The Worm in the Apple

1Brigham and Women's Hospital, Boston, MA

Meeting: Hospital Medicine 2013, May 16-19, National Harbor, Md.

Abstract number: 317

Case Presentation:

A 30‐year‐old woman was admitted with fever and headache. On physical examination temperature was 101.5°F; the jolt accentuation test was positive. Routine laboratory studies and head CT were normal. CSF analysis showed 220 WBC (98% lymphocytes), normal protein and glucose. Laboratory test results are summarized in the table. The patient was treated with analgesics for presumed viral meningitis. Her symptoms improved, and she was discharged after 10 days with mild residual headache and a low‐grade fever of 100°F. One day postdischarge her family noted belligerent behavior, followed over 48 hours by leg weakness, disorientation, and intermittent somnolence. She was readmitted; examination showed nuchal rigidity, inattentiveness, nystagmus, dysarthria, leg weakness, and hyperesthesia. CSF analysis showed 165 WBC (95% lymphocytes), 18 RBC, and protein of 244 mg/dL (10‐44 mg/dL). MRI of the neuraxis showed generalized leptomeningeal enhancement consistent with meningoencephalitis; a possible liver hemangioma was noted. CSF testing showed no antibodies to West Nile virus, Borrelia burgdorferi, or eastern equine encephalitis. High‐dose methylprednisone was begun and antimicrobial therapy resumed. Over the next 72 hours the patient's clinical status deteriorated with progressive weakness, obtundation, and dysautonomia (labile blood pressure and incontinence). Abdominal CT confirmed the hemangioma but also showed an ovarian teratoma. Following emergent oophorectomy and intravenous immunoglobulin, her cognitive function improved markedly within 3–4 days; motor function normalized, and dysautonomia resolved over the next 2 months.

Discussion:

Ovarian teratomas are associated with autoimmune anti‐NMDAR (N‐methyl‐d‐aspartate) receptor encephalitis. Although antibody testing was negative, the patient's behavioral changes, language dysfunction, and autonomic instability are strongly associated with anti‐NMDAR illness. Onconeural antibodies are not required for the diagnosis of paraneoplastic encephalitis when classic clinical features are present, and the prompt response to oophorectomy further supports the diagnosis. The California Encephalitis Project reported in 2012 that anti‐NMDAR was the most frequently proven cause of encephalitis in a referral cohort of patients under age 30 seen between 2007 and 2011. In addition to 32 proven cases, anti‐NMDAR disease was clinically suspected in 15 other cases in the cohort with negative serology.

Conclusions:

This case is presented to highlight the emerging prevalence of a treatable cause of encephalitis with a unique clinical profile. It also demonstrates the element of uncertainty that may accompany clinical diagnoses such as viral meningitis and the importance of revisiting diagnostic assumptions in the face of an atypical clinical course.

Laboratory Results



Test Result
CSF bacterial culture No growth
CSF VDRL Nonreactive
CSF HSV DNA (PCR) Not detected
CSF enteroviral RNA Not detected
CSF VZV DNA Not detected
Serum HIV 1/2 antibody (EIA) Negative
Serum quantitative HIV RNA (PCR) Not detected
Nasopharyngeal swab for influenza (EIA) Negative
CSF cryptococcal antigen Negative
Serum cryptococcal antigen Negative
EBV antiviral capsid antigen IgG 1:1280
EBV antiviral capsid antigen IgM Negative
Serum anti-NMDAR antibody Not detected

To cite this abstract:

Blair R. The Worm in the Apple. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 317. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/the-worm-in-the-apple/. Accessed July 22, 2019.

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