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Anti-N-methyl-D-aspartate receptor encephalitis concomitant with multifocal subcortical white matter lesions on magnetic resonance imaging: a case report and review of the literature.

Wang RJ, Chen BD, Qi D - BMC Neurol (2015)

Bottom Line: Identification of anti-N-methyl-D-aspartate receptor antibodies in serum and cerebrospinal fluid confirmed the diagnosis of anti-N-methyl-D-aspartate receptor encephalitis.Anti-N-methyl-D-aspartate receptor encephalitis may be concomitant with multifocal subcortical white matter lesions.Such lesions may resolve after appropriate immunotherapy.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong-An Road, Xicheng District, Beijing, 100050, People's Republic of China. wangrj73@sina.com.

ABSTRACT

Background: Anti-N-methyl-D-aspartate receptor encephalitis is a severe autoimmune disorder characterized by severe psychiatric symptoms, seizures, decreased consciousness, autonomic dysregulation, and dyskinesias. Multifocal subcortical white matter lesions on fluid-attenuated inversion recovery and diffuse weighted images have rarely been reported in previous literature, and serial magnetic resonance imaging changes after plasma exchange have not been presented before.

Case presentation: A previously healthy 24-year-old Chinese woman presented with acute psychiatric symptoms characterized by fear and agitation followed by decreased consciousness, dyskinesias, and seizures. Magnetic resonance imaging revealed hyperintense lesions on fluid-attenuated inversion recovery and diffuse weighted images in bilateral subcortical white matter. Cerebrospinal fluid analysis revealed a mild pleocytosis with lymphocytic predominance. Protein and glucose levels were normal. Aquaporin-4 antibodies in serum and cerebrospinal fluid were negative. Identification of anti-N-methyl-D-aspartate receptor antibodies in serum and cerebrospinal fluid confirmed the diagnosis of anti-N-methyl-D-aspartate receptor encephalitis. She was initially treated with combined intravenous immunoglobulin and methylprednisolone without improvement. Plasma exchange was then initiated with good response; the patient made a full recovery after several cycles of plasma exchange. Repeat magnetic resonance imaging performed 1 month after plasma exchange showed partial resolution of the hyperintense lesions in bilateral subcortical white matter, and follow-up magnetic resonance imaging 2 months after plasma exchange showed complete resolution.

Conclusion: Anti-N-methyl-D-aspartate receptor encephalitis may be concomitant with multifocal subcortical white matter lesions. Such lesions may resolve after appropriate immunotherapy.

No MeSH data available.


Related in: MedlinePlus

Serial MRI findings: hyperintense lesions on FLAIR and DWI in bilateral subcortical white matter before plasma exchange (a, b); partial resolution of the lesions on FLAIR and DWI 1 month after plasma exchange (c, d); full resolution of the lesions on FLAIR and DWI 2 months after plasma exchange (e, f)
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Fig1: Serial MRI findings: hyperintense lesions on FLAIR and DWI in bilateral subcortical white matter before plasma exchange (a, b); partial resolution of the lesions on FLAIR and DWI 1 month after plasma exchange (c, d); full resolution of the lesions on FLAIR and DWI 2 months after plasma exchange (e, f)

Mentions: Routine blood test and chemistry analysis including liver, renal, and thyroid function were unremarkable. The erythrocyte sedimentation rate was normal. Autoimmune markers including antinuclear antibodies, anti-double-stranded DNA (anti-dsDNA) antibodies, rheumatoid factor, and antineutrophilcytoplasmic antibodies (ANCA) were negative. Serum anti-thyroglobulin (anti-TG) antibodies (38.4U/ml, normal range 0–60 U/ml, radioimmunoassay) and anti-thyroid peroxidase (anti-TPO) antibodies (28 U/ml, normal range 0–60 U/ml, radioimmunoassay) were unremarkable. Brain computed tomography (CT) was normal. MRI revealed FLAIR and DWI hyperintense lesions in bilateral subcortical white matter (Fig. 1a,b). Electroencephalography (EEG) showed diffuse slow-waves. Cerebrospinal fluid (CSF) analysis revealed a mild pleocytosis (24 × 106/L) with lymphocytic predominance. Protein and glucose levels were normal. Aquaporin-4 antibodies in serum and CSF were negative. Serological and polymerase chain reaction (PCR) investigation in serum and CSF were negative for infectious etiologies including varicella zoster virus, enterovirus, herpes simplex virus, cytomegalovirus, and Epstein-Barr virus. Bacterial cultures of the CSF were negative. Anti-NMDA receptor antibodies were identified both in serum (titers 1:100) and CSF (titers 1:16).Fig. 1


Anti-N-methyl-D-aspartate receptor encephalitis concomitant with multifocal subcortical white matter lesions on magnetic resonance imaging: a case report and review of the literature.

