neuro-Behcet's Disease Research Articles

Neuro-behçet disease in an african american adolescent.

An 18-year-old African American adolescent presented with 1 week of fever, headache, and left arm and leg weakness. On examination, he had scrotal ulcers, left hemiparesis, dysarthria, and bilateral dysmetria. There was no uveitis or papilledema, but he had a history of recurrent oral ulcers. The cerebrospinal fluid (CSF) was remarkable for moderate lymphocytic pleocytosis to 175/µL but was negative for infectious etiologies. Oligoclonal bands were absent from the CSF and an immunoglobulin index was normal. A serum workup for rheumatological disease including erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody, rheumatoid factor, angiotensin-converting enzyme, anti-Ro, and anti-La antibodies was unrevealing. Magnetic resonance imaging of the brain showed T2-weighted hyperintensities with enhancement from the left internal capsule to the pons. Additionally, there were nonenhancing foci of T2-weighted hyperintensities in the right paracentral lobule, left corona radiata, hippocampi, and cervical spine (Figure 1A and ​andB).B). A head magnetic resonance venogram revealed near occlusion of the superior sagittal sinus (Figure 1D). A hypercoagulable workup including antiphospholipid antibodies was negative. Based on the clinical presentation and radiographic findings, a diagnosis of neuro-Behcet disease (NBD) was made. Treatment with therapeutic low-molecular-weight heparin, high-dose intravenous corticosteroids, and cyclophosphamide resulted in significant neurological improvement. Figure 1. Neuroimaging in neuro-Behcet disease: fluid-attenuated inversion recovery (FLAIR) sequence (A) shows hyperintensities in the right cerebral peduncle and hippocampi. T1-postcontrast sequence (B) shows enhancement in the right thalamus. Cervical ... Neurological complications of Behcet disease are rare, typically reported as a 1% to 3% incidence in all cases of Behcet disease.1 This case illustrates some of the typical manifestations of NBD, which often include meningoencephalitis, parenchymal lesions, cerebral venous thrombosis, and aneurysm formation.1,2

Neuro-Behçet dans sa forme parenchymateuse. Caractéristiques cliniques et paracliniques (40 cas)

Describe the clinical and paraclinical characteristics of parenchymal neuro-Behcet's disease. This is a prospective, descriptive study, concerning 40 patients with parenchymal neuro-Behcet's disease. The patients were followed during 3 years, benefited a thorough physical examination and paraclinical made of inflammatory laboratory tests, infectious serology, serum/CSF autoimmunity assessment, brain/spine MRI and evoked potentials. We also evaluated the frequency of HLA-B51. We identified 22 men and 18 women. The average age was 32 years. The beginning was poly-symptomatic in 65% cases. Twenty-eight patients (70%) reported a decrease in visual acuity, 40% (16 cases) associated with uveitis, 33 cases (82.5%) complained of headache and 11 cases (27.5%) with dizziness. Inaugural signs consisted of motor disorders (50%) and balance disorders (40%). The inflammatory serum markers were positive in 75% and oligoclonal bands present in CSF were found in 7 patients. Infracentimetric demyelinating lesions in MRI study were located in the brainstem (52.5%), the subcortical white matter (40%), the periventricular region (42.5%), cerebellum (32.5%), basal ganglia (30%), internal capsule (25%) and corpus callosum (12.5%). The HLA-B51 was found in 53% of cases. Behçet's disease mainly affects young male. The neurological complications are highly polymorphic, involving severe vital or functional prognosis.

Analysis of various factors on the relapse of acute neurological attacks in Behçet's disease

Objectives. To investigate the influences of various factors on the relapse of acute neurological attacks in patients with Behçet's disease (BD).Methods. Sixty-one patients, who met the international classification criteria for BD, had attacks of acute neuro-Behçet's disease (NBD) and could be followed up at least for 2 months, including 60 patients in our multicenter retrospective survey on BD patients in 2011. The factors associated with relapse of acute neurological attacks were assessed.Results. Twenty-one of 61 patients had been taking cyclosporine A (CyA) at the onset of acute NBD. All the 21 patients with CyA and 33 of the 40 patients without CyA had eye involvement. There were no significant differences in demographic features, clinical symptoms, MRI findings, the need for, and responses to corticosteroid therapy including pulse therapy between patients with CyA and those without CyA. CyA was withdrawn in 19 of 21 patients with CyA. Of note, patients with CyA showed significantly lower relapse rates than those without CyA (HR 0.1283, 95% CI: 0.0788–0.7836, p = 0.0186 as calculated by log-rank test). Moreover, colchicine was found to reduce the relapse rates in patients with acute NBD without CyA (HR 0.2771, 95% CI: 0.0827–0.9422, p = 0.0450 [log-rank test]).Conclusion. These results indicate that CyA-related acute neurological manifestations are almost identical with CyA-unrelated acute events of NBD, except for the paucity of relapse on withdrawal of CyA. The data also demonstrate that colchicine is effective to prevent relapses of acute neurological attacks especially in patients with CyA-unrelated acute NBD.

