Analysis of relapses in anti-NMDAR encephalitis.
ABSTRACT 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.
Article: Antibodies in epilepsy.[show abstract] [hide abstract]
ABSTRACT: Antibody mediated limbic encephalitis is an increasingly recognized cause of seizures in cryptogenic epilepsy. Autoimmune encephalitis and epilepsy have been linked to both neuronal intracellular antibodies (GAD65, ANNA-1, and Ma) and neuronal cell surface antibodies (VGKC complex, NMDAR, AMPA, GABA-B, and GluR5). This article outlines the latest data on these various antibodies with a focus on their association with acute seizures in limbic encephalitis and likely increased risk for chronic epilepsy. There is mounting evidence that these antibodies may play a role in acute onset and chronic seizures in the general epilepsy population without manifesting typical limbic encephalitis symptoms. This review will discuss the data supporting early recognition and treatment options, beyond typical antiepileptic medications, necessary to improve outcomes in this epilepsy subgroup.Current Neurology and Neuroscience Reports 05/2013; 13(5):348. · 3.78 Impact Factor
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ABSTRACT: Multiple lines of evidence support the pathogenic role of neuroinflammation in psychiatric illness. While systemic autoimmune diseases are well-documented causes of neuropsychiatric disorders, synaptic autoimmune encephalitides with psychotic symptoms often go under-recognized. Parallel to the link between psychiatric symptoms and autoimmunity in autoimmune diseases, neuroimmunological abnormalities occur in classical psychiatric disorders (for example, major depressive, bipolar, schizophrenia, and obsessive-compulsive disorders). The pathophysiology of these conditions traditionally stressed dysregulation of the glutamatergic and monoaminergic systems, but the mechanisms causing these neurotransmitter abnormalities remained elusive. We review autoimmunity and neuropsychiatric disorders, and the human and experimental evidence supporting the pathogenic role of neuroinflammation in selected classical psychiatric disorders. Understanding how psychosocial, genetic, immunological and neurotransmitter systems interact can reveal pathogenic clues and help target new preventive and symptomatic therapies.Journal of Neuroinflammation 04/2013; 10(1):43. · 4.35 Impact Factor
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ABSTRACT: BACKGROUND Anti- N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune disorder characterized by high intrathecal antibody synthesis. Little is known about the long-term follow-up of the cerebrospinal fluid antibody status. OBJECTIVE To describe persistent intrathecal antibody synthesis in a clinically healthy person 15 years after recovering from anti-NMDAR encephalitis. DESIGN Case report. SETTING Academic medical center. PATIENT A 40-year-old woman who had been diagnosed as having encephalitis of unknown origin in 1995. MAIN OUTCOME MEASURES Clinical evaluation and NMDAR antibody testing. RESULTS On reexamination in 2011, the patient had fully recovered. Investigation of archived as well as follow-up serum and cerebrospinal fluid samples revealed intrathecal synthesis of NMDAR antibodies. CONCLUSIONS This is the longest follow-up on a patient with anti-NMDAR encephalitis. Our findings emphasize that intrathecal antibody synthesis does not necessarily reflect disease activity and that the significance of NMDAR antibody titers needs to be interpreted for each patient according to the clinical context.JAMA neurology. 01/2013; 70(1):117-9.