[ XXXX in Beh  et disease: case report and review of the literature].

@entity1 @entity32 @entity1333 @entity1524 @entity229 @entity16 @entity14663 @entity166 @entity1829 @entity10 @entity3917 @entity50 @entity11635
BACKGROUND: Beh  et's disease is a chronic @entity32 , clinically characterised by multisystemic @entity1333 . Primary @entity16 in childhood is quite exceptional. We report a case of a @entity1 with recurrent @entity50 . Beh  et's disease was diagnosed. HISTORY AND SIGNS: A 13-year-old @entity1 from Eritrea was referred to our clinic with severe @entity10 and @entity3917 one month after onset. Ophthalmological examination disclosed a bilateral @entity1829 . Recurrent oral ulcers and @entity14663 were present. Pathergy test was positive. THERAPY AND OUTCOME: Complementary investigations disclosed an inflammatory syndrome. Computertomography was normal. Cranial magnetic resonance venography disclosed multiple @entity50 (superior sagittal sinus, sigmoid sinus and lateral left sinus). There was no evidence of @entity166 , @entity32 . A Beh  et's disease was diagnosed based on the medical history and clinical examination. Whilst his general health deteriorated rapidly before treatment, he improved quickly after the onset of therapy with immunosuppression ( @entity229 and @entity11635 ) in combination with anticoagulation ( @entity1524 /Sintrom). CONCLUSION: Beh  et's disease is uncommon and difficult to diagnose in @entity1 . Neurologic involvement as a first presentation is rarely described, but might be underestimated. We emphasise, therefore, the systematic application of magnetic resonance venography in @entity1 with unclear persistent @entity10 .
