Anti-NMDA Receptor Encephalitis Presenting as a Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL) (P04.251)

Abstract

Objective: To describe a patient with HaNDL and serum antibodies to the NMDA receptor. Background HaNDL is characterized by episodes of severe headache accompanied by transient neurological deficits and CSF pleocytosis in the presence of normal neuroimaging. Typically, it occurs in young males without history of migraine. Its etiology is unclear, and it has never been associated to anti-NMDA antibodies. Design/Methods: A previously healthy 18-year-old male presented with an unusual throbbing headache accompanied by right hemiparesis and sensory loss, and language difficulties that lasted for 8 hours. He was treated with acyclovir that was stopped 48 hours later, after a normal brain MRI, and a normal intracranial MR-angiography. During the following 4 days he also presented transient nocturnal episodes of confusion and agitation for several hours that required neuroleptics and that disappeared spontaneously; he was discharged from the hospital. Cerebrospinal fluid contained 130 lymphocytes per μl with normal glucose and protein levels. Extensive diagnostic work-up for tumors and infection was negative except for the presence of anti-NMDA antibodies in serum. He was then treated with a 5-day course of intravenous immunoglobulins. At present, 1 year after admission, he remains asymptomatic. Results: The differential diagnosis in this patient included viral encephalitis and HaNDL. The presence of antibodies to the NMDA receptor suggested that he had anti-NMDA receptor encephalitis of atypical presentation. This entity, first described in young females with ovarian teratoma, mainly causes psychiatric symptoms and memory problems, but its presentation as headache with focal neurological deficits fulfilling HaNDL criteria has not been described. Conclusions: Detecting anti-NMDA antibodies in patients with HaNDL may be important, since they can be relevant to the pathogenesis of this syndrome and their presence requires immune therapy to prevent relapses and accumulated disability. Disclosure: Dr. Garcia Monco has nothing to disclose. Dr. Gomez Beldarrain has nothing to disclose. Dr. Garcia Gorostiaga has nothing to disclose. Dr. Kortazar has nothing to disclose.