Neurological involvement in paediatric Behçet's disease.

Introduction: Behçet disease (BD) is a systemic vasculitis which can affect the neurological system. Neuro-Behçet's disease (NBD) is not well described in children.
Objective: Our study aimed to analyse the clinical patterns of paediatric NBD and to describe their repercussions on children's schooling.
Methods: We performed a retrospective chart review of 12 NBD children and a phone interview of patients and their physicians to investigate their last physical and schooling status.
Results: In 40 BD patients reviewed, 12 (sex ratio 8M/4F) had neurological involvement. The mean age of onset was 11 years. In 4 cases, neurological symptoms were the initial presentation. In other cases, NBD occurred within a mean time of 10 months after BD was diagnosed. Cerebral venous thromboses were frequent (5/12) as compared to recurrent meningoencephalitis (2/12), rhombencephalitis (2/12), transverse myelitis (1/12), peripheral neuropathy (1/12) or psychiatric disturbances (1/12). 9 patients had sequelae and 8 had difficulties in learning. 6 stopped at middle school level. For the other patients, an arrangement of the teaching environment was needed due to visual, auditory and concentration disorders.
Conclusion: Neurological involvement is frequent in BD children and its consequences could be severe. Timely accommodations are required to facilitate their ability to follow the normal curriculum.
(© Georg Thieme Verlag KG Stuttgart · New York.)