Clinical characteristics of paediatric neuro-Behçet's disease: a single tertiary centre experience.

OBJECTIVES: To investigate the demographic and neurological features and treatment modalities of neuro-Behçet's disease (NBD) in children, to share our experiences and to summarise the literature.

METHODS: We retrospectively reviewed the medical records of Behçet's disease (BD) patients who attended our paediatric rheumatology department between December 2005 and October 2013. Five patients had the diagnosis of NBD. Initial neurological presentation, clinical BD presentation, magnetic resonance imaging pictures of those five patients was recorded.

RESULTS: A total of 18 patients were diagnosed with BD. Among BD patients five of them were identified with NBD (27.8%). The mean age of NBD patients at the time of diagnosis was 12.4 years (range 5.5-15 years). The mean follow-up time after the neurological involvement was 5.2 years (range 0.5-14). In two cases neurological involvement occurred at the same time with the onset of other clinical findings of BD (40%). Both of these patients had parenchymal involvement. Three patients were admitted with headache as the initial neurological symptom. They revealed benign intracranial hypertension. One of them had cerebral venous sinus thrombosis (CVST). The other two had normal cranial magnetic resonance imagines. All patients received colchicine and steroid, two of them who had parenchymal involvement received also cytotoxic drugs.

CONCLUSIONS: This study has shown that neurological symptoms can be the first manifestations of BD in children. Clinicians should be aware of this possibility and when a patient presents with neurological manifestations, it would be valuable to query the patient for the clinical features of Behçet's disease.