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Bruce C. Cree, MD, PhD; Gary L. Bernardini, MD, PhD; Arthur P. Hays, MD; Gina Lowe, MD

Arch Neurol. 2003;60:107-112.

Background  Coxsackieviruses and echoviruses are common causes of aseptic meningitis, but they rarely cause life-threatening illness. We report a fatal case of coxsackievirus B4 meningoencephalitis in a woman who developed extrapyramidal symptoms suggestive of encephalitis lethargica. The exact causative agent of encephalitis lethargica has rarely been found, but most cases of the syndrome are assumed to be of viral origin.

Case Description  A 33-year-old woman previously treated with methylprednisolone and cyclophosphamide for Henoch-Schönlein purpura was transferred from a referring hospital because of sore throat, fever, and chills. Her neurologic findings progressed from headache with mild photophobia to lethargy, cogwheeling, increased tone in all 4 limbs, and brisk reflexes. The patient was diagnosed as having coxsackievirus B4 meningoencephalitis and, despite treatment with the experimental antiviral agent pleconaril, died of an overwhelming central nervous system infection and myocarditis. Magnetic resonance imaging showed focal hyperintense lesions in the substantia nigra that corresponded to the location of pathological changes seen at autopsy.

Conclusions  This patient had a fulminant coxsackievirus B4 viral meningoencephalitis with a clinical pattern reminiscent of encephalitis lethargica and striking focal abnormalities in the substantia nigra identified on magnetic resonance imaging. The magnetic resonance imaging findings correlated with pathological changes identified at autopsy that were similar to the pathological findings observed in patients with encephalitis lethargica and postencephalitic parkinsonism. It is likely that the patient's immunocompromised state led to an overwhelming infection from an otherwise relatively innocuous viral infection.

From the Neurological Institute of New York, New York–Presbyterian Hospital, Columbia-Presbyterian Medical Center, New York, NY (Drs Cree, Hays, and Lowe), and Division of Stroke and Neurocritical Care, Department of Neurology, Albany Medical Center, Albany, NY (Dr Bernardini).

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