Behcet's disease: diagnostic and prognostic aspects of neurological involvement

This study was conducted to describe clinical and prognostic aspects of neu rological involvement in Behcet's disease (BD). Patients referred for neuro logical evaluation fulfilled the criteria of the International Study Group for Behcet's Disease. We analyzed disability and survival by the Kaplan-Mei er method, using Kurtzke's Extended Disability Status Scale (modified for E D) and the prognostic effect of demographic and clinical factors by Cox reg ression analysis. We studied 164 patients; of the 107 diagnostic neuroimagi ng studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thromb osis (VST) and the others were normal. CSF studies were performed in 47 pat ients; all with inflammatory CSF findings (n=18) had parenchymal involvemen t. An isolated increase in pressure was compatible with either VST or norma l imaging. The final diagnoses were VST (12.2 %), neuro-Behcet syndrome (NB S) (75.6 %), isolated optic neuritis (0.6 %), psycho-Behcet syndrome (0.6 % ), and indefinite (11 %). VST and NBS were never diagnosed together. Ten ye ars from onset of ED 45.1 % (all NBS) reached a disability level of EDSS 6 or higher, and 95.7+/-2.1 % of the patients were still alive. Having accomp anying cerebellar symptoms at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurolog ical diagnoses in ED are NBS and VST. These are distinct in clinical, radio logical, and prognostic aspects, hence suggesting a difference in pathogene sis.