A rare case of anti-NMDA receptor encephalitis with CVST

Abstract

Anti N-methyl-D-aspartate (NMDA) receptor encephalitis is new entity discovered over a decade. It is a type of autoimmune encephalitis characterized by a sequential onset of neuropsychiatric symptoms especially in the young population. Early diagnosis and treatment lead to better outcome and may prevent permanent neurological deficit or even death. Most patients respond well to first-line treatment and the resection of tumors associated with the disease. Here we present a case, which based on radiological findings, was diagnosed as cerebral venous sinus thrombosis (CVST), and was started on anticoagulation. Failure to respond to treatment and development of new movement abnormalities warranted for further investigations and he was found to have anti-NMDA receptor antibody positive in cerebrospinal fluid. Due to overlapping symptoms of CVST and encephalitis, high incidence of cerebral venous sinus thrombosis in this part of the world, and radiological findings, was initially thought to have CVST which was also confirmed on brain imaging. Lack of consent by the parents for a lumbar puncture delayed the definitive diagnosis. Patient developed movement disorder suggestive of autoimmune encephalitis, and this time after extensive counselling, when the cerebrospinal fluid was send for work up, it showed a positive anti-NMDA receptor antibody. He was promptly started on plasmapheresis after which patient had dramatic improvement both in movement abnormalities and neuropsychiatric symptoms.

Keywords

• Anti N-methyl-D-aspartate (NMDA) receptor encephalitis
• Caverous Venous Sinus Thrombosis