Beh çet’s Syndrome and Nervous System Involvement

AbstractPurpose of ReviewAlthough Beh çet’s syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement “neuro-Behçet’s syndrome (NBS)” is commonly included in the differential diagnosis of many inflammatory and vascular central nervous system (CNS) disorders. Clinical and neuroi maging findings support two major forms of NBS: parenchymal NBS (p-NBS) and an extra-parenchymal form that presents with cerebral venous sinus thrombosis (CVST). The present review summarizes recent findings on the etiopathogenesis and clinico-radiological features of this disorder as well as its tr eatment options.Recent FindingsRecent laboratory and clinical findings include the discovery of antibodies to human and mouse neuro-fibrils showing cross-reactivity withStreptococcus spp. andMycobacterium tuberculosis heat shock proteins (HSP) in NBS. Diagnostically, two distinct magnetic resonance imaging (MRI) patterns of spinal cord involvement have been observed: (a) “Bagel sign” and (b) “motor neuron” patterns. Therapeutic studies have shown that infliximab effectively prevents further relapses and stabilizes the symptoms of patients experiencing ongoing clinical relapses while being treated with single or multiple immunosuppressant drugs.SummaryPrimary neurological involvement referred to as NBS. The majority of patients with NBS present with parenchymal involvement that commonly affects the brain stem-diencephalic region. Headache, d...
Source: Current Neurology and Neuroscience Reports - Category: Neuroscience Source Type: research