SESSION TITLE: Miscellaneous Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: Anti-N-methyl-D-aspartate receptor encephalitis is an immune-mediated paraneoplastic syndrome that affects predominantly young females with mature ovarian teratomas and presents with constellation of neuropsychiatric and autonomic dysfunction. We present an unusal case of anti-NMDA encephalitis without ovarian teratoma. Anti-NMDA encephalitis should be suspected in patients with acute seizures and abrupt onset of dyskinesia and behavioral changes. The diagnosis is made by detection of NMDR antibodies in the CSF. Treatment includes resection of ovarian teratoma, IV steroids, plasma exchange and IVIG. Because of late diagnosis, and treatment, many times NMDA encephalitis leads to death from neurologic and autonomic dysfunction. Our case reinforces that early diagnosis and treatment can lead to good outcomes.
CASE PRESENTATION: A previously healthy 38-year-old female presented with cervical lymphadenopathy, low-grade fever and headache for 14 days. On exam, hypotensive, tachycardic, ill appearing, and lethargic without focal neurological deficits. Subsequently, she developed confusion, and intractable generalized grand-mal seizures with status epilepticus and respiratory failure requiring mechanical ventilation. CBC, electrolytes and renal function were normal. Lumbar puncture had a normal opening pressure, CSF revealed a lymphocytic pleocytosis with negative bacterial, fungal cultures and viral panel including HSV, EBV, CMV, West Nile, HIV. A serum paraneoplastic panel revealed a positive NMDAR Antibodies. A repeat lumbar puncture with CSF analysis confirmed NMDAR antibodies. A lymph node biopsy showed non-specific inflammation. CT chest/abdomen, and PET scan shown no evidence of neoplasm. After IV steroids, IVIG and 6 treatments of plasmaphereses, noted a rapid recovery of neurologic function. At the time of discharge, she could stand, smile appropriately, and interact with eye movements and hand gestures. She was discharged to a Rehabilitation Facility due to chronic respiratory failure with tracheostomy and physical therapy. She was decannulated in a week’s time and discharged to home without any additional needs. At last follow up, the patient had only mild retrograde amnesia.
DISCUSSION: Despite a growing body of evidence, Anti-N-methyl-D-aspartate receptor encephalitis remains under-recognized. Prompt diagnosis and early immunotherapy can lead to full recovery of neuropsychiatric function as we report in our patient.
CONCLUSIONS: Our case emphasizes the need to have a high index of suspicion and increased awareness of this syndrome when dealing with viral encephalitis to improve outcomes, decrease morbidity and mortality.
Reference #1: Rosenfeld MR, Dalmau JO. Paraneoplastic disorders of the CNS and autoimmune synaptic encephalitis.Continuum (Minneap Minn). 2012 Apr;18(2):366-83
Reference #2: Day GS, High SM, Cot B, Tang-Wai DF.Anti-NMDA-receptor encephalitis: case report and literature review of an under-recognized condition.J Gen Intern Med. 2011 Jul;26(7):811-6.
DISCLOSURE: The following authors have nothing to disclose: Olena Lineberry, Amol Patil, Kristina Bailley
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