Neuro-Behcet's disease with multiple ring-enhanced brain lesions

Neuro-Behcet's disease with multiple ring-enhanced brain lesions

Neuro-Behçet’s Disease: An Unusual Cause of Headache

Neuro-Behçet's disease (NBD) is a potentially fatal complication of Behçet's disease (BD) that can sometimes masquerade as a primary neoplasm, aseptic meningitis or multiple sclerosis. Headache in patients with BD may portend onset of NBD, but the majority of headache in BD is benign. Clinicians who are unaware of the specific neurological manifestations of systemic inflammatory disorders like BD may fail to consider the possibility of serious intracranial pathology. We illustrate these challenges with the case of a 50-year-old woman with a history of BD who presented with headache in the absence of initial focal neurological deficits. The diagnosis of NBD was missed on multiple occasions before the correct diagnosis was made. We describe the etiology of headache in BD, the specific neurological manifestations of BD that suggest NBD, and the utility of routine neurological exams for BD patients with chronic headache. We further discuss the appropriate use of neuroimaging for headache in BD, and we recommend consideration of NBD as a diagnosis for headache in patients suspected of having an underlying systemic disease.

Neuro‐behçet's disease in South Korea: clinical characteristics and treatment response

Neuro-behçet's disease (NBD) is a rare complication of Behçet's disease (BD) but is still important due to its morbidity and mortality. In this study, we sought to identify the characteristics of NBD by examining the clinical characteristics, and whether there were differences in the clinical characteristics or the treatment between relapsed and non-relapsed groups. We retrospectively reviewed the medical history of all patients diagnosed with BD at the Asan Medical Center in South Korea from January 1996 to May 2012. NBD was defined as neurological symptoms and compatible abnormalities identified by magnetic resononance imaging. Among 624 BD patients, 22 were diagnosed with NBD. The common initial neurological symptoms were headache, gait disturbance and dysarthria. The common involved sites were the diencephalon, including internal capsule, the thalamus and basal ganglia, followed by the brainstem. An increased signal intensity on T2-weighted images was the most common finding. All patients had an improved disease state after steroid treatment, regardless of combined treatment with cyclophosphamide. However, eight patients experienced a disease recurrence at 7.3 ± 5.1 months after treatment. There were no significant differences in treatment patterns between the relapsed group and the non-relapsed group. NBD may present with various neurologic manifestations with multifocal diencephalon and brainstem involvement. Most NBD patients had a good clinical outcome after steroid treatment, with or without a cytotoxic agent, but showed a pattern of relapse-remission. Therefore, careful long-term follow-up is needed in most cases of NBD.

Successful Treatment of Child Neuro-Behcet's Disease with Infliximab

Behcet’s disease is a systemic vasculitis, characterized by recurrent oral aphthous ulcers, recurrent genital ulcers, skin lesion, and ocular involvement. Monoclonal antibody to the tumor necrosis factor-α (TNF-α) is considered as a possible therapeutic approach to achieve clinical improvement, preventing relapse in Behcet’s disease refractory to conventional anti-inflammatory drugs or immunosuppressive durgs. We report the use of infliximab, which is one of the TNF-α monoclonal antibodies, in a 17-year-old girl with Behcet’s disease exhibiting severe mucocutaneous, ocular and neurological involvement refractory to standard treatment.

