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Sophia R. Sharfstein, MD; Mark Forrest Gordon, MD; Richard B. Libman, MD; Elfrida S. Malkin, MD, MPH

Arch Neurol. 1999;56:241-243.

Objective  To report an unusual presentation of mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) manifested in late life with a clinical picture of herpes simplex encephalitis.

Design  Case report.

Setting  Clinical neurology department in a tertiary care hospital.

Case Description  A 55-year-old woman developed aphasia and delirium during ophthalmic herpes zoster infection treated with oral prednisone and ophthalmic steroids, which was followed by progressive cognitive decline without acute neurologic events for 5 years. At age 60, the patient presented with new onset of seizures, hemiparesis, and hemianopsia. Subsequently she developed cortical blindness, multiple tramautic soft tissue injuries from falls, acute psychosis, and severe dementia with periods of agitation. She died in a nursing home in March 1997, 6 years after initial presentation.

Results  Magnetic resonance imaging scan of the brain showed hyperintensity on T₂-weighted images involving temporal, parietal, and occipital lobes bilaterally as well as mild atrophy of brainstem and cerebellum. Single photon emission computed tomographic imaging showed hypoperfusion of temporal, parietal, and occipital lobes. Results of video electroencephalographic monitoring showed periodic lateralizing epileptiform discharges in temporal and occipital areas. The serum lactate level was normal in May 1996 and elevated in October 1996. The creatine kinase level was elevated with a 100% MM fraction in August 1991 and normal in March 1996. Results of repeated cerebrospinal fluid analyses indicated elevated protein levels. Analysis of DNA was diagnostic of MELAS by mitochondrial DNA point mutation at position 3243. The results of autopsy showed moderate cerebral, cerebellar, and brainstem atrophy with signs of infarction in temporal and parietal lobes bilaterally.

Conclusions  The clinical presentation as well as age at onset of MELAS are highly variable. Onset of mitochondrial disorders can be provoked by febrile illness when there is mismatch between energy requirements and availability. In the differential diagnosis of herpes encephalitides, MELAS syndrome should be considered.

From the Departments of Neurology, Long Island Jewish Medical Center, Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park (Drs Sharfstein, Gordon, and Libman) and Columbia Presbyterian Medical Center, New York (Dr Malkin), NY.

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