A Case of Acute Hemorrhagic Leukoencephalitis in Childhood

Mei Momomura^1）, Toshiyuki Hikita^1）, Natsue Nakamoto^1,3）, Yasushi Fujii^1）
Satoshi Koyama^1）, Suguru Wakita^1）, Hiroshi Oba^2）, Yukishige Yanagawa^1）
Department of Pediatrics^1）, Radiology^2）, Teikyo University School of Medicine
Department of Occupational Therapy, Mejiro University Faculty of Health Sciences^3）

Abstract
A case of acute hemorrhagic leukoencephalitis（AHLE）in a 5-year-old boy is reported.
　　Acute disseminated encephalomyelitis comprises a group of demyelinating disorders of the CNS, of which a hemorrhagic variant has the most severe clinical course（AHLE）. The early brain CT scan showed multiple high density spots surrounded by areas of low density. MRT2WI showed several foci of high intensity lesions, mainly involving the white matter, with massive hemorrhagic edematous swellings. Cerebrospinal fluid analysis showed 16 white blood cells/_l, 15.9 mg/dl of protein, and 98 mg/dl of glucose. He was diagnosed as AHLE, and methylprednisolone（mPLS）, 30 mg/kg, was given. An MR image obtained 3 weeks after hospitalization showed increasing cerebral lesions. Intravenous immunoglobulin（IVIG）was given at a dose of 400 mg/kg/day for 10 days. MR images obtained 4 months after admission showed a decrease of the mass effect. The patient’s clinical and neurologic features have continued to improve without relapse（follow-up period of 8 months）. This case suggests that IVIG is effective in AHLE and may give better results than steroids alone. MRI is valuable in detecting the pathophysiological changes of AHLE from the acute to the chronic phases.

Keywords： Acute hemorrhagic leukoencephalitis (AHLE), Hurst disease, Intravenous immunoglobulins (IVIG), Methylprednisolone (mPLS), Magnetic resonance imaging (MRI)