Increased IL-22 in cerebrospinal fluid of neuro-behçet's disease patients.

Remitting-Relapsing Multiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuro-inflammatory disorders leading to brain damage and disability in young adults. Herein, we investigated in these patients the cytokine response by beads-based multiplex assays during the early stages of these disorders. Cytokine investigations were carried out on treatment-naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Our findings demonstrate that Cerebrospinal Fluid (CSF) cells from NBD patients, but not RRMS, secrete significant high levels of IL-22 which is associated with elevated IL-22 mRNA expression. We also observed an increase in IL-22 levels in the definite NBD subgroup as compared to the probable NBD one, indicating a clear relationship between elevated IL-22 levels and diagnostic certainty. Interestingly, we found no correlation of IL-22 secretion between CSF and serum arguing about intrathecal release of IL-22 in the CNS of NBD patients. Moreover, we showed by correlogram analysis that this cytokine doesn't correlate with IL-17A, IL-17F and IL-21 suggesting that this cytokine is secreted by Th22 cells and not by Th17 cells in the CSF of NBD patients. Finally, we found elevated levels of IL-6 and a positive correlation between IL and 6 and IL-22 in the CSF of NBD. In conclusion, these results suggest that IL-6 contributes to the production of IL-22 by T cells leading to the exacerbation of inflammation and damage within the CNS of NBD patients.

Neurologic manifestations of Behçet disease: rheumatology experience.

OBJECTIVE: Neurologic involvement in Behçet disease (BD) is a rare manifestation. Herein, we aimed to evaluate the clinical features and treatment choices of neuro-Behçet (NB) patients. METHODS: There were records of 800 BD patients between 1998 and 2021. Fifty-five of the BD patients had NB and the files of these patients were retrospectively evaluated. Patients were grouped into three subgroups: 22 (40%) had non-parenchymal, 25 (45%) had parenchymal, and 8 (15%) had both parenchymal and non-parenchymal (mixed) involvement. RESULTS: Of the 55 patients, 32 were male. Twenty-six of the NB patients were diagnosed with BD simultaneously. The most common complaint was headache (n = 24, 44%). The most affected site was periventricular white matter (n = 21, 38%). All patients had received corticosteroids. Azathioprine (AZA; n = 39, 71%) was the most common immunosuppressive agent after corticosteroids, followed by cyclophosphamide (n = 16, 29%). CONCLUSION: Neurologic involvement is a rare complication of BD but is related to increased mortality and morbidity. Neurologic manifestations may be the initial symptom of BD, thus leading to diagnosis. Both neurology and rheumatology specialists should be aware of this rare condition.

Myelin basic protein and index for neuro-Behçet's disease.

Neuro-Behçet's disease (NBD) contributes to poor prognosis in BD patients which lacks reliable laboratory biomarkers in assessing intrathecal injury. This study aimed to determine the diagnostic value of myelin basic protein (MBP), an indicator of central nervous system (CNS) myelin damage, in NBD patients and disease controls. Paired samples of cerebrospinal fluid (CSF) and serum MBP were measured using ELISA, while IgG and Alb were routinely examined before the MBP index was developed. CSF and serum MBP in NBD were significantly higher than in NIND, which could distinguish NBD from NIND with a specificity exceeding 90%, moreover, they could also be excellent discriminators for acute NBD and chronic progressive ones. We found positive linkage between MBP index and IgG index. Serial MBP monitoring confirmed serum MBP's sensitive response to disease recurrences and drug effects, whereas MBP index suggests relapses prior to clinical symptoms. MBP has high diagnostic yield for NBD with demyelination and identifies CNS pathogenic processes before imaging or clinical diagnosis.

Diagnosis and management of Neuro-Behçet disease with isolated intracranial hypertension: a case report and literature review.

