Behçet’s Disease and Neuro-Behçet’s Syndrome

Behçet’s disease is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin, which usually presents with orogenital ulceration and uveitis and is identified as the triple-symptom complex. Primary neurological involvement in Behçet’s disease is known as neuro-Behçet’s syndrome (NBS). Clinical findings and neuroimaging demonstrate that there are two major forms of NBS: a central nervous system inflammatory parenchymal disease, and a less common nonparenchymal form that involves the large extraparenchymal vascular structures, mainly the venous dural sinuses. Cranial magnetic resonance imaging (MRI) typically reveals brainstem lesions with parenchymal involvement and an occluded dural sinus may be seen in the extraparenchymal type. Cerebrospinal fluid studies typically indicate inflammatory changes in the parenchyma and increased pressure with extraparenchymal involvement. Drugs used for the preventive treatment of NBS include azathioprine, cyclophosphamide, and anti-TNF agents

Oligoclonal bands increase the specificity of MRI criteria to predict multiple sclerosis in children with radiologically isolated syndrome

Background: Steps towards the development of diagnostic criteria are needed for children with the radiologically isolated syndrome to identify children at risk of clinical demyelination. Objectives: To evaluate the 2005 and 2016 MAGNIMS magnetic resonance imaging criteria for dissemination in space for multiple sclerosis, both alone and with oligoclonal bands in cerebrospinal fluid added, as predictors of a first clinical event consistent with central nervous system demyelination in children with radiologically isolated syndrome. Methods: We analysed an international historical cohort of 61 children with radiologically isolated syndrome (18 years), defined using the 2010 magnetic resonance imaging dissemination in space criteria (Ped-RIS) who were followed longitudinally (mean 4.2 4.7 years). All index scans also met the 2017 magnetic resonance imaging dissemination in space criteria. Results: Diagnostic indices (95% confidence intervals) for the 2005 dissemination in space criteria, with and without oligoclonal bands, were: sensitivity 66.7% (38.4\u201388.2%) versus 72.7% (49.8\u201389.3%); specificity 83.3% (58.6\u201396.4%) versus 53.9% (37.2\u201369.9%). For the 2016 MAGNIMS dissemination in space criteria diagnostic indices were: sensitivity 76.5% (50.1\u201393.2%) versus 100% (84.6\u2013100%); specificity 72.7% (49.8\u201389.3%) versus 25.6% (13.0\u201342.1%). Conclusions: Oligoclonal bands increased the specificity of magnetic resonance imaging criteria in children with Ped-RIS. Clinicians should consider testing cerebrospinal fluid to improve diagnostic certainty. There is rationale to include cerebrospinal fluid analysis for biomarkers including oligoclonal bands in planned prospective studies to develop optimal diagnostic criteria for radiologically isolated syndrome in children

Nervous system involvement in Behcet's syndrome

Purpose of review Neurological involvement in Behcet's syndrome is defined as ` the occurrence of neurological symptoms and signs in a patient who meets the International Diagnostic Criteria for BS not otherwise explained by any other known systemic or neurological disease or treatment, and in whom objective abnormalities consistent with neuro-Behcet's syndrome (NBS) are detected either on neurological examination, neuroimaging studies (magnetic resonance imaging [MRI]), and/ or on cerebrospinal fluid (CSF) examination'. Given that the neurological involvement of Behcet's syndrome carries a poor prognosis, we aimed to describe the differential diagnosis of NBS and highlight the different radiological patterns together with the treatment options. Recent findings Two distinct MRI patterns of spinal cord involvement in Behcet's syndrome according to T2-weighted axial images were described: ` Bagel Sign' pattern: a central lesion with hypointense core and hyperintense rim with or without contrast enhancement; and ` Motor Neuron' pattern: a symmetric involvement of the anterior horn cells. Infliximab prevents patients from having further attacks and even led to improvement in the neurological examination. Summary As the treatment options completely differ, a NBS diagnosis should be carefully made in patients with clinical and MRI features mimicking other central nervous system inflammatory disorders

An uncommon disease included commonly in the differential diagnosis of neurological diseases: Neuro-Behcet's syndrome

Behcet's Syndrome (BS) may present with different neurological problems, related either directly (primary) or indirectly (secondary) to BS. Primary neurological involvement is named as neuro-Behcet's syndrome (NBS), and its two major subtypes that are classified mainly on the clinical and MRI findings are (1) Parenchymal-NBS (pNBS) and (2) Extra parenchymal neuro-vascular involvement mostly seen as cerebral dural venous sinus thrombosis (CVST). The less commonly seen forms of NBS are cognitive and behavioral syndromes and peripheral nervous system involvement. Parenchymal-NBS is the most common clinical neurological presentation of BS. It is a rare disease with distinct MRI features and is often included in the differential diagnosis of neuro-vascular and neuro-inflammatory disorders. The most commonly affected neuro-anatomical site in p-NBS is the meso-diencephalic junction (MDJ), followed by the ponto-bulbar and thalamic regions, the basal ganglia, and the spinal cord. These varied locations may explain to a certain extent why BS is considered in the differential of so many neurological disorders. The other relatively common form of NBS that results in CVST may also be confused with other conditions resulting in CVST, especially when the systemic clinical features suggestive of BS are missed

Behcet's Syndrome and the Nervous System Involvement

Behcet's syndrome (BS), a distinct disease with orogenital ulceration and uveitis known as the triple-symptom complex, is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin. The neurological involvement of BS is termed as neuro-Behcet's syndrome (NBS). Direct neurological involvement of BS may be classified into two forms: (1) parenchymal (p-NBS) and (2) vascular involvement. p-NBS with the rate of 75% among neurological involvements of BS usually presents with an acute-subacute brain stem syndrome. Headache, cranial neuropathy, dysarthria, ataxia, and hemiparesis are the most prominent symptoms. The most common areas affected in p-NBS are the mesodiencephalic junction (MDJ), pons, and medulla oblongata. MDJ lesions tend to extend upward to involve the diencephalic structures and downward to involve the pontobulbar region which is the most common radiological finding observed in p-NBS. The only drug that has been shown to be effective based on the Class IV evidence is infliximab for the treatment of p-NBS

Behcet's Syndrome and Nervous System Involvement

Although Beh double dagger et's syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement "neuro-Beh double dagger et's syndrome (NBS)" is commonly included in the differential diagnosis of many inflammatory and vascular central nervous system (CNS) disorders. Clinical and neuroimaging 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 treatment options

Long-Term Prognosis After Revascularization Therapy in Patients with Atherothrombotic Stroke

AIM: Many guidelines recommend carotid endarterectomy (CEA) in symptomatic patients with carotid stenosis of greater than 70%, and carotid artery stenting (CAS) as an alternative to CEA. In our study, we evaluated the clinical characteristics of patients who underwent revascularizaton therapy