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Review
. 2023 Apr 15;13(4):662.
doi: 10.3390/brainsci13040662.

The Leading Role of Brain and Abdominal Radiological Features in the Work-Up of Anti-NMDAR Encephalitis in Children: An Up-To-Date Review

Miriana Guarino  1 , Saverio La Bella  1 , Marco Santoro  2 , Daniele Caposiena  2 , Enza Di Lembo  3 , Francesco Chiarelli  1 , Giovanni Iannetti  3
Affiliations
Review

The Leading Role of Brain and Abdominal Radiological Features in the Work-Up of Anti-NMDAR Encephalitis in Children: An Up-To-Date Review

Miriana Guarino et al. Brain Sci. .

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis (NMDARe) is the most common cause of nonviral encephalitis, mostly affecting young women and adolescents with a strong female predominance (F/M ratio of around 4:1). NMDARe is characterized by the presence of cerebrospinal fluid (CSF) antibodies against NMDARs, even though its pathophysiological mechanisms have not totally been clarified. The clinical phenotype of NMDARe is composed of both severe neurological and neuropsychiatric symptoms, including generalized seizures with desaturations, behavioral abnormalities, and movement disorders. NMDARe is often a paraneoplastic illness, mainly due to the common presence of concomitant ovarian teratomas in young women. Abdominal ultrasonography (US) is a key imaging technique that should always be performed in suspected patients. The timely use of abdominal US and the peculiar radiological features observed in NMDARe may allow for a quick diagnosis and a good prognosis, with rapid improvement after the resection of the tumor and the correct drug therapy.

Keywords: NMDARe; abdominal US; autoimmune encephalitis; encephalitis; ultrasound.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
A diagnostic approach for autoimmune encephalitis. * Blood tests should include infectious diseases (erythrocyte sedimentation rate, C-reactive protein, complete blood cell count, and common viruses involved in pediatric encephalitis), neuroinflammatory studies (autoantibodies involved in common pediatric encephalitis, anti-myelin oligodendrocyte glycoprotein antibodies, anti-aquaporin-4 antibodies, and oligoclonal bands), neurorheumatologic studies (angiotensin converting enzyme, anti-nuclear antibody testing, anti-neutrophil cytoplasmic antibody testing, and double-stranded DNA testing), metabolic and mitochondrial testing (lactate/pyruvate ratio, comprehensive metabolic panel, plasma amino acids, ammonia level, copper, ceruloplasmin, vitamin B12, and vitamin B1), and thyroid studies (thyroid stimulating hormone, thyroxine, anti-thyroglobulin antibodies, and anti-thyroid peroxidase antibodies). AE: Autoimmune encephalitis, MRI: Magnetic Resonance Imaging, EEG: electroencephalography, and CSF: cerebrospinal fluid.
Figure 2
Figure 2
US transverse scan in a female adolescent with NMDARe showing a poor vascularized right ovarian teratoma of about 40 × 22 mm with a well-defined contour and irregular hyperechoic areas inside. D1 is the longitudinal diameter of the teratoma. In the right figure blue and red dots represent the poor vascularization of the ovarian teratoma.
Figure 3
Figure 3
Enhanced CT scan with contrast; portal venous phase: right ovarian teratoma of 43 × 33 mm (indicated by colored lines in the image) with a well-defined contour in a female adolescent with NMDARe. Predominately cystic attenuation with scattered regions of hyperattenuating and fatty tissue attenuation.
See this image and copyright information in PMC

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