Acute disseminated encephalomyelitis and mucocutaneous ulcerations

Case report. We report the case of a 15-year-old Libyan girl who developed headache, gait disturbances, and vomiting in Libya. Due to multiple high-density lesions in the T2-weighted MRI pictures of the brain with an exceptional isolated lesion of the corpus callosum, diagnosis of acute disseminated encephalomyelitis was made. Because recurrent cycles of prednisolone treatment failed, the girl was transferred to our hospital. In addition to neurologic complaints such as gait disturbances, ataxia, and hypesthesia of both legs, she then developed uveitis as well as severe ulcerations of the mouth and genital region. Diagnosis of neuro-Behçet's disease was made. Under treatment with prednisolone and azathioprine, the girl's condition improved rapidly so that we were able to discharge her home after 4 weeks. Discussion. The presented disease course of neuro-Behçet's disease is quite exceptional in children, because neurologic symptoms rarely precede the typical clinical findings of Behçet's disease in children. Furthermore, an isolated, high-density MRI lesion of the corpus callosum, like in the present case, has not been described in neuro-Behçet's disease before. Therefore, it must be recognized that neurologic symptoms may precede Behçet's disease by months even in children and that this disorder must be incorporated into the differential diagnosis of miscellaneous neurologic disorders. © Springer Medizin Verlag 2004.