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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 2 | Page : 398-400
Anti-NMDAR encephalitis combined with a subependymoma
Duan Xiao1, Yihui Lin2, Xiaofeng Wang2, Canhong Yang2, Xiaoyu Huang2, Bo Fu3, Qingzhu Wei3, Tianming Lü2
1 Department of Neurology, The Third Affiliated Hospital of Southern Medical University (Academy of Orthopedics-Guangdong Province); Department of Internal Medicine, Guangdong Women and Children Hospital, Guangdong, China
2 Department of Neurology, The Third Affiliated Hospital of Southern Medical University (Academy of Orthopedics-Guangdong Province), Guangdong, China
3 Department of Pathology, The Third Affiliated Hospital of Southern Medical University (Academy of Orthopedics-Guangdong Province), Guangdong, China
|Date of Web Publication||10-Mar-2017|
Department of Neurology, The Third Affiliated Hospital of Southern Medical University (Academy of Orthopedics-Guangdong Province), 183 Zhongshan Road West, Guangzhou, Guangdong - 510630
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Xiao D, Lin Y, Wang X, Yang C, Huang X, Fu B, Wei Q, Lü T. Anti-NMDAR encephalitis combined with a subependymoma. Neurol India 2017;65:398-400
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a novel NMDAR-mediated form of autoimmune encephalitis. The main clinical manifestations include psychiatric symptoms, epilepsy, movement disorders, disturbance of consciousness, autonomic nervous system disorders, and central hypoventilation. It is necessary to detect anti-NMDAR antibodies as early as possible to confirm the diagnosis of anti-NMDAR encephalitis. Anti-NMDAR encephalitis is usually associated with a teratoma; however, anti-NMDAR encephalitis combined with a subependymoma has still not been reported.
A 34-year-old male patient presented with neurological symptoms of blurred and magnified vision, paroxysmal amaurosis, and malaise. A heterogeneous space-occupying lesion was detected in the patient's right lateral ventricle, with slight hypointensity on T1 weighted imaging (WI), heterogeneous hyperintensity on T2-WI and T2-FLAIR imaging, without gadolinium contrast enhancement [Figure 1]. Anti-NMDAR antibodies were present in the cerebrospinal fluid (CSF), but not in the serum.
|Figure 1: A heterogeneous space-occupying lesion was detected in the patient's right lateral ventricle on MRI. (a) Slight hypointensity on T1-WI. (b) Hyperintensity on T2-WI. (c) Slight hyperintensity on T2-flare. (d) No enhancement with gadolinium contrast on T1-WI|
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Six months after conservative treatment, the patient's symptoms had almost resolved. The mass was removed by surgical resection, and a series of histopathological examinations were performed. Hematoxylin and eosin staining showed that there were clusters of cellular neoplastic proliferation with islands of high nuclear density and abundant fibrillary matrices [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d. Immunofluorescence staining revealed that there were sporadic NMDAR-positive cells distributed in the glial fibrillary acidic protein (GFAP) positive cells and neuropil [Figure 2]e,[Figure 2]f,[Figure 2]g,[Figure 2]h. The tumor was histopathologically confirmed to be a subependymoma (WHO grade 1).
|Figure 2: Histopathological observations. (a) Hematoxylin and eosin staining; (b) GFAP-positive cells; (c) S100-positive cells; (d) Immunopositive reaction; (e) NMDAR-positive cells; (f) GFAP-positive cells; (g) Nuclear staining with DAPI; (h) The merged image (e-g). (Bars = 50 μm)|
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The patient was, therefore, diagnosed as the first case of anti-NMDAR encephalitis associated subependymoma. Anti-NMDAR antibodies were detected in the CSF, but not in the serum, which strongly indicated that the anti-NMDAR antibodies were synthesized intrathecally. As the only intracranial neoplasm expressing immunogenicity to NMDAR in the central nervous system, the presence of a subependymoma may be closely related to the intrathecal composition of anti-NMDAR antibodies. Since the resection of subependymomas, the patient has not had a relapse at 1 year of follow-up observation.
A subependymoma is considered to be a variant or subtype of an ependymoma and may be originate from subependymal cells. The cell of origin of a subependymoma is controversial and its ultrastructural features exhibit both astrocytic and ependymal differentiation. The activated tumor cells of the subependymoma express functional NMDAR. As the patient's immune allergens stimulate the body to produce anti-NMDAR antibodies, anti-NMDAR encephalitis may be induced.
This is the first reported case of anti-NMDAR encephalitis associated with a subependymoma, with NMDAR-positive immunoreactive cells and possible intrathecal anti-NMDAR antibody synthesis. The observations of this case would contribute towards ascertaining the mechanisms of anti-NMDAR encephalitis.
Financial support and sponsorship
National Natural Science Foundation of China (Grant No. 61072033); Natural Science Foundation of Guangdong Province (Grant No. 2014A030313273, 8151051501000053).
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]