.cnr.it
This report concerns a 26-year-old Italian woman who was given the diagnosis of anti-NMDAR encephalitis after the incidental identification of an ovarian tumour. Neuropsychiatric symptoms and hyperkinetic movements are very commonly seen as initial symptoms of paraneoplastic encephalitis. Interestingly, our patient showed stereotypical movements predominantly located to lower limbs, mimicking a psychogenic seizure. This latter feature further extends the clinical spectrum of dyskinetic movements of anti-NMDAR encephalitis.

PMID: 19713171 [PubMed - indexed for MEDLINE]

ANTI-NMDA RECEPTOR ENCEPHALITIS

Epileptic Disord. 2009 Sep;11(3):261-5. Epub 2009 Sep 7.
Complex partial status epilepticus revealing anti-NMDA receptor encephalitis.

Bayreuther C, Bourg V, Dellamonica J, Borg M, Bernardin G, Thomas P.

Department of Neurology, University of Nice Sophia-Antipolis, Pasteur Hospital, Nice, France.
Encephalitis with anti-NMDA receptor antibodies is a recently-recognised form of paraneoplastic encephalitis characterized by a prodromal phase of unspecific illness with fever resembling viral disease, followed by memory loss, psychiatric features, seizures, disturbed consciousness, prominent abnormal movements and autonomic imbalance. Association with ovarian teratoma is common. Neurological outcome can be good, especially when surgery is performed at an early stage. Here, we report a case of anti-NMDA receptor encephalitis associated with ovarian teratoma presenting with inaugural complex partial status epilepticus. The nature of abnormal movements at early stages was unclear and abnormal movements were misinterpreted as the recurrence of partial epileptic seizures. Despite its rarity, all clinicians treating epilepsy and movement disorders should be familiar with anti-NMDA receptor encephalitis, that appears to be a very severe but curable disease.

PMID: 19736168 [PubMed - indexed for MEDLINE]

ACUTE PSYCHOTIC MANIA: ANTI-NMDA ENCEPHALOPATHY

Med J Aust. 2009 Sep 7;191(5):284-6.
Acute psychiatric illness in a young woman: an unusual form of encephalitis.

Parratt KL, Allan M, Lewis SJ, Dalmau J, Halmagyi GM, Spies JM.
Department of Neurology, Royal Prince Alfred Hospital, Sydney, NSW. drksharp@bigpond.net.au

A 21-year-old woman was admitted to hospital with a diagnosis of acute psychotic mania, but developed, over approximately 6 weeks, seizures, delirium, catatonia, movement disorder and autonomic dysfunction. She was found to have antibodies to N-methyl-D-aspartate (NMDA) NR1-NR2 receptors in both serum and cerebrospinal fluid, consistent with anti-NMDA-receptor encephalitis, a severe, potentially lethal but treatment-responsive encephalitis often associated with ovarian tumour. With aggressive immunotherapy and bilateral oophorectomy, she recovered over a period of 14 months from her initial presentation. No ovarian tumour was identified.

PMID: 19740054 [PubMed - indexed for MEDLINE]

ANTI-NMDA ENCEPHALITIS

Med Mal Infect. 2009 Nov 24. [Epub ahead of print]

[Anti-NMDA-receptor encephalitis.]
[Article in French]
de Broucker T, Martinez-Almoyna L.

Service de neurologie, hôpital Delafontaine, 2, rue du Dr-Delafontaine, 93200 Saint-Denis, France.

Anti-NMDA-receptor encephalitis has been described only recently among other causes of paraneoplastic and auto-immune limbic encephalitis. Its frequency is probably underestimated. The very characteristic clinical presentation, the severity of symptoms frequently leading to the intensive care unit, the therapeutic implications of the diagnosis whatever the cause, paraneoplastic or not and, once treated, the possibility of a full recovery or mild sequels in the majority of cases justify a surveillance either in neurology wards or in infectious, psychiatric, intensive care, or pediatric units. The authors review the history of this disease, the available epidemiological data, the characteristic clinical presentation of patients, the differential diagnosis, and the suggested treatment according to an up-to-date literature review.

