Neuro-Behçet syndrome.
Sabahattin Saip1, Gulsen Akman-Demir2, Aksel Siva1
1Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
Handbook of Clinical Neurology
|December 25, 2013
Summary
Behçet syndrome (BS) involves the nervous system in 5-10% of cases, presenting as either central nervous system inflammation or venous sinus thrombosis. Understanding these neuro-Behçet syndrome (NBS) subtypes is crucial for managing this complex vascular-inflammatory condition.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Behçet syndrome (BS) is a chronic, relapsing, multisystem vascular-inflammatory disorder of unknown etiology.
- Nervous system involvement, termed neuro-Behçet syndrome (NBS), affects 5-10% of patients, presenting with diverse clinical manifestations.
- BS is characterized by a wide range of organ involvement, necessitating its classification as a syndrome due to its heterogeneity.
Purpose of the Study:
- To subclassify primary neurologic involvement in Behçet syndrome.
- To differentiate between intra-axial and extra-axial forms of neuro-Behçet syndrome.
- To discuss the clinical characteristics, pathogenesis, and treatment challenges of neuro-Behçet syndrome.
Main Methods:
- Clinical and imaging evidence analysis to identify distinct patterns of neurological involvement.
- Review of histopathological findings to understand the underlying disease mechanisms.
- Assessment of current treatment strategies and their limitations in neuro-Behçet syndrome.
Main Results:
- Neuro-Behçet syndrome (NBS) can be subclassified into two main forms: intra-axial (vascular-inflammatory CNS disease) and extra-axial (cerebral venous sinus thrombosis with intracranial hypertension).
- Intra-axial NBS often presents with subacute brainstem syndromes and hemiparesis, while extra-axial NBS typically involves fewer symptoms and has a better prognosis.
- Vascular-type headaches are common in BS, independent of neurological involvement, and rare manifestations include isolated behavioral syndromes and peripheral nervous system issues.
Conclusions:
- Neuro-Behçet syndrome is a heterogeneous condition with distinct intra-axial and extra-axial subtypes, likely differing in pathogenesis.
- The diverse nature of NBS complicates prediction of its course, prognosis, and response to treatment.
- Current treatment options for NBS, including immunosuppressants and anti-TNF agents, lack robust evidence for efficacy, highlighting the need for further research.
Keywords:
Behçet’s diseasecerebral venous sinus thrombosisdifferential diagnosisnervous system involvementneuro-Behçet syndrometreatmentMore Related Videos
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