Журнал клинических случаев

Communicating Hydrocephalus Associated with Relapsing Anti-N-Methyl-DAspartate Receptor (NMDAR) Encephalitis in an Adolescent Girl

Abstract

Jayaprakash A and Osama Al-Shuker

Anti–N-methyl-D-aspartate receptor (NMDAR) encephalitis is uncommon cause of immune mediated encephalopathy in children. It usually manifests as psychosis, seizures and dyskinesia. We describe an unusual association with the phenotype of this disease. An 11 year-old girl presented with psycho-behavioral disturbances, headache, fever and episodic disorientation few weeks after fever and upper respiratory tract infection. Her MRI showed communicating hydrocephalus. She was found to have positive anti-NMDAR antibodies. She underwent VP shunt followed by immunomodulatory therapy resulting in resolution of headache and substantial improvement of alertness and psycho-behavior. Several months later she relapsed and remission was induced by a repeated course of immunomodulatory therapy. This report illustrates the remarkable phenotypic variability in this condition. The diagnosis of anti-NMDAR encephalitis should be considered in patients who present with subacute or relapsing encephalopathy associated with communicating hydrocephalus.