PMID: 19942390 [PubMed - as supplied by publisher]

SEVERE CHILDHOOD ENCEPHALOPATHY

Dev Med Child Neurol. 2009 Dec 23. [Epub ahead of print]
Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-d-aspartate receptor encephalitis.
Poloni C, Korff CM, Ricotti V, King MD, Perez ER, Mayor-Dubois C, Haenggeli CA, Deonna T.

Department of Paediatrics, Child Neurology, University Hospital Lausanne, Lausanne, Switzerland.

Aim We report four cases of acquired severe encephalopathy with massive hyperkinesia, marked neurological and cognitive regression, sleep disturbance, prolonged mutism, and a remarkably delayed recovery (time to full recovery between 5 and 18mo) with an overall good outcome, and its association with anti-N-methyl-d-aspartate (anti-NMDA) receptor antibodies. Method We reviewed the four cases retrospectively and we also reviewed the literature. Results Anti-NMDA receptor antibodies (without ovarian teratoma detected so far) were found in the two children tested in this study. Interpretation The clinical features are similar to those first reported in 1992 by Sebire et al.,(1) and rarely recognized since. Sleep disturbance was not emphasized as part of the disorder, but appears to be an important feature, whereas coma is less certain and difficult to evaluate in this setting. The combination of symptoms, evolution (mainly seizures at onset), severity, paucity of abnormal laboratory findings, very slow recovery, and difficult management justify its recognition as a specific entity. The neuropathological substrate may be anatomically close to that involved in encephalitis lethargica, in which the same target functions (sleep and movement) are affected but in reverse, with hypersomnolence and bradykinesia. This syndrome closely resembles anti-NMDA receptor encephalitis, which has been reported in adults and is often paraneoplastic.

PMID: 20041934 [PubMed - as supplied by publisher]

PROMINENT PSYCHIATRIC SYMPTOMS IN ANTI-NMDAR ENCEPHALITIS

Eur J Clin Microbiol Infect Dis. 2009 Dec;28(12):1421-9. Epub 2009 Aug 29.

Anti-NMDA receptor encephalitis: report of ten cases and comparison with viral encephalitis.
Gable MS, Gavali S, Radner A, Tilley DH, Lee B, Dyner L, Collins A, Dengel A, Dalmau J, Glaser CA.

Department of Psychiatry, University of California San Francisco, Fresno, CA, USA. ms2gable@yahoo.com

The California Encephalitis Project (CEP), established in 1998 to explore encephalitic etiologies, has identified patients with N-methyl-D-aspartate receptor (NMDAR) antibodies, the likely etiology of their encephalitis. This study compares the presentation of such patients to those with viral encephalitis, so that infectious disease clinicians may identify individuals with this treatable disorder. Patients were physician-referred, and standardized forms were used to gather demographic, clinical, and laboratory data. Features of anti-NMDAR+ patients were compared with the viral encephalitides of enteroviral (EV), rabies, and herpes simplex-1 (HSV-1) origins. Sixteen cases with confirmed viral etiologies were all negative on NMDAR antibody testing. Ten anti-NMDAR+ patients were profiled with a median age of 18.5 years (range 11-31 years). None were Caucasian. They had a characteristic progression with prominent psychiatric symptoms, autonomic instability, significant neurologic abnormalities, and seizures. Two had a teratoma, and, of the remaining eight, four had serologic evidence of acute Mycoplasma infection. The clinical and imaging features of anti-NMDAR+ patients served to differentiate this autoimmune disorder from HSV-1, EV, and rabies. Unlike classic paraneoplastic encephalitis, anti-NMDAR encephalitis affects younger patients and is often treatable. The association of NMDAR antibodies in patients with possible Mycoplasma pneumoniae infection warrants further study.

PMID: 19718525 [PubMed - in process]