Anti-NMDA receptor encephalitis

Anti-NMDA receptor encephalitis
Synonyms	NMDA receptor antibody encephalitis
Classification and external resources
ICD-10	Xxx.x
ICD-9-CM	xxx
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Anti-NMDA receptor encephalitis, also known as NMDA receptor antibody encephalitis, is an acute form of encephalitis that is potentially lethal but has a high probability for recovery with treatment. It is caused by an autoimmune reaction, primarily against the NR1 subunit of the NMDA receptor (N-methyl D-aspartate receptor).

The condition is associated with tumours, mostly teratomas of the ovaries, and thus can be considered a paraneoplastic syndrome. However, there are a substantial number of cases with no detectable tumour, and in fact it appears that most patients do not have a tumour.

The disease was officially categorized and named by Josep Dalmau and colleagues in 2007.

Prior to the development of a symptom complex that is specific to anti-NMDA receptor encephalitis, people may experience prodromal symptoms, including headaches, flu-like illness, or symptoms similar to an upper respiratory infection. These symptoms may be present for weeks or months prior to disease onset. Beyond the prodromal symptoms, the disease progresses at varying rates, and patients may present with a variety of neurologic symptoms. During the initial stage of the disease, symptoms vary slightly between children and adults. However, behavior changes are a common first symptom within both groups. These changes often include agitation, paranoia, psychosis, and violent behaviors. Other common first manifestations include seizures and bizarre movements, mostly of the lips and mouth, but also including pedaling motions with the legs or hand movements resembling playing a piano. Some other symptoms typical during the disease onset include impaired cognition, memory deficits, and speech problems (including aphasia, perseveration or mutism).

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