BACKGROUND: Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe the treatment of a pediatric patient with NBD, who exhibited isolated intracranial pressure elevation as indicated by the initial manifestation of diplopia and physical examination finding of papilledema. CASE PRESENTATION: An 8-year-old boy was admitted to the hospital with a confirmed diagnosis of Behçet's disease (BD) over eight months. The patient also presented with the symptom of diplopia for three days. The evaluation of the patient's nervous system did not reveal any apparent abnormalities. The measurement of cerebrospinal fluid pressure yielded a reading of 470 mm H2O. The examination of the fundus indicated papilledema, and imaging scans revealed evidence of focal demyelination. The symptoms of the child showed alleviation after the administration of mannitol, methylprednisolone, and azathioprine. Furthermore, this study involved a comprehensive analysis of 18 cases of NBD with isolated intracranial hypertension, comprising one case reported herein and 17 cases from the literature review. Three cases were children, and an equal distribution of males and females 9:9 was noted. The average age at the onset of symptoms was 24.7 years (8-38 years). Headache (90%) was the most commonly reported clinical manifestation, followed by blurred vision or diplopia (80%). The ocular manifestations included papilledema (100%), abducent nerve paralysis (20%) and local eye hemorrhages in the retina (30%). Notably, 88.9% of these ocular manifestations were relieved or cured after treatment. CONCLUSION: This study presents the first reported case of NBD with isolated intracranial hypertension in the pediatric population of China. In a child with Bechet's disease presenting with features of raised intracranial pressure, it is important to be aware of neuro Bechet's presenting with intracranial hypertension without other neurological abnormalities. This will help make early diagnosis, institute treatment and prevent sequelae resulting from untreated raised intracranial pressure.

Neuro-Behcet's disease misdiagnosed and treated as multiple sclerosis: a deceiving masquerader.

Behcet's disease is a chronic polysymptomatic systemic vasculitis disorder of unknown etiology characterized by several clinical manifestations in multiple organ systems. Involvement of the nervous system occurs in ∼9% of patients with Behcet's disease (ranging from 3 to 30%). Neuro-Behcet's disease is a great masquerader of multiple sclerosis. Diagnosing this disorder might be challenging, especially in a patient who does not fulfill the criteria of Behcet's disease while having a neurological presentation. We report a case of neuro-Behcet's disease who was misdiagnosed as having multiple sclerosis for many years and started on unnecessary disease-modifying therapy for multiple sclerosis. A thorough history, physical examination, and systematic investigations are mandatory to differentiate between these two conditions. Our case presentation raises awareness of the importance of differentiating between these two conditions since the consequences of misdiagnosis are catastrophic. The main challenges differentiating between multiple sclerosis and neuro-Behcet's are clinical and paraclinical, including neuroimaging.

Neuro-Behçet's Disease Onset in the Context of Tuberculous Meningoencephalitis: A Case Report.

Behçet's disease (BD) is a systemic vasculitis that frequently presents with a relapsing-remitting pattern. CNS involvement (Neuro-Behçet) is rare, affecting approximately 10% of patients. Its etiological mechanisms are not yet fully understood. The most commonly accepted hypothesis is that of a systemic inflammatory reaction triggered by an infectious agent or by an autoantigen, such as heat shock protein, in genetically predisposed individuals. Mycobacterium tuberculosis is known to be closely interconnected with BD, both affecting cell-mediated immunity to a certain extent and probably sharing a common genetic background. We present the case of a 34-year-old Caucasian woman who had been diagnosed with tuberculous meningitis 15 months prior, with significant neurological deficits and lesional burden on MRI with repeated relapses whenever treatment withdrawal was attempted. These relapses were initially considered as reactivation of tuberculous meningoencephalitis, and symptoms improved after a combination of antituberculous treatment and corticosteroid therapy. After the second relapse, the diagnosis was reconsidered, as new information emerged about oral and genital aphthous lesions, making us suspect a BD diagnosis. HLA B51 testing was positive, antituberculous treatment was stopped, and the patient was started on high doses of oral Cortisone and Azathioprine. Consequently, the evolution was favorable, with no further relapses and slow improvements in neurological deficits. To our knowledge, this is the first report of Neuro-Behçet's disease onset precipitated by tuberculous meningitis. We include a review of the available literature on this subject. Our case reinforces the fact that Mycobacterium tuberculosis infection can precipitate BD in genetically predisposed patients, and we recommend HLA B51 screening in patients with prolonged or relapsing meningoencephalitis, even if an infectious agent is apparently involved.

Cerebrospinal fluid level of neurofilament light chain is associated with increased disease activity in neuro-Behçet's disease.