Wang RJ, Chen BD, Qi D - BMC Neurol (2015)

Serial MRI findings: hyperintense lesions on FLAIR and DWI in bilateral subcortical white matter before plasma exchange (a, b); partial resolution of the lesions on FLAIR and DWI 1 month after plasma exchange (c, d); full resolution of the lesions on FLAIR and DWI 2 months after plasma exchange (e, f)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4495850&req=5

Fig1: Serial MRI findings: hyperintense lesions on FLAIR and DWI in bilateral subcortical white matter before plasma exchange (a, b); partial resolution of the lesions on FLAIR and DWI 1 month after plasma exchange (c, d); full resolution of the lesions on FLAIR and DWI 2 months after plasma exchange (e, f)
Mentions: Routine blood test and chemistry analysis including liver, renal, and thyroid function were unremarkable. The erythrocyte sedimentation rate was normal. Autoimmune markers including antinuclear antibodies, anti-double-stranded DNA (anti-dsDNA) antibodies, rheumatoid factor, and antineutrophilcytoplasmic antibodies (ANCA) were negative. Serum anti-thyroglobulin (anti-TG) antibodies (38.4U/ml, normal range 0–60 U/ml, radioimmunoassay) and anti-thyroid peroxidase (anti-TPO) antibodies (28 U/ml, normal range 0–60 U/ml, radioimmunoassay) were unremarkable. Brain computed tomography (CT) was normal. MRI revealed FLAIR and DWI hyperintense lesions in bilateral subcortical white matter (Fig. 1a,b). Electroencephalography (EEG) showed diffuse slow-waves. Cerebrospinal fluid (CSF) analysis revealed a mild pleocytosis (24 × 106/L) with lymphocytic predominance. Protein and glucose levels were normal. Aquaporin-4 antibodies in serum and CSF were negative. Serological and polymerase chain reaction (PCR) investigation in serum and CSF were negative for infectious etiologies including varicella zoster virus, enterovirus, herpes simplex virus, cytomegalovirus, and Epstein-Barr virus. Bacterial cultures of the CSF were negative. Anti-NMDA receptor antibodies were identified both in serum (titers 1:100) and CSF (titers 1:16).Fig. 1

Bottom Line: Identification of anti-N-methyl-D-aspartate receptor antibodies in serum and cerebrospinal fluid confirmed the diagnosis of anti-N-methyl-D-aspartate receptor encephalitis.Anti-N-methyl-D-aspartate receptor encephalitis may be concomitant with multifocal subcortical white matter lesions.Such lesions may resolve after appropriate immunotherapy.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong-An Road, Xicheng District, Beijing, 100050, People's Republic of China. wangrj73@sina.com.

ABSTRACT

Background: Anti-N-methyl-D-aspartate receptor encephalitis is a severe autoimmune disorder characterized by severe psychiatric symptoms, seizures, decreased consciousness, autonomic dysregulation, and dyskinesias. Multifocal subcortical white matter lesions on fluid-attenuated inversion recovery and diffuse weighted images have rarely been reported in previous literature, and serial magnetic resonance imaging changes after plasma exchange have not been presented before.

Case presentation: A previously healthy 24-year-old Chinese woman presented with acute psychiatric symptoms characterized by fear and agitation followed by decreased consciousness, dyskinesias, and seizures. Magnetic resonance imaging revealed hyperintense lesions on fluid-attenuated inversion recovery and diffuse weighted images in bilateral subcortical white matter. Cerebrospinal fluid analysis revealed a mild pleocytosis with lymphocytic predominance. Protein and glucose levels were normal. Aquaporin-4 antibodies in serum and cerebrospinal fluid were negative. Identification of anti-N-methyl-D-aspartate receptor antibodies in serum and cerebrospinal fluid confirmed the diagnosis of anti-N-methyl-D-aspartate receptor encephalitis. She was initially treated with combined intravenous immunoglobulin and methylprednisolone without improvement. Plasma exchange was then initiated with good response; the patient made a full recovery after several cycles of plasma exchange. Repeat magnetic resonance imaging performed 1 month after plasma exchange showed partial resolution of the hyperintense lesions in bilateral subcortical white matter, and follow-up magnetic resonance imaging 2 months after plasma exchange showed complete resolution.

Conclusion: Anti-N-methyl-D-aspartate receptor encephalitis may be concomitant with multifocal subcortical white matter lesions. Such lesions may resolve after appropriate immunotherapy.

No MeSH data available.


Related in: MedlinePlus