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Delta brush variant: A novel ictal EEG pattern in anti-NMDAR encephalitis.

ABSTRACT: Seizure is one of the main symptoms of anti-NMDAR encephalitis, but data of ictal electroencephalogram (EEG) patterns remain limited. In this study, we aimed to introduce a unique ictal pattern. This delta brush variant (DBV) was characterized as generalized delta rhythm with fast spike activity riding on it. We retrospectively evaluated the ictal pattern from six patients with anti-NMDAR encephalitis, and patients were grouped based on the presence of DBV. DBV was found in two patients who were in the florid phase of the disease: (a) A 17-year-old girl experienced rhythmical jerking of bilateral limbs. Corresponding EEG patterns showed generalized DBV. Seizure terminated after intravenous injection of midazolam, but oral-facial dyskinesia reappeared; and (b) a 24-year-old man suffered stiffening of the right limbs and oral-facial dyskinesia. The EEG pattern showed frontal DBV with left prominence. Seizure was controlled, but oral-facial dyskinesia remained after intravenous injection of midazolam. Compared with patients without DBV, patients in this group were more likely to have prolonged excessive delta brush (100% vs 25%) and hyperpyrexia (39.7? vs 38.2?). Duration in ICU (36 days vs 18 days) and hospital (52 days vs 36 days) was relatively longer in DBV group, and no significant difference was found in terms of the mRS score (1 vs 0.5) and seizure relapse rate (0% vs 25%) during 3-month follow-up. DBV is a peculiar pattern in anti-NMDAR encephalitis. An EEG-based monitoring should be considered to avoid misleading this ictal EEG pattern to the electromyographic artifact.

SUBMITTER: Huang Q

PROVIDER: S-EPMC7469758 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Delta brush variant: A novel ictal EEG pattern in anti-NMDAR encephalitis.

Huang Qi Q Liao Yuhan Y Ma Meigang M Wu Yuan Y

Epilepsia open 20200812 3

Seizure is one of the main symptoms of anti-NMDAR encephalitis, but data of ictal electroencephalogram (EEG) patterns remain limited. In this study, we aimed to introduce a unique ictal pattern. This delta brush variant (DBV) was characterized as generalized delta rhythm with fast spike activity riding on it. We retrospectively evaluated the ictal pattern from six patients with anti-NMDAR encephalitis, and patients were grouped based on the presence of DBV. DBV was found in two patients who were ...[more]

PMID: 32913959

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Project description:ObjectiveTo investigate the nature of prodromal headache in anti-NMDA receptor (NMDAR) encephalitis.MethodsRetrospective review of the clinical information of 39 patients with anti-NMDAR encephalitis admitted between January 1999 and September 2017. Five patients with an atypical presentation were excluded. Thus, in 34 patients (median 27 years [range, 12-47 years]; 28 [82%] female), the clinical features were compared between patients who initially reported headache and those who did not report.ResultsTwenty-two patients (65%) reported headache either transiently (n = 5) or continuously (n = 17). Encephalitic symptoms (psychobehavioral memory alterations, seizure, dyskinesias, or altered level of consciousness) developed in 20 patients with median 5.5 days (range, 1-29 days) after headache onset. In one patient, NMDAR antibodies were detected in CSF 3 days after headache onset. Patients with headache had more frequently fever (14/22 [64%] vs. 2/12 [17%] p = 0.013) and higher CSF pleocytosis (median white blood cells 79/?l [range, 6-311/?l] vs. 30/?l [range, 2-69/?l], p = 0.035) than those without headache, but there was no difference in gender, age at onset, seizure, migraine, CSF oligoclonal band detection, elevated IgG index, tumor association, or brain MRI abnormalities between them.ConclusionsHeadache often developed with fever and pleocytosis, but it was rapidly replaced by psychiatric symptoms. Based on current knowledge on the antibody-mediated mechanisms that cause a decrease of synaptic NMDAR through crosslinking and internalization leading to a state mimicking "dissociative anesthesia," we speculated that prodromal headache is not likely caused by direct effect of the autoantibodies but rather meningeal inflammation (noninfectious aseptic meningitis) that occurs in parallel to intrathecal antibody synthesis as an epiphenomenon of NMDAR autoimmunity. Psychobehavioral alterations following headache is an important clue to the diagnosis.

Project description:Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination. Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination. Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid. Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

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Delta brush variant: A novel ictal EEG pattern in anti-NMDAR encephalitis.

Publications

Delta brush variant: A novel ictal EEG pattern in anti-NMDAR encephalitis.

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