Behçet's syndrome and the nervous system.
OBJECTIVE
Behçet's syndrome (BS) is a multi-system, vascular-inflammatory disease of unknown origin, involving the nervous system in a subgroup of patients. The syndrome is rare, but as patients with BS are young and frequently present with an acute or subacute brainstem syndrome or hemiparesis, as well as with other various neurological manifestations, the syndrome is often included in the differential diagnosis of multiple sclerosis, stroke of the young adult, and another wide range of neurological disorders. The present review summarizes the neurological involvement in BS, and emphasizes recent clinical concepts and ethiopathogenetic findings.


RESULTS
Over the last years the growing clinical and imaging evidence had suggested that neurological involvement in BS may be subclassified into two major forms: one, which is seen in the majority of patients, may be characterized as a vascular-inflammatory CNS disease, with focal or multifocal parenchymal involvement; the other, which has few symptoms and a better neurological prognosis, may be caused by isolated cerebral venous sinus thrombosis and intracranial hypertension. These two types rarely occur in the same individual, and their pathogenesis is likely to be different. A nonstructural vascular type headache is relatively common, whereas isolated behavioral syndromes and peripheral nervous system involvement are rare.


CONCLUSIONS
The involvement of the nervous system in BS is heterogeneous as clinical and imaging data reveal. Currently it is unknown which factors determine or have a role in the development of neurological involvement, but some progress has been achieved in understanding the neurological spectrum of the syndrome, which may lead to a better management of these patients.
Siva
Aksel
A
Department of Neurology, Clinical Neuroimmunology Division, Cerrahpaşa School of Medicine, University of Istanbul, Cerrahpaşa 34303, Istanbul, Turkey. asiva@ynn.net
Altintas
Ayse
A
Saip
Sabahattin
S
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D001933:Brain Stem / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D019586:Intracranial Hypertension / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009420:Nervous System / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D010291:Paresis / Q000503:physiopathology; D011859:Radiography; D014657:Vasculitis / Q000473:pathology / Q000503:physiopathology
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D008775:Methylprednisolone