Intro
Preface
Contents
Neuro-Behçet's Disease: Epidemiology and Genetics
Introduction
Epidemiology
Prevalence of Neuro-Behçet's Disease
Age
Gender
Genetic Factors
Familial Aggregation
HLA-B51
Other MHC Class I Genes
Non-MHC Complex Genes
Genome-Wide Association Studies
Il-10
IL23R-IL12RB2
IL12A
ERAP1
STAT4
TNFAIP3
CCR1-CCR3
KLR
GIMAP
FUT2
CPLX-1
Other Polymorphism Studies
Rare Non-synonymous Variants
Epigenetic Studies
Conclusion
References
Immunology of Neuro-Behcet's Disease (NBD)

Immune Response in the CSF
Cellularity of CSF
Immune Mediators as Indications of Activity in CSF
Oligoclonal Bands (OCB)
Other Markers in the CSF
Cytokines
Candidate Antigens of Possible Autoimmune Response in NBD
Heat Shock Protein (HSP) and Related Responses
Neuronal and Other Proteins
Cellular Immune Response in NBD
Other Mechanisms in BD
Inflammation, Coagulation, and Fibrinolytic Pathway Abnormalities
Severity and Gender
Conclusions
References
Autoantibodies in Neuro-Behçet Disease
References
Animal Models of Behçet's Disease
Introduction

Infection-Induced Models
Herpes Simplex Virus-Induced Model
Streptococcal Infection Model
Genetic Models
Immunological Models
Heat Shock Protein Model
Anti-neuronal Antibody Model
Tropomyosin Model
Retinal S-Antigen Model
Environmental Models
Therapeutic Studies of BD Animal Models
Conclusion
References
Pathology
Parenchymal NBD
Histological Findings
Non-parenchymal NBD
Peripheral Nervous System Involvement
References
Behçet's Disease: Clinical Features
Introduction
Clinical Features
Mucocutaneous Involvement

Recurrent Aphthous Ulcers
Genital Ulcers
Cutaneous Lesions
Pathergy Reaction
Eye Involvement
Musculoskeletal Disease
Vascular Involvement
Gastrointestinal Involvement
Other Clinical Findings
References
Neuro-Behçet Syndrome: Differential Diagnosis
Differentiation of p-NBS from Other Mimickers by MRI Patterns
Differentiation of p-NBS from Other Mimickers by Laboratory Findings
Differentiation of p-NBS from Other Mimickers by Pathological Findings
References
Neuro-Behçet Syndrome: Clinical Features
Parenchymal NBS
Neurovascular Involvement

Cerebral Venous Sinus Thrombosis (CVST)
Arterial Occlusion and Aneurysm Formation
Primary Neurological Conditions Other than p-NBS and Neurovascular Involvement
Regarding Clinical Features, When Should We Suspect NBS?
References
The Cerebrospinal Fluid Presentations of Neuro-Behçet Disease
CSF Cells
CSF Protein
CSF Glucose
Oligoclonal Bands
Interleukin-6
Other CSF Studies
References
Imaging of Neuro-Behçet's Disease
Introduction
Neuroimaging Tools in NBD