Analysis of relapses in anti-NMDAR encephalitis

IDIBAPS August Pi i Sunyer Biomedical Research Institute, Barcino, Catalonia, Spain
Neurology (Impact Factor: 8.29). 08/2011; 77(10):996-9. DOI: 10.1212/WNL.0b013e31822cfc6b
Source: PubMed


The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series of anti-NMDAR encephalitis.
We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously.
A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode.
Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.

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Available from: Iñigo Gabilondo, Nov 14, 2014
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    • "Following this dramatic picture, one to three relapses can occur in patients who improve [2]. The risk for a relaps increases in patients who receive no immunotherapy following the first attack, and who have no tumor [3]. Neuromyelitis optica is an inflammatory, demyelinating disease caused by autoantibodies against a protein so called aquaporin [4] (AQP4) located in the water channels of astrocytes, involving optic nerves and the spinal cord. "
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    DESCRIPTION: Relaps of NMDA Encephalitis Superimposed Upon Neuromiyelitis Optica.
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    • "And in their review of literature, in 109 anti-NMDAR-encephalitis patients elevated cell counts were found in 79/107 (74%) patients and elevated protein counts in 30/101 (30%) patients. while some case series reported slight CSF changes [1] [15] [16]. These studies are consistent in showing that in patients with anti-NMDAR-encephalitis, CSF findings are either normal or exhibit only slight changes. "
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