Cerebrospinal fluid MOG-antibodies in anti-NMDA receptor encephalitis with leptomeningeal enhancement

Abstract Background Magnetic resonance imaging findings of anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis have been reported to lack specificity. The hippocampus, frontal lobe, white matter, basal ganglia, and even spinal cord can be involved. However, isolated leptomeningeal enhancement is rare in anti-NMDA receptor encephalitis. Case presentation We report a 17-year-old boy who presented with a 2-week history of mild fever and progressive encephalopathy with behavioral and psychological abnormalities. Finally, a diagnosis of anti-NMDA receptor encephalitis was made. Initial magnetic resonance imaging results revealed diffuse contrast enhancement of the leptomeninges without parenchymal lesions. After treatment with high-dose intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin, the enhancement showed substantially decrease at the 3-week follow-up. Conclusions This case is unusual because isolated leptomeningeal enhancement is rare in anti-NMDA receptor encephalitis. The present case suggests that isolated diffuse leptomeningeal enhancement could be one of the imaging findings in patients with anti-NMDA receptor encephalitis.

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ENSEFALITIS ANTIRESEPTOR NMDA: ENSEFALITIS DENGAN GEJALA AWAL PSIKOSIS

Majalah Kedokteran Neurosains Perhimpunan Dokter Spesialis Saraf Indonesia ◽

10.52386/neurona.v35i1.35 ◽

2020 ◽

Vol 35 (1) ◽

Author(s):

Riwanti Estiasari ◽

Darma Imran ◽

Kartika Maharani ◽

David Pangeran ◽

Fitri Octaviana ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Nmda Receptor ◽

Psychiatric Symptom ◽

Autoimmune Encephalitis ◽

Antibody Test ◽

The Other ◽

Receptor Antibody ◽

The Third ◽

Nmda Receptor Encephalitis ◽

Cognitive Disturbance

ANTI-NMDA RECEPTOR ENCEPHALITIS: ENCEPHALITIS PRESENTING WITH EARLY PSYCHOSIS SYMPTOMSABSTRACTAnti N-methyl-D-aspartate (NMDA) receptor encephalitis is seldom found in Indonesia. The atypical initial presentation of psychosis and cognitive disturbance results in underdiagnosed, or even undiagnosed case. This report described a 12-year-old girl presenting with initial psychiatric symptom followed by seizure and oropharyngeal dystonia. Anti-NMDA receptor antibody test carried out on both serum and cerebrospinal fluid (CSF) were positive and no other pathogen was detected in the CSF. Clinical improvement was seen following the third day of methylprednisolone administration. This case demonstrates the importance of considering anti-NMDA receptor encephalitis or the other autoimmune encephalitis as the differential of infective encephalitis. Anti-NMDA receptor antibody test, which can already be performed in Indonesia, will greatly guide to the definitive diagnosis.Keywords: Encephalitis, NMDA, oropharyngeal dystonia, psychiatricABSTRAKEnsefalitis antireseptor N-metil-D-aspartat (NMDA) masih jarang ditemukan di Indonesia. Gejala awal yang tidak khas berupa psikosis dan gangguan kognitif menyebabkan keterlambatan diagnosis, atau bahkan tidak terdiagnosis, ensefalitis jenis ini. Berikut dilaporkan kasus seorang perempuan 12 tahun dengan gejala awal berupa gejala psikiatrik yang diikuti dengan kejang dan munculnya distonia orofaring. Pemeriksaan antibodi antireseptor NMDA pada serum dan cairan serebrospinal (CSS) positif dan tidak ditemukan patogen lain pada pemerikaan CSS. Perbaikan klinis terlihat setelah pemberian metilprednisolon selama 3 hari. Kasus ini memperlihatkan pentingnya mempertimbangkan diagnosis ensefalitis antireseptor NMDA atau ensefalitis autoimun lainnya sebagai diagnosis banding pada kasus ensefalitis infeksi. Oleh karena itu, pemeriksaan antibodi antireseptor NMDA yang telah dapat dilakukan di Indonesia akan sangat membantu proses penegakan diagnosis.Kata kunci: Distonia orofaring, ensefalitis, NMDA, psikiatrik

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Suppression of synaptic plasticity by cerebrospinal fluid from anti-NMDA receptor encephalitis patients

Neurobiology of Disease ◽

10.1016/j.nbd.2011.09.019 ◽

2012 ◽

Vol 45 (1) ◽

pp. 610-615 ◽

Cited By ~ 63

Author(s):

