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 Volume 66, Number 9, May 9, 2006
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NEUROLOGY 2006;66:1304-1309
© 2006 American Academy of Neurology

Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy

J. Takanashi, MD, H. Oba, MD, A. J. Barkovich, MD, H. Tada, MD, Y. Tanabe, MD, H. Yamanouchi, MD, S. Fujimoto, MD, M. Kato, MD, M. Kawatani, MD, A. Sudo, MD, H. Ozawa, MD, T. Okanishi, MD, M. Ishitobi, MD, Y. Maegaki, MD and Y. Koyasu, MD

From the Department of Pediatrics (J.T.), Kameda Medical Center, Kamogawa, Department of Radiology (H.O.), Teikyo University School of Medicine, Tokyo, Department of Pediatrics (H.T.), Chiba University Graduate School of Medicine, Department of Pediatrics (Y.T.), Chiba Children's Hospital, Department of Pediatrics (H.Y.), Dokkyo University School of Medicine, Tochigi, Department of Pediatrics (S.F.), Graduate School of Medical Sciences, Nagoya City University, Department of Pediatrics (M.K.), Yamagata University School of Medicine, Department of Pediatrics (M.K.), Faculty of Medical Sciences, University of Fukui, Department of Pediatrics (A.S.), Sapporo Municipal Hospital, Department of Pediatrics (H.O.), Tokyo Metropolitan Hachioji Children's Hospital, Hachioji, Department of Pediatrics (T.O.), Seirei Mikatahara General Hospital, Hamamatsu, Department of Pediatrics (M.I.), Tohoku University School of Medicine, Sendai, Department of Child Neurology (Y.M.), Faculty of Medicine, Tottori University, and Department of Pediatrics (Y.K.), Showa General Hospital, Kodaira, Japan; and Neuroradiology Section (A.J.B.), Department of Radiology, University of California San Francisco.

Address correspondence and reprint requests to Dr. J.-I. Takanashi, Department of Pediatrics, Kameda Medical Center, 929 Higashi-cho, Kamogawa-shi, Chiba 296-8602, Japan; e-mail: jtaka{at}kameda.jp

Background: Patients with encephalopathy heralded by a prolonged seizure as the initial symptom often have abnormal subcortical white matter on diffusion-weighted MRI (DWI).

Objective: To determine if these patients share other common features.

Methods: Patients with encephalopathy heralded by a prolonged seizure and followed by the identification of abnormal subcortical white matter on MRI were collected retrospectively. Their clinical, laboratory, and radiologic data were reviewed.

Results: Seventeen patients were identified, ages 10 months to 4 years. All had a prolonged febrile seizure (longer than 1 hour in 12 patients) as their initial symptom. Subsequent seizures, most often in clusters of complex partial seizures, were seen 4 to 6 days after the initial seizure in 16 patients. Outcome ranged from almost normal to severe mental retardation. MRI performed within 2 days of presentation showed no abnormality. Subcortical white matter lesions were observed on DWI between 3 and 9 days in all 17 patients. T2-weighted images showed linear high intensity of subcortical U fibers in 13 patients. The lesions were predominantly frontal or frontoparietal in location with sparing of the perirolandic region. The diffusion abnormality disappeared between days 9 and 25, and cerebral atrophy was detected later than 2 weeks. Three patients having only frontal lesions had relatively good clinical outcome.

Conclusions: Although the pathophysiologic mechanism remains unknown, these patients seem to have a distinctive encephalopathy syndrome. MRI is helpful in establishing the diagnosis of this encephalopathy.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 9 issue to find the title link for this article.

Commentary, see page 1291

Supported in part by the Research Grant (17A-11) for Nervous and Mental Disorders from the Ministry of Health, Labor, and Welfare of Japan.

Disclosure: The authors report no conflicts of interest.

Received August 29, 2005. Accepted in final form January 19, 2006.


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