痛みをともなわず1日で横断性脊髄症を生じ広範な脊髄病変を呈した神経Behçet病の1例

A 64-year-old man had transverse myelopathy that rapidly progressed without pain over the course of 1 day. The cerebrospinal fluid interleukin-6 (CSF IL-6) level was extremely high (1,120 pg/dl). Spinal cord magnetic resonance imaging (MRI) showed a longitudinal extensive lesion extending from Th8 to the conus medullaris. Despite treatment with steroids and cyclophosphamide, the symptoms worsened, and the range of sensory disturbance spread. MRI showed that the lesion expanded to Th3 over the course of 2 months. Neuro-Behçet disease (NBD) was diagnosed on basis of the high CSF IL-6 level and HLA-B51 positivity, and treatment with infliximab was begun. The sensory disturbance improved slightly, and the CSF IL-6 level fell to the normal range (7.0 pg/ml). It is important to include NBD, which rarely presents with extensive spinal cord lesion, in the differential diagnosis when patients present with acute transverse myelopathy without pain.

Neurobehcet syndrome: Ear canal ulcer with ipsilateral facial nerve palsy: A rare presentation

Neurobehcet syndrome is the disease with multisystem involvement. It has known otological manifestations which mainly include hearing loss due the involvement of the labyrinth. Though literature reports some cases of Neurobehcet disease with facial nerve palsy, it is one of the rarest presentations. The diagnosis of neurobehcets is mainly based on the symptomatology as no definitive pathological tests are available. As an otologist is one of the first clinicians to come across a patient with facial nerve palsy, we report this case of neurobehcets disease presenting to us with an ear ulcer and ipsilateral facial nerve palsy.

Diagnosis and management of Neuro-Behçet’s disease: international consensus recommendations

Neuro-Behçet’s disease (NBD) is one of the more serious manifestations of Behçet’s disease (BD), which is a relapsing inflammatory multisystem disease with an interesting epidemiology. Though NBD is relatively uncommon, being potentially treatable, neurologists need to consider it in the differential diagnosis of inflammatory, infective, or demyelinating CNS disorders. Evidence-based information on key issues of NBD diagnosis and management is scarce, and planning for such studies is challenging. We therefore initiated this project to develop expert consensus recommendations that might be helpful to neurologists and other clinicians, created through an extensive literature review and wide consultations with an international advisory panel, followed by a Delphi exercise. We agreed on consensus criteria for the diagnosis of NBD with two levels of certainty in addition to recommendations on when to consider NBD in a neurological patient, and on the use of various paraclinical tests. The management recommendations included treatment of the parenchymal NBD and cerebral venous thrombosis, the use of disease modifying therapies, prognostic factors, outcome measures, and headache in BD. Future studies are needed to validate the proposed criteria and provide evidence-based treatments.

Efficacy of infliximab in neuro-Behçet’s disease presenting with isolated longitudinally extensive transverse myelitis

Efficacy of infliximab in neuro-Behçet’s disease presenting with isolated longitudinally extensive transverse myelitis

Recurrent headaches: a case of neurological Behçet's disease

A 48-year-old black male, of Nigerian heritage, presented with a 24-hour history of frontal headache of gradual onset. The headache characteristic was migranous, being described as throbbing in nature and located to the right frontal area with associated blurring of vision. Although similar to prior frequent headaches, there was now increasing unsteadiness on walking. Diagnosed 10 years earlier with Behçet's disease, the initial presentation was with oral and genital ulceration. Recurrent episodes of headache caused by neurological flare-ups resulted in a stroke at the age of 46 years. This previous stroke was ischaemic in character with involvement of the brainstem, pons, midbrain and right cerebral peduncle with extension into the right internal capsule. Surveillance brain imaging (computed tomographic and magnetic resonance imaging with venography) 10 months earlier showed brainstem disease activity (Figure 1a) with disease quiescence a month later (Figure 1b) following an escalation of immunosuppressant therapy. Regular medications comprised prednisolone 10 mg (however, regular recurrences had resulted in him taking doses of between 20 and 30 mg/day of prednisolone for most of the past 24 months) and azathioprine 150 mg daily, aspirin 75 mg daily, one adcal D3 twice daily with weekly alendronic acid, and omeprazole 20 mg daily. For headache he took topiramate 25 mg daily and for depression mirtazepine 15 mg daily. The patient was also addicted to a high level of cannabis use which he was reluctant to stop as he felt it helped his symptoms. On examination he was apyrexial and cardiovascularly stable. Neurological examination revealed a residual horizontal nystagmus to the right on lateral gaze, mild left hemiparesis with moderate spasticity, in addition to dysarthria and dysphonia from his prior stroke. A new feature was an exacerbation of gait unsteadiness. Blood tests were unremarkable and specifically the erythrocyte sedimentation rate was normal at 2 mm/hr (normal range 0-10 mm/hr). Immediate therapy involved an increase in steroid dosage to methyl prednisolone 1 g/day for 3 days, followed by oral prednisolone 60 mg daily. This was maintained for 2 weeks and then reduced by 10 mg/week to a maintenance dose of 10 mg/day. Magnetic resonance scanning revealed a marked increase in inflammation of the brainstem (Figure 1c). The patient required physiotherapy and occupational therapy with psychiatric input and was able to leave the hospital after 29 days.