BACKGROUND: Clinical exacerbations characterized with neurological symptoms are observed in around 10% of Behçet's disease (BD) patients and may culminate in severe disability. Although certain immunological factors have been associated with disease activity in neuro-Behçet's disease (NBD), biomarkers for monitoring the clinical outcome of NBD have not been properly investigated. METHODS: Levels of neurofilament light chain (NFL), homeobox protein Hox-B3 (HoxB3), and YKL-40 were measured in cerebrospinal fluid (CSF) samples of 23 parenchymal (n = 16) and nonparenchymal (n = 7) NBD patients obtained during NBD attacks by ELISA. Parameters of clinical progression and outcome were assessed for an average follow-up period of 3.9 ± 1.3 years. RESULTS: Parenchymal NBD patients showed elevated CSF levels of NFL, HoxB3, and YKL-40 as compared to nonparenchymal patients. NBD patients showing an increase in modified Rankin score (mRS) values during follow-up had significantly higher CSF NFL levels. Patients with relatively lower CSF NFL levels (<1000 ng/L) did not develop attacks or cognitive impairment interfering with daily life activities during follow-up. NFL levels correlated with disease duration and mRS at the last follow-up visit, while HoxB3 levels correlated with a number of attacks during follow-up. DISCUSSION: CSF level of NFL appears to predict the prospective somatic and cognitive disability in NBD patients and may thus be potentially used as a biomarker of clinical outcome in this disease.

Quality of life in neuro-Behçet's disease: a cross-sectional study.

PURPOSE: Behçet's disease (BD) is a multisystemic perivasculitis with recurrent oral aphthous ulcers, genital ulcers, and eye involvement which was first described in 1937. Neurological involvement in BD patients is referred to as neuro-Behçet's disease (NBD). Studies have shown that BD worsens quality of life. However, there is no study that conducts a head-to-head comparison of quality of life between NBD patients and healthy controls. METHODS: Forty patients with NBD and 39 age-sex-matched healthy control subjects were included in this study. Sociodemographic data of all participants were recorded; SF-36 was used to assess quality of life. RESULTS: NBD patients had significantly lower SF-36 scores of physical functioning, physical difficulty, bodily pain, and general health perception compared with healthy controls. However, there was no significant difference between the groups according to the SF-36 scores of role limitation due to emotional problems, mental health, vitality, and social functioning. There was no significant difference between parenchymal and non-parenchymal NBD groups according to SF-36, Beck's Depression Inventory, and Beck's Anxiety Inventory scores. Patients with cerebellar system involvement had significantly lower physical and social functioning scores compared with those who did not. Patients with brain stem involvement were found to have higher role limitation due to emotional problems, vitality, social functioning, physical pain, and general health perception scores compared with those without brain stem findings. CONCLUSIONS: NBD negatively affects many aspects of the quality of life.

Cerebral venous thrombosis in a sub-saharan African country: A preliminary monocentric study of a 70 case series at the neurology department of Fann teaching hospital in Dakar - Senegal.

INTRODUCTION: Cerebral venous thrombosis (CVT) are underdiagnosed in sub-saharan Africa where publications are uncommon. Our study aim was to describe the CVT diagnostic and therapeutic features through a senegalese case series. PATIENTS AND METHOD: A monocentric retrospective and prospective study was conducted at the adult Neurology department of Fann Teaching Hospital in Dakar (Senegal), between January 01, 2013 and April 30, 2020. It had included all CVT cases diagnosed by neurovascular imaging. RESULTS: Seventy CVT cases were collected including 48 women (68.6%). The average age of the patients was 35.2±14 years. The main neurological signs were headache (92.8%) and motor deficit (41.4%), with subacute onset in 67.2% of cases. The superior sagittal sinus (54.3%) and the transverse sinus (38.6%) were the most affected with multiple involvements in 27 patients (38.6%). Thirty patients (42.8%) had indirect parenchymal signs such as venous infarction (15.7%), cerebral edema (11.4%) or intracerebral hemorrhage (12.8%). The etiological factors were mostly infectious (41.4%) with meningoencephalitis (12.8%) and otorhinolaryngological infection (10%). Gyneco-obstetric factors (27%) and Behçet's disease (7%) were the main aseptic factors. In the short-term clinical course, curative anticoagulation (98.6%) had enabled a favourable outcome (mRS 0-1) in half of the patients. CONCLUSION: Our study, the largest series in sub-saharan Africa to this date, confirms that CVT is a young women disease. Infectious etiology is the most frequent at the Fann national teaching hospital (41.4% in Dakar against 6.5% in Germaine Bousser's series) even if the etiological assessment is limited by financial constraints (no coagulopathy/thrombophilia check-up).

Nutritional Status in patients with Neuro-Behçet's disease.

