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

TLDR

The study aims to define neurological involvement patterns in Behçet's disease and evaluate prognostic factors. The authors reviewed 558 clinic files, excluded non‑Behçet or non‑neurological cases, and evaluated 200 patients (155 male, 45 female) for parenchymal versus non‑parenchymal CNS involvement, classifying lesions and performing survival analysis on those with at least three years of neurological disease. Parenchymal involvement was present in 81% of patients, most commonly affecting the brainstem and associated with pyramidal signs, hemiparesis, behavioral changes, and CSF hypercellularity or elevated protein; non‑parenchymal cases mainly manifested as raised intracranial pressure from dural sinus thrombosis, MRI revealed brainstem or basal ganglia lesions in over half of parenchymal cases, and poorer prognosis correlated with parenchymal disease, CSF abnormalities, brainstem‑plus type, progressive course, and relapse during steroid tapering.

Abstract

In order to define the patterns of neurological involvement in Behçet's disease and to assess prognostic factors, 558 files of the neuro-Behçet out-patient clinic were reviewed. Those patients without any evidence of objective neurological involvement as well as the patients with other possible explanations for the neurological picture, and cases not fulfilling the criteria for Behçet'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%, hemispheric involvement in 15% and isolated pyramidal signs in 19%) while 38 had secondary or non-parenchymal CNS involvement. In the first group the most common findings were pyramidal signs, hemiparesis, behavioural changes and sphincter disturbance, whereas in the second group the syndrome of raised intracranial pressure due to dural sinus thrombosis was the main clinical manifestation. In 60% of the cases with parenchymal involvement, CSF was hypercellular and/or had an elevated protein level, whereas in cases with non-parenchymal involvement the CSF was usually normal except for the elevated pressure. In more than half of the patients with parenchymal involvement, MRI showed brainstem and/or basal ganglion lesions. Forty-one per cent of the cases had a course with at least one attack and remission, another 28% also had attack(s) but showed secondary progression, 10% had primary progression and 21% had silent neurological involvement. Survival analysis was performed in patients who had at least a 3-year duration of neurological disease. Parenchymal involvement, elevated protein and/or pleocytosis in the CSF, 'brainstem +' type involvement, primary or secondary progressive course and relapse during steroid tapering were all associated with a poorer prognosis.