Coexistence of amyotrophic lateral sclerosis with neuro‐Behçet's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient

We report the clinical and autopsy features of a 65-year-old Japanese man who clinically exhibited overlap of both neuro-Behçet's disease (NBD) and amyotrophic lateral sclerosis (ALS). The patient had a HLA-B51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. A brain and spine MRI study revealed a longitudinally extensive thoracic cord (Th) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from Th4 to Th9, exhibiting low intensity on T1-weighted images, high intensity on T2-weighted and fluid-attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both NBD and ALS, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, Bunina bodies and skein-like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between NBD and ALS.

Diagnosis and management of neuro-Behçet's disease: International consensus recommendations

Neuro-Behcet’s disease (NBD) is one of the more serious manifestations of Behcet’s disease (BD), which is a relapsing inflammatory multisystem disease with an interesting epidemiology. Though NBD is relatively uncommon, being potentially treatable, neurologists need to consider it in the differential diagnosis of inflammatory, infective, or demyelinating CNS disorders. Evidence-based information on key issues of NBD diagnosis and management is scarce, and planning for such studies is challenging. We therefore initiated this project to develop expert consensus recommendations that might be helpful to neurologists and other clinicians, created through an extensive literature review and wide consultations with an international advisory panel, followed by a Delphi exercise. We agreed on consensus criteria for the diagnosis of NBD with two levels of certainty in addition to recommendations on when to consider NBD in a neurological patient, and on the use of various paraclinical tests. The management recommendations included treatment of the parenchymal NBD and cerebral venous thrombosis, the use of disease modifying therapies, prognostic factors, outcome measures, and headache in BD. Future studies are needed to validate the proposed criteria and provide evidence-based treatments.

Mitochondrial carrier homolog 1 (Mtch1) antibodies in neuro-Behçet's disease

Efforts for the identification of diagnostic autoantibodies for neuro-Behcet's disease (NBD) have failed. Screening of NBD patients' sera with protein macroarray identified mitochondrial carrier homolog 1 (Mtch1), an apoptosis-related protein, as a potential autoantigen. ELISA studies showed serum Mtch1 antibodies in 68 of 144 BD patients with or without neurological involvement and in 4 of 168 controls corresponding to a sensitivity of 47.2% and specificity of 97.6%. Mtch1 antibody positive NBD patients had more attacks, increased disability and lower serum nucleosome levels. Mtch1 antibody might be involved in pathogenic mechanisms of NBD rather than being a coincidental byproduct of autoinflammation.

Characteristics of neurological manifestations of Behçet's disease: A retrospective monocentric study in Tunisia

ObjectiveThe aim of the present study was to analyze demographic, clinical and genetic features of Behçet's disease patients with neurological involvement through a monocentric study of a homogenous group of hospitalized patients observed in the same department and to compare them with those of other ethnic and geographic groups. MethodsFour hundred and thirty Behçet's disease (BD) patients were retrospectively studied. Diagnosis of BD was made according to the international study group for Behçet's disease criteria. Patients with neurological findings suggestive of involvement of the nervous system by BD were further studied according to clinical examination, laboratory tests and neuroradiological investigations. ResultsNeurological involvement was observed in 121 patients (28.1%). The mean age at neuro-Behçet's disease (NBD) onset was 29.7 years. Average disease duration of BD before neurological manifestations onset was 6.4 years. Male to female ratio was 1.8. Of the 121 NBD patients, parenchymal involvement occurred in 74 patients (61%). Among them 26 (21.4%) presented with brainstem involvement, 24 (19.8%) with hemispheric involvement and 2 (1.6%) with spinal cord involvement. Non-parenchymal NBD occurred in 47 patients (39%). Involvement of the main vascular structures (Vasculo-NBD) was the most common non-parenchymal NBD lesion found in 35 patients (28.9%) consisting of cerebral vein thrombosis (CVT) in 24 cases and cerebral arterial thrombosis in 11 cases. Forty-nine (40.5%) patients with NBD have been followed-up for a median of 3 years (range 1–19 years). Forty-one of them recovered well without significant residual disability, 5 patients made no improvement and are left with severe neurological impairments and 3 died. Male gender and CNS parenchymal lesions occurrence were significantly associated with a poorer prognosis. ConclusionClinical and epidemiological features of NBD are various. In our Tunisian cohort of NBD patients the main characteristic features were male predominance, a relatively high prevalence of CVT, a low prevalence of intra-cranial hypertension and a significant lower frequency of HLA-B51 haplotype.

