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Semin Neurol 2014; 34(04): 437-443
DOI: 10.1055/s-0034-1390392
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Neuro-Behçet Disease and Autoinflammatory Disorders

Julie J. Miller
1   Department of Neurology, Massachusetts General Hospital and Brigham and Women's Hospital, Boston, Massachusetts
,
Nagagopal Venna
2   Neurology Clinic and Neuro-Infectious Disease Clinic, Massachusetts General Hospital, Neurology Associates, Boston, Massachusetts
,
Aksel Siva
3   Department of Neurology, Istanbul University, Cerrahpaşa School of Medicine, Cerrahpaşa, Istanbul, Turkey
› Author Affiliations
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Publication History

Publication Date:
04 November 2014 (online)

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Abstract

Misregulation of innate immunity leads to autoinflammation. Behçet disease is an autoinflammatory condition involving recurrent attacks of inflammation in skin, eyes, joints, and even the nervous system. The etiology may involve vascular inflammation. Central nervous system involvement in neuro-Behçet disease (NBD) comes in the form of parenchymal NBD or nonparenchymal NBD. The parenchymal form has a predilection for the brainstem, diencephalon and cerebral hemispheres, and represents a meningoencephalitis thought to be related to small vessel vasculitis. Cerebral venous sinus thrombosis, arising from a vasculitic process of large veins, comprises the majority of vascular NBD cases. The rarer monogenetic autoinflammatory syndromes are characterized by periodic fever, and typically present in the pediatric population. Neurologic involvement in these syndromes typically presents in the form of an aseptic meningitis. Treatment of autoinflammatory disorders involves immune modulation with corticosteroids, disease-modifying antirheumatic medications, and increasingly antibodies targeting cytokines like tumor necrosis factor α and interleukin 1.

Keywords

neuro-Behçet - autoinflammatory syndromes - neurologic disease - cerebral venous thrombosis - vasculitis
 

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