Susac Syndrome: Clinical characteristics, diagnostic findings and treatment in 19 cases.

Susac's Syndrome (SS), which was first described in 1979, is a rare and presumably autoimmune disorder characterized by encephalopathy, hearing loss, and visual disturbance resulting from branch retinal artery occlusion (BRAO). This study reports 19 SS patients' clinical characteristics, MRI features, CSF analysis, treatment strategies and outcomes. At initial presentation, only three of 19 patients demonstrated the complete clinical triad. Clinic presentation varied from isolated hemiparesis to the full triad (encephalopathy, hearing loss and visual disturbances). Corpus callosum (CC) involvement was noted in the MRI of 18 patients (97%) and BRAO was detected in 17 (95%) patients. All patients were treated with intravenous methylprednisolone after the initial assessment. This case series is presented to emphasize the differences in clinical presentation of SS and the importance of MRI and FFA in diagnosis.

Hemodialysis-related headache and how to prevent it.

BACKGROUND AND PURPOSE: Hemodialysis (HD) may have some adverse effects on the nervous system. Headache is the most commonly reported neurological symptom amongst HD patients. Our aim was to determine the frequency, clinical characteristics and triggering factors of HD-related headache (HRH) and to evaluate preventive strategies for reducing HRH. METHOD: In all, 494 patients were included. Comparative controls (CC) were classified within the same patients without headache. Arterial systolic/diastolic blood pressure, blood urea nitrogen (BUN) and creatinine were correlated before/after one HD. The urea reduction ratio during the dialysis session was determined. RESULTS: A total of 175 patients (35.4%) with a mean age of 57.3 ± 15.7 years were diagnosed with HRH. HRH was more common in males (P < 0.001). Headache was started a mean of 2.90 ± 0.86 h after the HD. The common localization of pain was reported to be bifrontal in 41.7% (n = 73). The mean duration of headache was 6.22 ± 7.8 h, with a duration of ≤4 h reported by 64.0% of patients. The mean Visual Analog Scale score was 5.64 ± 2.05. The differences between pre/post-dialysis BUN values were 94.6 ± 31.1 in HRH patients and 86.8 ± 28.5 in the CC group (P = 0.006). The systolic blood pressure difference between the pre/post-dialysis measurements was 22.4 ± 16.5 mmHg in HRH patients and 12.8 ± 19.4 mmHg in CC(P < 0.001). Patients with HRH had significantly higher mean systolic and diastolic blood pressure pre-dialysis values (systolic, P = 0.002; diastolic, P < 0.001). The differences in systolic/diastolic blood pressure between pre/post-dialysis were higher in the HRH group (P < 0.001, P = 0.001, respectively). CONCLUSION: Regulating the frequency and timing of dialysis may provide better management in HRH with high BUN levels and high pre-dialysis blood pressure.

Reduced inhibition in brainstem circuits in classical trigeminal neuralgia.

OBJECTIVE: We hypothesized that prepulse modulation (PPM) would be altered in trigeminal neuralgia (TN) if suprasegmental inhibitory network involvement was present and tested our hypothesis in a group of patients with classical TN. METHODS: The study enrolled nine consecutive patients with classical TN and 14 healthy subjects. Diagnosis and classification followed the International Classification of Headache Disorders-third edition (beta version). The blink reflex (BR) and BR-PPM were recorded. Ipsilateral recordings were made after stimulating each trigeminal nerve in the patient group whereas right-sided recordings were performed after stimulating the right trigeminal nerve in the healthy subjects. A conditioning electrical stimulus was applied to the ipsilateral median nerve at interstimulus intervals (ISIs) of 50 and 100 ms before the test stimulus to the supraorbital nerve. RESULTS: The unconditioned BR recordings were similar in all groups. In the healthy subjects, the prepulse stimulus resulted in a reduced R2 magnitude (p = 0.000, Friedman's test) and longer R2 latency (p = 0.008, Friedman's test) at ISIs of 50 and 100 ms in comparison with unconditioned recordings. The R2 latency differed significantly between the unconditioned recordings and the ISI of 100 ms. In the patients with TN, no significant change was observed on either the symptomatic or asymptomatic sides. CONCLUSIONS: There is a bilateral prepulse inhibition deficit in TN, even on the asymptomatic side. Our findings provide electrophysiological evidence for suprasegmental changes and loss of filtering activity at the brainstem in level TN.

Conversion from clinically isolated syndrome to multiple sclerosis: A large multicentre study.

