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Post-transplant acute limbic encephalitis

Clinical features and relationship to HHV6

From the Department of Neurology (W.W.S., T.M.H., K.U., E.B.B.), the Division of Infectious Diseases (F.M.M., L.R.B.), and the Division of Hematology/Oncology (R.J.S., J.H.A.), Brigham &Women's Hospital; Harvard Medical School (W.W.S., F.M.M., T.M.H., K.U., R.J.S., J.H.A., L.R.B., E.B.B.); and Dana-Farber Cancer Institute (F.M.M., R.J.S., J.H.A., L.R.B.), Boston, MAS. is currently with the Department of Neurology, University of California, San Francisco. K.U. is currently with the Department of Neurosurgery, University of California, Los Angeles.

Address correspondence and reprint requests to Dr. William W. Seeley, UCSF Memory and Aging Center, Box 1207, San Francisco, CA 94143-1207 wseeley{at}memory.ucsf.edu

Background: Acute limbic encephalitis has been reported in the setting of treatment-related immunosuppression and attributed to human herpesvirus-6 (HHV6) infection. Clinical and laboratory features of the syndrome, however, have not been well characterized.

Methods: We describe the clinical, EEG, MRI, and laboratory features of nine patients with acute limbic encephalitis after allogeneic hematopoietic stem cell transplantation (HSCT). To explore the relationship between HHV6 and this syndrome, we reviewed available CSF HHV6 PCR results from all HSCT patients seen at our center from March 17, 2003, through March 31, 2005.

Results: Patients displayed a consistent and distinctive clinical syndrome featuring anterograde amnesia, the syndrome of inappropriate antidiuretic hormone secretion, mild CSF pleocytosis, and temporal EEG abnormalities, often reflecting clinical or subclinical seizures. MRI showed hyperintensities within the uncus, amygdala, entorhinal area, and hippocampus on T2, fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI) sequences. CSF PCR assays for HHV6 were positive in six of nine patients on initial lumbar puncture. All patients were treated with foscarnet or ganciclovir. Cognitive recovery varied among long-term survivors. The one brain autopsy showed limbic gliosis and profound neuronal loss in amygdala and hippocampus. Among 27 HSCT patients with CSF tested for HHV6 over a 2-year period, positive results occurred only in patients with clinical limbic encephalitis.

Conclusions: Patients undergoing allogeneic hematopoietic stem cell transplantation are at risk for post-transplant acute limbic encephalitis (PALE), a distinct neurologic syndrome. Treatment considerations should include aggressive seizure control and, possibly, antiviral therapy. PALE can be associated with the CSF presence of human herpesvirus-6, but the pathogenic role of the virus requires further exploration.

Supplemental data at www.neurology.org

*W.W.S. and F.M.M. contributed equally to this work.

Disclosure: The authors report no conflicts of interest.

Received May 1, 2006. Accepted in final form February 15, 2007.

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