Ovarian Teratoma Associated with Coexisting Anti-N-Methyl-D-Aspartate Receptor and Glial Fibrillary Acidic Protein Autoimmune Meningoencephalitis in an Adolescent Girl: A Case Report

Martin, Alexandra; Jolliffe, Evan; Hertweck, S. Paige

doi:10.1016/j.jpag.2017.12.009

Cited by 20 publications

(15 citation statements)

References 7 publications

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“…The only common oncological association was ovarian teratoma, which occurred in 75% of paraneoplastic cases (usually mature teratoma). Other cancers are rare and diverse: adenocarcinomas (of breast, lung, ovary, endometrium, esophagus, and kidney), head and neck squamous cell carcinoma, thymoma, pleomorphic parotid adenoma, gliomas, multiple myeloma, small-cell carcinoma, and carcinoid [2 ▪ ,4 ▪▪ ,8 ▪ ,9 ▪ ,15]. It was also of interest that a patient ultimately determined to have temporal lobe astrocytoma (initially suspected to be encephalitis) had GFAP-IgG detected in CSF [4 ▪▪ ].…”

Section: Potential Etiologic Clues: Coexisting Autoimmunity Cancersmentioning

confidence: 99%

Autoimmune glial fibrillary acidic protein astrocytopathy

Kunchok¹,

Ζεκερίδου

McKeon

2019

Current Opinion in Neurology

175

223

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Purpose of review To describe a recently characterized autoimmune, inflammatory central nervous system (CNS) disorder known as autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Recent findings Affected patients present with symptoms of one or more of meningitis (headache and neck ache), encephalitis (delirium, tremor, seizures, or psychiatric symptoms), and myelitis (sensory symptoms and weakness). Optic disc papillitis (blurred vision) is common. CNS inflammation is evident in characteristic T1 postgadolinium enhancement of GFAP-enriched CNS regions, and lymphocytic cerebrospinal fluid (CSF) white cell count elevation. CSF is more reliable than serum for GFAP-immunoglobulin G (IgG) testing. Ovarian teratoma commonly coexists, particularly among patients with accompanying N-methyl-D-aspartate receptor or aquaporin-4 autoimmunity. Parainfectious autoimmunity is suspected in some other patients, though the culprit organism is rarely verified. Pathophysiologic relevance of T cells is underscored by neuropathology and cases of dysregulated T-cell function (HIV or checkpoint inhibitor cancer therapy). Corticosteroid-responsiveness is a hallmark of the disease. Relapses occur in approximately 20% of patients, necessitating transition to a steroid-sparing drug. Reported outcomes vary, though in the authors’ experience, early and sustained intervention usually portends recovery. Summary Autoimmune GFAP astrocytopathy is a treatable autoimmune CNS disease diagnosable by GFAP-IgG testing in CSF. This disease presents opportunities to explore novel mechanisms of CNS autoimmunity and inflammation.

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Section: Potential Etiologic Clues: Coexisting Autoimmunity Cancersmentioning

confidence: 99%

Autoimmune glial fibrillary acidic protein astrocytopathy

Kunchok¹,

Ζεκερίδου

McKeon

2019

Current Opinion in Neurology

175

223

View full text Add to dashboard Cite

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“…In this study 17 percent of the patients with a positive NMDA receptor antibody had an ovarian teratoma, which is significantly lower than in other published series; some epidemiological studies suggested that this association depends on demographic features of the population analyzed [15]. Case reports and observational studies have estimated that 36%-50% of patients with anti-NMDA receptor encephalitis have an ovarian teratoma [16, 17]. Gresa-Arribas et al reported more frequently NMDA receptor antibodies in patients who had an underlying teratoma than in those who did not have a tumour [9].…”

Section: Discussionmentioning

confidence: 73%

Anti-N-Methyl-D-Aspartate Encephalitis as Paraneoplastic Manifestation of Germ-Cells Tumours: A Cases Report and Literature Review