BACKGROUND AND OBJECTIVE: We explored which clinical and biochemical variables predict conversion from clinically isolated syndrome (CIS) to clinically definite multiple sclerosis (CDMS) in a large international cohort. METHODS: Thirty-three centres provided serum samples from 1047 CIS cases with at least two years' follow-up. Age, sex, clinical presentation, T2-hyperintense lesions, cerebrospinal fluid (CSF) oligoclonal bands (OCBs), CSF IgG index, CSF cell count, serum 25-hydroxyvitamin D3 (25-OH-D), cotinine and IgG titres against Epstein-Barr nuclear antigen 1 (EBNA-1) and cytomegalovirus were tested for association with risk of CDMS. RESULTS: At median follow-up of 4.31 years, 623 CIS cases converted to CDMS. Predictors of conversion in multivariable analyses were OCB (HR = 2.18, 95% CI = 1.71-2.77, p < 0.001), number of T2 lesions (two to nine lesions vs 0/1 lesions: HR = 1.97, 95% CI = 1.52-2.55, p < 0.001; >9 lesions vs 0/1 lesions: HR = 2.74, 95% CI = 2.04-3.68, p < 0.001) and age at CIS (HR per year inversely increase = 0.98, 95% CI = 0.98-0.99, p < 0.001). Lower 25-OH-D levels were associated with CDMS in univariable analysis, but this was attenuated in the multivariable model. OCB positivity was associated with higher EBNA-1 IgG titres. CONCLUSIONS: We validated MRI lesion load, OCB and age at CIS as the strongest independent predictors of conversion to CDMS in this multicentre setting. A role for vitamin D is suggested but requires further investigation.

Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.

BACKGROUND: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). OBJECTIVE: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. METHODS: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. RESULTS: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. CONCLUSION: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.

Clinical and radiological characteristics of tumefactive demyelinating lesions: follow-up study.

BACKGROUND: Demyelinating lesions over 20 mm in size, referred to as tumefactive demyelinating lesions, can be misdiagnosed as being either a tumor or an abscess. Although some radiological characteristics can help make a differential diagnosis easier, a cerebral biopsy may still be necessary. OBJECTIVE: Our objective was to assess the clinical characteristics of tumefactive lesions, with or without a diagnosis of multiple sclerosis (MS), and present follow-up data for 54 patients with tumefactive lesions. METHODS: Demographic, clinical, radiological and laboratory data were gathered and treatment responses were evaluated in a total of 54 patients from five medical centers. RESULT: Twenty-nine patients were diagnosed with tumefactive lesions at the onset, whereas 25 patients were diagnosed with tumefactive lesions after a diagnosis of MS. Median follow-up was 38.12 months. At final examination, 19 of the patients with a tumefactive lesion diagnosis at the onset eventually developed relapsing-remitting MS, while 10 remained with the condition as a clinically isolated syndrome. The tumefactive lesions studied were mostly focal, with closed-ring enhancement. We found that oligoclonal band positivity was less frequent in the patients with tumefactive onset. CONCLUSION: Although our demographic data were similar to formerly collected Turkish MS data, we found that the distribution of the patients' clinical course differed if there was an absence of primary progressive MS and that there was a lower frequency of secondary progressive MS cases in our group of patients. We believe that less frequent oligoclonal band positivity and the difference we witnessed in the clinical course of disease in our study groups suggest that there is a need for further studies to compare all the biological and immunological differences between MS and tumefactive lesion cases, in order to reveal whether there are different pathogenetic mechanisms involved.

Clinical characteristics of pediatric-onset neuro-Behçet disease.

OBJECTIVES: Neurologic involvement in the pediatric population with Behçet disease (BD) is limited to case reports. The aim of this study is to examine the frequency and type of neurologic involvement in pediatric patients with BD. METHODS: Medical records of 728 patients with a diagnosis of neuro-BD (NBD) of 2 large BD cohorts followed in Istanbul University were included in the study. Patients with an onset of both systemic and neurologic symptoms at or before age 16 (pediatric neuro-BD) were identified. Demographic and clinical characteristics of pediatric patients with NBD were compared with adult patients with NBD. RESULTS: There were 26 cases with pediatric BD (3.6%) and 702 (96.4%) adult-onset patients. Gender ratio was equal in the general pediatric BD cohort, whereas male/female ratio was 5.5/1 in pediatric NBD cases. Mean age at BD onset and neurologic involvement onset were 13.0 ± 3.0 and 13.5 ± 2.4, respectively, and in the adult population mean age at onset of BD was 26.7 ± 8.0 and neurologic involvement occurred a mean of 5.3 ± 4.5 years later. Clinical and MRI evaluation revealed that 3 children had CNS parenchymal involvement and 23 had dural venous sinus thrombosis (88.5%). We observed parenchymal involvement in 74.8% of the adults, contrary to the low 17.2% of cases with venous sinus thrombosis. CONCLUSIONS: Pediatric NBD comprises 3.6% of our whole NBD cohort, with a male predominance, mainly in the form of dural venous sinus thrombosis, whereas in the adult NBD population the dominant form of neurologic involvement is parenchymal, suggesting that the pathogenesis of NBD may be different according to the age at disease onset.

