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The status of cerebral autoregulation (CA) is an important prognostic factor for acute head trauma, but the role of CA in patients with acute encephalitis has not been previously discussed. We present the case of a 30-year-old woman with severe acute encephalitis who underwent craniectomy for intractable increased intracranial pressure (ICP). Preoperatively, adjustments of blood pressure (BP) with simultaneous recording of changes in cerebral blood flow velocity with transcranial Doppler indicated increased ICP and impaired CA. Postoperatively, ICP declined remarkably but CA remained impaired when the relationship between spontaneous fluctuation of mean BP and ICP was analyzed. Increased ICP recurred again within 24 hours of the decompression surgery and caused death of the patient. We propose that evaluating the status of CA could be of prognostic importance in patients with severe encephalitis. [J Formos Med Assoc 2007;106(2 Suppl):S7-S12]