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Neurologic Onset of Behçet's Disease: A Diagnostic Enigma in Childhood

Department of Pediatrics Hôpital Nord, Marseilles

Brigitte Chabrol, MD

Department of Pediatric Neurology, Hopital de la Timone

Jean-Marc Riss, MD

Department of Ophthalmology Hôpital de la Timone

Josette Mancini, MD

Department of Pediatric Neurology, Hopital de la Timone

Charles Raybaud, MD

Department of Neuroradiology Hôpital Nord, Marseilles, France

Jean-Marc Garnier, MD

Department of Pediatrics Hôpital Nord, Marseilles

Neuro-Behçet's disease, which is uncommonly reported in childhood, encompasses a wide variety of clinical features since any part of the neuraxis may be involved. It carries a serious prognosis and represents a leading cause of death or severe disability. Neuro-Behçet's disease may occur in 5%.to 50% of adults with Behçet's disease and is usually subsequent to other systemic manifestations. In this report, we express the possibility of a primary neurologic presentation of Behçet's disease in childhood with pseudotumor cerebri and meningoencephalitis as exclusive initial features. We focus on diagnostic problems when major features of Behçet's disease are missing at the outset. We emphasize the high sensitivity of magnetic resonance imaging to make the diagnosis of cerebral vasculitis or thrombosis with a good reliability to clinical features. (J Child Neurol 1997;12:237—241).

Journal of Child Neurology, Vol. 12, No. 4, 237-241 (1997)
DOI: 10.1177/088307389701200402


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