Background/aim: The aim of this study was to assess the nutritional status and risk factors for malnutrition in Behçet's disease (BD) and neuro-Behçet's disease (NBD) patients. Materials and methods: Medical recordings of 74 patients with BD without neurological involvement (BDWoNI), 72 patients with NBD, and 50 patients with other diseases (carpal tunnel syndrome or lumbar discopathy) were analyzed retrospectively. The serum analyses were performed in the inactive period of disease. Chronic malnutrition was defined as low levels of serum albumin (<3.5 g/dL) with normal sedimentation rate and normal serum CRP levels. Results: Six (8.3%) of the patients in NBD group, 1 (1.4%) of the patients in BDWoNI group, and none of the patients in control group had chronic malnutrition (p = 0.029). Malnutrition rate was higher in NBD than control group (p = 0.036). The mean expanded disability status scale score was 2.92 ± 3.35 (range: 0­8) in patients with malnutrition and 1.44 ± 1.76 (range: 0­9) in patients without malnutrition in NBD group (p = 0.457). The ratio of patients having a progressive disease course was 33.3% and 7.6% in patients with and without malnutrition in NBD group, respectively. The median value of the duration of neurological involvement was 2 years (0­16) in patients with malnutrition and 6.5 years (0­18) in patients without malnutrition in NBD group (p = 0.046). There was no association between malnutrition and medications, disability scores, functional system involvement or findings on brain MRI. Conclusion: Malnutrition was higher in patients with NBD. Higher disability level and progressive disease course may be risk factors for malnutrition in NBD. Malnutrition may be seen more frequently in earlier phases of neurological involvement.

Discriminative expression of CD39 and CD73 in Cerebrospinal fluid of patients with Multiple Sclerosis and Neuro-Behçet's disease.

Treg-mediated immune suppression involves many molecular mechanisms including the cleavage of inflammatory extracellular ATP to adenosine by CD39 ectoenzyme. In the peripheral blood of Multiple Sclerosis (MS) patients, it has been suggested that CD39+ Treg cells have the potential to suppress pro-inflammatory IL-17 secreting cells. Herein, we studied cellular phenotype and mRNA expression of CD39 and CD73 ectoenzymes in the Cerebrospinal fluid (CSF) of MS patients and another neuro-inflammatory disease: the Neuro-behçet's disease (NBD). Using qRT-PCR, we assessed mRNA expression of CD39 and CD73 as well as anti-inflammatory (IL-10) and pro-inflammatory (IL-6, TNF-α, IL-1ß) cytokines in patients Peripheral blood mononuclear cells (PBMCs) and CSF of 28 relapsing-remitting multiple sclerosis (RRMS), 20 NBD and 22 controls with non inflammatory neurological disorders (NIND). The most substantial result in the CSF was the higher expression of CD39 in both RRMS and NBD patients compared to NIND. While, the expression of CD73 in CSF samples of NBD was low. In RRMS samples, we detected a significant positive correlation of both CD39 and CD73 with IL-10 expression. Moreover, results by flow cytometry revealed a high percentage of CD39 Treg cells in RRMS CSF. CD39 was preferentially expressed on B cells of NBD. Regarding inflammatory response, we showed a significant increase of IL-6 mRNA expression in NBD patients CSF while in RRMS this increase concerned TNF-α. These results bring evidence that CD39 correlates positively with an anti-inflammatory IL-10 response in RRMS. In contrast, no such association was observed in CSF of NBD patients and CD39 was preferentially expressed on B cells.

Headache in Behçet's Disease.

PURPOSE OF REVIEW: Our objective is to describe the various neurologic manifestations of Behcet's syndrome with special attention to headache syndromes. RECENT FINDINGS: Most recent studies have focused on data collection to better characterize the relatively rare disorder of neuro-Behcets (NBD). In patients with Behcet's disease (BD) who are experiencing headache, most are primary headache disorders, specifically tension type or migraines. Worsening headache can, however, be a sign of increased Behcets disease activity. Behcet's disease is a chronic, systemic, inflammatory disorder of unknown etiology. The prevalence of BD is most notable in countries along the ancient Silk Road, particularly the Middle East. BD occasionally has neurologic manifestations, further categorized into parenchymal and non-parenchymal syndromes. Headache is the most frequently reported neurological symptom of BD. Primary headaches are the most common headache type among patients with BD with comorbid headache disorders with debate regarding whether migraine or tension-type headache is most prevalent. Of the secondary causes of headache in BD, cerebral venous thrombosis is the most common.

