Neurological Presentation of Neuro-Behçet’s Syndrome: Clinical Categories

Abstract

In an attempt to categorise the diverse neurological manifestations of Behçet’s syndrome, a group of 10 patients was prospectively studied; the diagnosis of Behpet’s syndrome was not known in any of them prior to their neurological presentation. A reasonably clear distinction can be made to divide the manifestations into three general categories with some overlap. Category I includes patients with increased intracranial pressure with or without cerebral venous sinus thrombosis. Category II are those presenting with a stroke. Category III are those patients who present with spinal cord involvement and CSF pleocytosis. Brainstem involvement was noted independently of the group. In general, patients with Neuro-Behçet’s syndrome showed little ocular involvement. The course of the illness both in morbidity and mortality tended to be better in category I, and mortality was only seen in category II patients. In populations susceptible to the disease, the clinical categorisation proposed may alert the clinician to the possibility of neuro-Behçet’s syndrome whenever patients with pseudotumour cerebri, dural sinus thrombosis, stroke in the young, unexplained myelitis or possible multiple sclerosis are encountered.

Clinico-Radiological Findings of Neuro-Behcet's Syndrome.

“…Previous investigators reported 4 cases of dural sinus thrombosis from 10 Behcet's syndromecases, but none of the present patients exhibited this (8). This result could be due to racial differences backed by some immunogenetic mechanism, or maybe one of the unique features of Japanese Neuro-Behcet's syndrome.…”

“…Approximately 5% of Behcet's syndrome patients may not develop mucocutaneous manifestations even after neurologic involvement (4,5). Previous reports have clinically determined the characteristic neurological involvement of Behcet's syndrome: brain stem type and meningoencephalitic type (6)(7)(8). However, using computed tomographic scans (CT scans), investigators often failed to detect the precise lesions corresponding to the clinical deficit.…”

Neuro-Behcet's Disease Manifesting as a Neoplasm-Like Lesion. Case Report.

“…2,13) Neuro-Behçet's disease (NBD), which comprises 10-20% of all cases of Behçet's disease, predominantly manifests as central nervous system symptoms, often involving the brainstem, basal ganglia, and the white matter of the cerebrum. 17) However, NBD occasionally manifests as a neoplasm-like lesion with significant mass effect. 2,4,6,[11][12][13][14][15][16] We describe a case of NBD with a large intracranial mass.…”

Intracranial Hemorrhage in Neuro-Behcet's Syndrome.

“…Clinically, it is characterized by uveitis, oral aphthae, genital ulcers, and skin lesions, although gastrointestinal, articular, pulmonary, and neurological lesions can also develop. Central nervous system (CNS) involvement has been reported in 10% to 49% of cases (2,3) and was characterized by meningoencephalitis, benign intracranial hypertension, headaches, seizures, cerebral vessel thrombosis, and vasculitis (4,5). Five percent of Behcet's syndrome patients were found to develop mucocutaneous lesions only after the appearance of CNSlesions (6).…”