Anti-N-methyl-D-aspartate-receptor encephalitis: diagnosis, optimal management, and challenges

Abstract

ObjectiveAnti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is a new autoimmune disorder, often paraneoplastic in nature, presenting with complex neuropsychiatric symptoms. Diagnosed serologically, this disorder is often responsive to immunosuppressant treatment. The objective of this review is to educate clinicians on the challenges of diagnosis and management of this disorder.Materials and methodsA review of the relevant literature on clinical presentation, pathophysiology, and recommended management was conducted using a PubMed search. Examination of the results identified articles published between 2007 and 2014.ResultsThe literature highlights the importance of recognizing early common signs and symptoms, which include hallucinations, seizures, altered mental status, and movement disorders, often in the absence of fever. Although the presence of blood and/or cerebrospinal fluid autoantibodies confirms diagnosis, approximately 15% of patients have only positive cerebrospinal fluid titers. Antibody detection should prompt a search for an underlying teratoma or other underlying neoplasm and the initiation of first-line immunosuppressant therapy: intravenous methylprednisolone, intravenous immunoglobulin, or plasmapheresis, or a combination thereof. Second-line treatment with rituximab or cyclophosphamide should be implemented if no improvement is noted after 10 days. Complications can include behavioral problems (eg, aggression and insomnia), hypoventilation, catatonia, and autonomic instability. Those patients who can be managed outside an intensive care unit and whose tumors are identified and removed typically have better rates of remission and functional outcomes.ConclusionThere is an increasing need for clinicians of different specialties, including psychiatrists, neurologists, oncologists, neurooncologists, immunologists, and intensivists to become familiar with this disorder and its potential complications. Remission can be optimized with prompt detection and aggressive, collaborative treatment within a multidisciplinary team.

A case report of anti-N-methyl-d-aspartate receptor autoimmune encephalitis with sensory attack. Is limbic encephalitis only “limbic”?

“…[2] This kind of encephalitis is usually subacute at onset with significant psychiatric symptoms, including agitation, mania, hallucination, aggression as well as cognitive dysfunction.…”

Acute psychosis as a harbinger of a tumor: A case of anti-N-methyl-D-aspartate receptor encephalitis

“…In a patient with suspected encephalitis, workup should include lumbar puncture, with viral and autoimmune CSF studies, electroencephalogram (EEG), and MRI of the brain. [3] CSF studies will typically show lymphocytosis with few oligoclonal bands, no red blood cells, and normal glucose and protein levels. MRI abnormalities are present in 23%-50% of cases and typically show non-specific enhancement in the cortex or subcortical structures, although diffusion tensor imaging (DTI) may reveal widespread white matter damage.…”

Acute psychosis as a harbinger of a tumor: A case of anti-N-methyl-D-aspartate receptor encephalitis

“…[4] EEG shows non-specific generalized slowing in up to 90% of cases. [3] Brain biopsy has been done in some patients, but typically shows normal tissue or nonspecific changes, therefore is generally not indicated. Serum anti-NMDA receptor antibodies are present in only 15% of cases.…”

“…Serum anti-NMDA receptor antibodies are present in only 15% of cases. [3] Definitive diagnosis is made by the detection of anti-NMDA receptor antibodies in the CSF, which should prompt pelvic ultrasound (US) and MRI to evaluate for presence of teratoma, which is found in up to 50% of females older than 18 with this disorder. [5] Clinical course can be positively altered with immunosuppressants and removal of teratomas, which are frequently associated with the disorder, however physicians must have a high index of suspicion so that they order appropriate serol-ogy to make the diagnosis.…”