A Systematic Review of Neurocognitive Functioning in Behçet's Disease.

Behçet's disease (BD) is a vascular, inflammatory multisystem disorder with neuro-Behçet's (NBD) diagnosed in a subset of patients with neurological manifestations. The objective of this review was to determine whether neurocognitive dysfunction is observed in BD, in which neurocognitive domains, and whether there are differences in rates of dysfunction observed between BD and NBD groups. Studies of any methodology were included that reported results from standardized neurocognitive assessment measures in participants with BD or NBD. Twelve group comparison studies met the criteria for inclusion in the review (totalling 284 BD and 157 NBD participants), as well as 17 case study/series papers (11 BD, 35 NBD). Issues with blinding, incomplete data reporting and selective reporting bias were found across the group and case study/series papers, as well as inadequate statistical adjustment for multiple comparisons in the group studies, and the lack of the use of appropriate norms or adjustment for premorbid ability in the case series/studies papers. These quality issues impacted on the conclusions that could be drawn from the current literature. Neurocognitive dysfunction was found in NBD compared to health controls (HC) in a higher proportion of results across studies, than in comparisons between BD and HC groups. The domains in which neurocognitive attenuation was most often reported were visual spatial ability, working memory and acquired knowledge, with more than 25% of these results showing significantly lower functioning in both the BD and NBD groups compared to HC. More than 25% of the processing speed and long-term memory encoding and retrieval results were also lower for the NBD group, compared to HC. Group comparisons between NBD and multiple sclerosis participants indicated few significant differences in neurocognitive test results. The majority of case study/series participants were found to have some degree of attenuated neurocognitive functioning, as defined by case study/series authors.

What is the role of diffusion tensor imaging (DTI) in detecting subclinical pyramidal tract dysfunction in Behçet's and neuro-Behçet's cases?

The aim of this study is to investigate the pyramidal tract integrity with DTI in Behçet's and neuro-Behçet's cases. We performed this technique in two subgroups of neuro-Behçet's patients (parenchymal and vascular), and Behçet's cases without neurological involvement and control group. Totally, 28 patients were investigated. The control group was composed of 14 healthy people. Cranial MR and DTI were performed in three patient groups and the control group. At DTI, circular regions of interest (ROI) were symmetrically drawn on axial slices on the left and right sides along the pyramidal tract pathway at two levels: middle one third of the cerebral peduncle and posterior limb of the internal capsule. Fractional anisotropy (FA) values for each ROI were obtained by averaging all voxels within the ROI. Calculated FA values on both sides (left and right) of the posterior limb of the internal capsule and cerebral peduncle are significantly lower in all three patient groups when compared to the control group. But there is no any difference of FA values in the selected brain regions of three patient groups. FA values on the posterior limb of the internal capsule and cerebral peduncle do not show a statistically significant difference in parenchymal neuro-Behçet's cases. Our study demonstrates that DTI can detect subclinical pyramidal tract dysfunction in neuro-Behçet's and Behçet's patients. Detection of subclinical nervous system involvement is crucial for morbidity in Behçet's disease. For this reason, studies based on DTI, which include a large number of patients and explore different brain regions, are needed to guide clinicians.

Cerebrospinal fluid IL-10 as an early stage discriminative marker between multiple sclerosis and neuro-Behçet disease.

Multiple Sclerosis (MS) and Neuro-Behçet's Disease (NBD) are two recurrent disorders affecting the central nervous system (CNS) by causing inflammation and irreversible damage. Inaugural clinical symptoms for both diseases might be very similar and definitive diagnosis could be delayed. The present study aimed to find out possible differences at early stages in the transcription factors/cytokines expression profiles in blood and cerebrospinal fluid (CSF) of MS and NBD patients which could be useful discriminative markers. Cytokines and transcription factors related to Th1, Th2, Th17 and T regulatory populations were studied by quantitative RT-PCR simultaneously in PBMCs and CSF, from 40 patients presenting a first episode of clinical features related to CNS inflammation and 22 controls with non inflammatory neurological diseases enrolled mainly for severe headache. The follow up of 12 months did allow a definitive diagnosis of remitting relapsing MS (RRMS) in 21 patients and of NBD in the other 19 among those with CNS inflammation compared to controls. In initial blood samples, T-bet was significantly increased in NBD patients only while IFN-γ was elevated in patients who evolved into RRMS or NBD. IL-17a, GATA-3 and IL-4 were significantly lower in RRMS patients than in the NBD group. In initial CSF samples, ROR-γt, IL-17a and IFN-γ were significantly elevated in patients compared to controls. The most striking finding was the significant increase of CSF IL-10 that we did observe in NBD patients only. Thus, we propose CSF IL-10 as a predictive marker to help clinicians discriminating between these two neurological disorders.