Rita

Nieto-Gañán

Escrig

et al. 2019

Case Reports in Immunology

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Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common form of autoimmune encephalitis, caused by the interaction between an antibody and its target, located on glutamate receptor type N-methyl-D-aspartate (NMDA) of neuronal surface. There is a wide spectrum of clinical features starting by a viral-like prodrome, followed by symptoms such as psychosis, aggressive behaviour, memory loss, seizures, movement disorders, and autonomic instability. Up to 50% of the affected young female patients have germ-cells tumours as ovarian teratoma, making it essential to establish an early diagnosis through detection of specific antibodies in serum and cerebrospinal fluid (CSF). This retrospective observational study was performed in patients whom positive anti-NMDA receptor antibodies have been tested, associated with clinical manifestations that suggest autoimmune encephalitis and a germ-cell tumour confirmed by pathology. Six patients have tested positive for anti-NMDA receptor antibodies associated with a germ-cell tumour and clinical manifestations of autoimmune encephalitis. Management includes aggressive immunosuppression and surgical removal.

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“…An ovarian teratoma associated with coexisting NMDA‐R and GFAP autoimmunity in an adolescent girl had extensive CD3 + T‐cell infiltrate . In another report, an ovarian teratoma from a patient with GFAP‐A with coexisting NMDA‐R‐IgG had robust GFAP staining and sparse NMDA‐R staining …”

Section: Neuropathologymentioning

confidence: 97%

Autoimmune glial fibrillary acidic protein astrocytopathy

Kimura

Takekoshi

Yagi

et al. 2019

Clinical & Exp Neuroim

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Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP‐A) was recently reported as a spectrum of autoimmune inflammatory central nervous system disorders characterized by the detection of immunoglobulin G (IgG) against GFAP in cerebrospinal fluid using tissue‐ and cell‐based testing. The most common phenotypes of GFAP‐A are meningoencephalitis with or without myelitis. Patients present with headache and fever followed by consciousness disturbance and meningeal signs. Movement disorders, ataxia, hyperreflexia and blurred vision related to optic disc edema are sometimes observed. Some patients with GFAP‐A have a neoplasm. The most commonly detected neoplasm is ovarian teratoma. Coexisting neural antibodies are also sometimes detected. N‐methyl‐D‐aspartate receptor (NMDA‐R)‐IgG is the most common coexisting antibody, and patients with coexisting N‐methyl‐D‐aspartate receptor‐IgG have a clinical course resembling N‐methyl‐D‐aspartate receptor encephalitis. Lymphocytic pleocytosis lasts for several months and brain linear perivascular radial gadolinium‐enhancement patterns, an imaging hallmark of GFAP‐A, are sometimes observed. A previous neuropathological study reported inflammatory infiltrates including T‐, B‐ and plasma cells surrounding blood vessels. Although the pathophysiological mechanisms of GFAP‐A remain to be elucidated, GFAP‐specific CD8+ T cells are likely effectors of this disorder. GFAP‐A is usually corticosteroid responsive in the acute stage. However, some patients are prone to relapse or even die. In relapsed or refractory patients, the administration of oral prednisolone or a steroid‐sparing agent might be necessary. Because the timing of corticosteroid therapy might affect patient prognosis, cerebrospinal fluid GFAP‐IgG should be examined in patients with meningoencephalitis with or without myelitis who present with characteristic clinical features.

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Ovarian Teratoma Associated with Coexisting Anti-N-Methyl-D-Aspartate Receptor and Glial Fibrillary Acidic Protein Autoimmune Meningoencephalitis in an Adolescent Girl: A Case Report

Cited by 20 publications

References 7 publications

Autoimmune glial fibrillary acidic protein astrocytopathy

Autoimmune glial fibrillary acidic protein astrocytopathy

Anti-N-Methyl-D-Aspartate Encephalitis as Paraneoplastic Manifestation of Germ-Cells Tumours: A Cases Report and Literature Review

Autoimmune glial fibrillary acidic protein astrocytopathy

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