First Bahraini adolescent with anti-NMDAR-Ab encephalitis

Abstract

Anti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is a new autoimmune, often paraneoplastic disorder that presents with complex neuropsychiatric symptoms. It was first described in 2007 by Dalmau et al. Our patient presented with headache, behavioral changes and then seizures with hallucinations. She was initially misdiagnosed to have schizophrenia and was prescribed antipsychotics. She deteriorated and developed further seizures with hypoventilation and choreoathetosis. Her blood investigations were positive for mycoplasma IGM. Her CSF studies showed high white cell counts, predominantly lymphocytes, and high anti-NMDA-R titre. Her brain MRI scans showed high tbl2 and FLAIR intensities in the grey and white matter of the left cerebellar hemisphere suggestive of acute disseminated encephalomyelitis. She responded to treatment with antibiotics, multiple antiepileptics, steroids and needed five sessions of plasmapheresis. There was no underlying malignancy on repeated scanning of the abdomen. She needed around one year for full recovery with intensive rehabilitation. The objective of this paper was to highlight the occurrence of this fairly new, challenging, easily missed, not-so-rare form of encephalitis often occurring in the absence of fever.

MR Imaging of Acute Cerebellar Involvement in Pediatric Anti-N-Methyl-D-Aspartate Receptor Encephalitis

“…1 Cerebellitis is commonly caused by infectious agents such as Epstein-Barr virus, varicella, rotavirus, mycoplasma, mumps, and human herpesvirus-6. 1 Cerebellar involvement is uncommon in anti-NMDA receptor encephalitis 2,3 and may be secondary to excitotoxicity to cerebellar granule cells. 4 Anti-NMDA receptor encephalitis should be considered as a differential diagnosis in children with neuroimaging findings suggestive of acute cerebellitis, particularly when the presentation includes extrapyramidal movement disorders.…”