Behçet's Syndrome and Nervous System Involvement.

PURPOSE OF REVIEW: Although Behçet's syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement "neuro-Behçet'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. RECENT FINDINGS: Recent laboratory and clinical findings include the discovery of antibodies to human and mouse neuro-fibrils showing cross-reactivity with Streptococcus spp. and Mycobacterium tuberculosis heat shock proteins (HSP) in NBS. Diagnostically, two distinct magnetic resonance imaging (MRI) patterns of spinal cord involvement have been observed: (a) "Bagel sign" and (b) "motor neuron" patterns. Therapeutic studies have shown that infliximab effectively prevents further relapses and stabilizes the symptoms of patients experiencing ongoing clinical relapses while being treated with single or multiple immunosuppressant drugs. Primary neurological involvement referred to as NBS. The majority of patients with NBS present with parenchymal involvement that commonly affects the brain stem-diencephalic region. Headache, dysarthria, ataxia, and hemiparesis are the main clinical features of NBS.

Parenchymal neuro-Behçet disease with erectile dysfunction and micturition disturbances: case report and literature review.

Parenchymal neuro-Behçet disease (NBD) is a serious clinic condition with a sub-acute or chronic disease course that results in incapability through pyramidal tract involvement. Though well-known consequences can deter a patient's life, both urinary symptoms and sexual dysfunction are underestimated complications of NBD and closely related in timing. Here, we report the case of a young male patient with parenchymal NBD who developed urinary incontinence and erectile dysfunction in addition to widespread pyramidal tract signs and symptoms. We discuss clinical features, prognosis and treatment of the case. A review of English literature was conducted for cases of concurrent urinary or sexual dysfunction complicating parenchymal-NBD.

Effects of in vivo and in vitro administration of neuro-Behcet's disease IgG.

Antibodies directed against membrane antigens of neuronal axonal processes (neuropil) have been recently identified in neuro-Behcet's disease (NBD) patients. To delineate the potential pathogenic action of these antibodies, pooled sera from seven NBD patients with neuropil antibodies and seven healthy controls were divided into purified IgG and IgG-depleted serum (IgG-DS) fractions and each fraction was administered into lateral ventricles of rats. NBD IgG-injected rats showed reduced locomotor activity in the open field test as compared to NBD IgG-DS, healthy control IgG, healthy control IgG-DS and PBS injected rats (n = 10 for each group). There were no significant differences among treatment groups by means of anxiety-like behaviors (assessed by elevated plus maze test) and learning/memory functions (assessed by passive avoidance test). Administration of NBD IgG on cultured SH-SY5Y neuroblastoma cells induced significantly increased cell death and apoptosis (as measured by nucleosome levels in the supernatants) as compared to other treatment groups. Our results suggest that IgGs isolated from sera of neuropil antibody-positive NBD patients have a neurotoxic action, which is presumably mediated by apoptotic mechanisms. Motor deficits frequently observed in NBD patients might at least partially be caused by the pathogenic action of anti-neuronal IgG.

Osteonecrosis in the both femoral heads in a patient with Neuro-Behcet's Disease.

Neuro-Behçet's disease (NBD) is a rare clinical entity that frequently presents neuro-psychiatric symptoms, and mesodiencephalic and pontobulbar lesions. There is only one published report about osteonecrosis in NBD. We report a patient whose first presentation was neurological NBD with presenting bilateral femoral heads osteonecrosis. A 36-year-old male was hospitalized with gait disorder, diplopia and speech disorder. The past medical history of the patient was unremarkable. The MR image showed mesencephalic lesion with oedemaas a hyperintense area. The present case was diagnosed as NBD and treated with methylprednisolone (1g /day) only for five days. One year after, bilateral hip pain developed. MR image of both hips showed well-demarcated areas of osteonecrosis in the bilateral femoral heads. The patient was operated by an orthopedic surgeon. Because early diagnosis and immediate treatment of osteonecrosis is very important, the physician must bear in mind that osteonecrosis might result from impaired microvascular involvement in even NBD.