Case Presentation:
An 18 year‐old African American woman with a distant history of seizures presented with two weeks of progressive bizarre behavior and memory loss, followed by refractory status epilepticus requiring mechanical ventilator support. She had been evaluated as an out‐patient and was diagnosed with an acute psychiatric illness. Neuroimaging was unremarkable and cerebrospinal fluid [CSF] analysis showed lymphocytic pleocytosis, consistent with viral or autoimmune encephalitis. She was treated empirically with antiviral therapy and antieplipetic drugs without response to therapy. Non‐infectious causes on encephalitis were considered. On abdominal imaging an ovarian mass was seen, which upon surgical resection was found to be an immature ovarian teratoma. Aggressive immunomodulatory therapy was initiated with high dose steroids and intravenous immunoglobulin. The diagnosis of anti NMDAR encephalitis was eventually confirmed by the identification of anti‐NMDAR antibodies in a second CSF sample. She was transferred to a rehabilitation facility with ventilator capabilities on immunomodulatory therapy. At her last outpatient follow up appointment she had no residual cognitive or neurologic deficits.
Discussion:
Anti‐N‐methyl‐D‐aspartate receptor (anti‐NMDAR) encephalitis is a newly described form of encephalitis associated with prominent psychiatric symptoms at onset. usually present with psychiatric manifestation, memory loss and seizure. autonomic instability and central hypoventalation can be part of the sever features of this syndrome with fatal outcome if not diagnosed early.
Its highly associated with tumor as mature teratoma in our case.patients with prompt tumor removal and immunotherapy has better outcome and fewer neurological deficit.
the management of NMDAR encephalitis can prove to be very effective and involves the identification and treatment of the tumor, along with immunotherapy.
Conclusions:
The causes of encephalitis are numerous, and most patients undergo extensive testing to identify infectious etiologies. However, non‐infectious etiologies need to be considered Anti‐NMDAR encephalitis is a potentially devastating but reversible illness that can have good outcomes, particularly with aggressive and prompt therapy. Clinicians must have a high index of suspicion, when evaluating young patients with apparently idiopathic encephalitis or encephalopathy.