Clinical patterns of neurological involvement in Behcet's disease: evaluation of 200 patients

In order to define the patterns of neurological involvement in Behcet's dis ease and to assess prognostic factors, 558 files of the neuro-Behcet out-pa tient clinic were reviewed. Those patients without any evidence of objectiv e neurological involvement as well as the patients with other possible expl anations for the neurological picture, and cases not fulfilling the criteri a for Behcet's disease were excluded. The remaining 200 cases (155 male, 45 female) were evaluated: 162 had parenchymal CNS involvement (brainstem or 'brainstem +' involvement in 51 %, spinal cord involvement in 14%, hemisphe ric involvement in 15% and isolated pyramidal signs in 19%) while 38 had se condary or non-parenchymal CNS involvement. In the first group the most com mon findings were pyramidal signs, hemiparesis, behavioural changes and sph incter disturbance, whereas in the second group the syndrome of raised intr acranial pressure due to dural sinus thrombosis was the main clinical manif estation. In 60% of the cases with parenchymal involvement, CSF was hyperce llular and/or had an elevated protein level, whereas in cases with non-pare nchymal involvement the CSF was usually normal except for the elevated pres sure. In more than half of the patients with parenchymal involvement, MRI s howed brainstem and/or basal ganglion lesions. Forty-one per cent of the ca ses had a course with at least one attack and remission, another 28% also h ad attack(s) but showed secondary progression, 10% had primary progression and 21 % had silent neurological involvement. Survival analysis was perform ed in patients who had at least a 3-year duration of neurological disease. Parenchymal involvement, elevated protein and/or pleocytosis in the CSF,'br ainstem +' type involvement, primary or secondary progressive course and re lapse during steroid tapering were all associated with a poorer prognosis.