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Acute Necrotizing Encephalopathy of Childhood in Non-Asian Patients: Report of Three Cases and Literature Review

Department of Neurology, University of Athens, "P & A Kyriakou" Children's Hospital, Athens, Greece, smastr{at}otenet.gr.

Dimitrios Gionnis, MD

Pediatric Intensive Care, University of Athens, "P & A Kyriakou" Children's Hospital, Athens, Greece,

Konstantinos Voudris, MD

Department of Neurology, University of Athens, "P & A Kyriakou" Children's Hospital, Athens, Greece

Angeliki Skardoutsou, MD

Second Department of Pediatrics, University of Athens, "P & A Kyriakou'' Children's Hospital, Athens, Greece

Masashi Mizuguchi, MD, PhD

Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

Acute necrotizing encephalopathy of childhood is a novel type of parainfectious encephalopathy with a racial and geographic predilection, rarely reported from other than East Asian areas. The objective was to describe the clinical, imaging, and other laboratory findings of non-Asian patients with acute necrotizing encephalopathy. Data were collected from three patients diagnosed in Athens over a 4-year period plus 16 cases reported from other European and North American countries. One of the Greek children died, and the other two had a normal outcome. A neuropathologic examination in the fatal case showed edematous necrosis without inflammatory, reactive, or proliferative changes. Data from Greek and other non-Asian patients support the homogeneity of the disease worldwide. (J Child Neurol 2006;21:872—879; DOI 10.2310/7010.2006.00210).

Journal of Child Neurology, Vol. 21, No. 10, 872-879 (2006)
DOI: 10.1177/08830738060210101401


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