Qing Zhang ◽

Keiko Tanaka ◽

Peng Sun ◽

Michiyo Nakata ◽

Ryo Yamamoto ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Synaptic Plasticity ◽

Nmda Receptor ◽

Nmda Receptor Encephalitis

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Rate of Leptomeningeal Enhancement in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody–Associated Encephalomyelitis

Journal of Child Neurology ◽

10.1177/08830738211025867 ◽

2021 ◽

Vol 36 (11) ◽

pp. 1042-1046

Author(s):

Judith A. Gadde ◽

David S. Wolf ◽

Stephanie Keller ◽

Grace Y. Gombolay

Keyword(s):

Cerebrospinal Fluid ◽

Blood Cell ◽

Myelin Oligodendrocyte Glycoprotein ◽

Demyelinating Diseases ◽

Blood Cell Count ◽

Leptomeningeal Enhancement ◽

Cortical Edema ◽

Demyelinating Lesions ◽

Mog Antibodies ◽

Cerebrospinal Fluid Pleocytosis

Introduction: Myelin oligodendrocyte glycoprotein antibodies (MOG-abs) are associated with demyelinating diseases. Leptomeningeal enhancement occurs in 6% of adult MOG-abs patients but rates in pediatric MOG-abs patients are unknown. Methods: Retrospective review of pediatric MOG-abs patients was performed. Results: Twenty-one patients (7 boys, 14 girls) were included with an average age of 8.6 years (range 2-15 years). Seven of 21 (33%) pediatric MOG-abs patients had leptomeningeal enhancement. Two patients’ relapses were manifested by leptomeningeal enhancement alone and another patient presented with seizures, encephalopathy, and aseptic meningitis without demyelinating lesions. Cerebrospinal fluid pleocytosis was seen in both leptomeningeal (4/7 patients) and nonleptomeningeal enhancement (10/14 patients). Interestingly, 3 patients with leptomeningeal enhancement had normal cerebrospinal fluid white blood cell count. Cortical edema was more likely in patients with leptomeningeal enhancement ( P = .0263). Conclusion: We expand the clinical spectrum of anti-MOG antibody–associated disorder. Patients with recurrent leptomeningeal enhancement without demyelinating lesions should be tested for MOG antibodies.

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Anti-NMDA Receptor Encephalitis Presenting as a Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL) (P04.251)

Neurology ◽

10.1212/wnl.78.1_meetingabstracts.p04.251 ◽

2012 ◽

Vol 78 (Meeting Abstracts 1) ◽

pp. P04.251-P04.251 ◽

Cited By ~ 2

Author(s):

J. Garcia-Monco ◽

M. Gomez Beldarrain ◽

I. Garcia Gorostiaga ◽

I. Kortazar

Keyword(s):

Cerebrospinal Fluid ◽

Nmda Receptor ◽

Neurological Deficits ◽

Nmda Receptor Encephalitis

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Anti-NMDAR encephalitis mimicking HaNDL syndrome

Cephalalgia ◽

10.1177/0333102414526070 ◽

2014 ◽

Vol 34 (12) ◽

pp. 1012-1014 ◽

Cited By ~ 14

Author(s):

Carsten Finke ◽

Annerose Mengel ◽

Harald Prüss ◽

Winfried Stöcker ◽

Andreas Meisel ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Nmda Receptor ◽

Diagnostic Criteria ◽

Clinical Picture ◽

Clinical Phenotype ◽

Neurological Deficits ◽

Igg Antibodies ◽

Nmda Receptor Encephalitis ◽

Csf Analysis ◽

Nmdar Encephalitis

Background Anti-NMDA receptor encephalitis typically manifests as severe multistage neuropsychiatric syndrome. However, milder or incomplete forms of the disorder have been recognised. Here, we report on a patient with anti-NMDA receptor encephalitis with a clinical phenotype mimicking the syndrome of headache with neurological deficits and cerebrospinal fluid (CSF) lymphocytosis (HaNDL). Case A 67-year-old man presented with recurrent stereotyped episodes of hemianopia, aphasia and right hemiparesis accompanied by throbbing headaches as well as confusion and agitation. CSF analysis showed lymphocytic pleocytosis. Additional analysis revealed NMDA receptor IgG antibodies in the patient’s CSF. Following immunotherapy, no further episodes occurred and NMDAR antibodies became undetectable. No NMDAR or other neuronal antibodies were detected in archived serum and CSF samples of 12 HaNDL patients fulfilling the current diagnostic criteria. Conclusions While anti-NMDAR encephalitis can manifest with a HaNDL-like clinical picture, HaNDL syndrome itself does not appear to be mediated by anti-NMDAR antibodies.

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