Case of neuro‐Behçet's disease successfully maintained a remission using infliximab for 2 years

Behçet's disease is a systemic inflammatory disorder with chronic remissions and relapses. Involvement of the central nervous system, called neuro-Behçet's disease (NBD), is a serious complication. Although corticosteroids and immunosuppressive agents are used, there is no definite standard of treatment with long term-effects for NBD. Here, we report a female case with NBD successfully treated by infliximab and corticosteroids for 2years. Our report suggests treatment with infliximab may be effective for long-term remission of NBD.

Teaching Neuro Images : Longitudinally extensive transverse myelitis in neuro-Behçet disease

A 25-year-old man presented with subacute spastic paraparesis. He reported 2 previous episodes of spastic paraparesis with partial recovery. Recurrent oral and genital ulceration, pustular skin eruptions, and fever coexisted. Profound motor weakness, a sensory level at T10, oral ulceration, and a pustular eruption on the anterior abdominal wall were noted. Marked neutrophilia was noted in both blood and CSF. Neuromyelitis optica–immunoglobulin G autoantibody was negative. MRI (figure) demonstrated marked inflammatory changes. IV and oral steroids, followed by 6 months of pulsed IV cyclophosphamide, resulted in marked clinical improvement. Neuro-Behcet disease lies within the clinical differential for longitudinally extensive transverse myelitis.1,2

Cortical and cerebellar atrophy, but no lesion in a patient with neuro-Behçet’s disease

Neuro-Behçet's disease (NBD) is a rare clinical entity. There are no reports about cortical atrophy in NBD. We report a patient with NBD exhibiting only cortical and cerebellar atrophy without any lesions. A 38-year-old male was hospitalized due to gait disorder. He had experienced forgetfulness, irritability, sexual and behavioral dyscontrol, and incontinence for 5 years. Magnetic resonance imaging (MRI) showed cortical and cerebellar atrophy, but there were no lesions. In the differential diagnosis of NBD, physicians should take into account the presence of both brainstem and cerebellar and cortical atrophy even in the absence of typical lesions for NBD, particularly in the presence of cognitive dysfunctions.

Cognitive impairment in Brazilian patients with Behçet's disease occurs independently of neurologic manifestation

Neuro-Behçet's disease (NBD) presents cognitive and behavioral symptoms possibly explained by secondary dysfunction of frontal and temporal cortices due to subcortical damage, as NBD commonly involves the brainstem and basal ganglia. Nonetheless, there are reports of cognitive impairment in patients without neurological manifestations. ObjectiveTo evaluate cognitive function in Behçet's disease (BD) patients with and without neurological manifestations and to analyze clinical variables associated with cognitive deficits. MethodsThis is a cross-sectional study that compared healthy controls, BD patients without neurological manifestations and NBD patients. Each group comprised 24 participants. All participants underwent neuropsychological evaluation, Hamilton Anxiety Rating Scale (HAM-A), Beck Depression Inventory (BDI) application and brain MRI. Cumulative prednisone dose, years of education, and presence of white-matter lesions in brain MRI were recorded. Results41.6% of BD and 41.6% of NBD patients showed impaired language and executive function, whereas visual memory was impaired only in NBD patients. Multiple logistic regression revealed that anxiety (OR 1.09 95% CI 1.03–1.16, p=0.003) and lower educational level (OR 0.62 95% CI 0.48–0.80, p<0.0001) were independently associated with cognitive impairment. ConclusionCognitive impairment occurs frequently in patients with BD independently of neurological manifestation. Low educational level and anxiety are risk factors for cognitive impairment in BD.