Behçet's disease is a multisystem vasculopathy of unknown cause, commonly involving the centrai nervous system. The disease is characterised by narrowing of vascular calibre, possible thrombosis and fibrinoid necrosis with rupture of the vessel wallleading to haemorrhagic ischaemia areas and sometimes confluent areas of demyelination in addition to necrosis and gliosis. Without specific laboratory findings, diagnosis is based on coded criteria. The disease is characterized by the classic triad of mucous membrane ulceration of mouth, genitals and uvea which may be associated with thrombophlebitis, arthritis, pyoderma and intestinal ulcerations. Neurologic abngrmalities worsen the prognosis: they occur in between 16 and 40% of cases and may present with cerebral or less commonly spinal cord lesions. Magnetic resonance imaging offer additional diagnostic information in acute disease and in acute presentations of chronic forms with intermittent or progressive course. The most common lesions in acute disease have been described in the brain stem (mainly the pons and mesencephalon), basai ganglia, cerebellum and supratententorial (less frequently periventricular) white matter. Chronic disease is more difficult to diagnose as it presents diffuse involvement of the hemispheric white matter. Brain stepz atrophy is rare, but when not associated with cortical atrophy is 100% specific for neuro-Behçet's disease. MR investigation is also useful in follow-up to monitor treatment efficacy. Spinallesions are much rarer than cerebral involvement and mainly affect the dorsal spine. We describe a patient presenting concomitant brain and spinal cord involvement who underwent magnetic resonance study.
|Titolo:||Brain and spinal cord involvement in Behcet's disease: a case report|
|Data di pubblicazione:||2001|
|Appare nelle tipologie:||1.1 Articolo in rivista|