Evaluation of parenchymal neuro-behçet disease by using susceptibility-weighted imaging.

BACKGROUND AND PURPOSE: Neurologic involvement in Behçet disease, also known as NBD, is one of the most devastating manifestations of the disease. The precise pathologic mechanism of parenchymal NBD lesions has not been established. We evaluated lesion characteristics and probable venous hemorrhage in parenchymal NBD by using SWI, and we compared the imaging results with conventional MR imaging sequences. MATERIALS AND METHODS: We performed cranial MR imaging by using a 1.5T scanner in 23 patients with a definitive diagnosis of parenchymal NBD. We compared the proportion of lesion detection and the performance of hemorrhagic detection with the T2 FSE, T2*GE, and SWI magnitude, and SWI mIP by using the χ(2) test. RESULTS: The proportion of lesion detection with both SWI magnitude and SWI MinMIP was significantly larger than that with T2*GE. The proportions of lesion detection among all other pairs of methods were not significantly different according to the corresponding P value (χ(2) = 17.4929, df = 3, P = .0006). Proportions of hypointense hemorrhagic lesions with T2 FSE and T2*GE were not significantly different, and likewise for the proportions of hypointense hemorrhagic lesions with SWI magnitude and SWI mIP. In contrast, the proportions of hypointense hemorrhagic lesions with SWI magnitude and SWI mIP were significantly larger than that with T2 FSE and T2*GE (χ(2) = 108.5396, df = 3, P < .0001). CONCLUSIONS: Most of the lesions in parenchymal NBD were found to be hemorrhagic with SWI, supporting the proposed venous theory in pathology. In addition, compared with T2 FSE and T2*GE sequences, SWI was more successful in the determination of widespread involvement of the disease, particularly in nonchronic cases.

Multiple sclerosis risk in radiologically uncovered asymptomatic possible inflammatory-demyelinating disease.

BACKGROUND: Natural history of patients with incidentally discovered lesions that fulfill magnetic resonance imaging (MRI) criteria for multiple sclerosis (MS) in the absence of objective clinical symptoms suggestive of central nervous system (CNS) inflammatory-demyelinating disease is not well defined. OBJECTIVE: We evaluated the risk of developing symptomatic MS in patients with radiologically uncovered asymptomatic possible inflammatory-demyelinating disease (RAPIDD). METHODS: We identified and longitudinally followed a cohort of 22 patients from two tertiary care MS centers: Istanbul University, Cerrahpasa School of Medicine, Istanbul, Turkey, and Mayo Clinic, Rochester, Minnesota, after an initial MRI study fulfilling the Barkhof-Tintore MRI criteria completed for other reasons unrelated to MS. RESULTS: Eight of 22 patients developed an objective clinical symptom consistent with a CNS inflammatory-demyelinating syndrome and fulfilled dissemination in space and time criteria for definite MS. Median age at the time of diagnosis of MS was 44.8 years (range 28.3-71.4 years). Time taken for the development of definite MS was studied by survival analysis. Cumulative event rates were; 12 months: 9%, 24 months: 15%, 36 months: 30.4%, and 60 months: 44.6%. Six of 22 patients were followed beyond 60 months. Two of these six patients developed MS later (at 66 and 112 months, respectively). Three patients remained asymptomatic despite follow-up of 10 years. PATIENTS: with RAPIDD develop MS at a similar rate to treated patients (and less frequently than placebo groups) with clinically isolated syndromes from prior randomized controlled studies. Some patients with RAPIDD continue to have radiological evolution of subclinical disease without MS symptoms despite long follow-up periods.

A comparative ID migraine screener study in ophthalmology, ENT and neurology out-patient clinics.

Migraine is more likely to be misdiagnosed in patients with comorbid diseases. Not only primary care physicians, but also specialists might misdiagnose it due to the lack of diagnostic criteria awareness. The ID migraine test is a reliable screening instrument that may facilitate and accelerate migraine recognition. This study aimed to compare the prevalence and characteristics of migraine in a large sample of patients admitted to clinics of ophthalmology (OC), ear, nose and throat diseases (ENTC) and neurology (NC), as well as to validate the use of the ID migraine test in OC and ENTC settings. This was a multicentre (11 cites) study of out-patients admitting either to NC, ENTC or OC of the study sites during five consecutive working days within 1 week. From each of the clinics, 100 patients were planned to be recruited. All recruited patients were interviewed and those having a headache complaint received an ID migraine test and were examined for headache diagnosis by a neurologist, blinded to the ID migraine test result. A total of 2625 subjects were recruited. Only 1.3% of OC patients and 5.4% of ENTC patients have been admitted with a primary complaint of headache, whereas the percentage of NC patients suffering from headache was 37.6%. Whereas 138 patients (19.3%) in OC, 154 (17.3%) in ENTC and 347 (34%) in NC were found to be ID migraine test positive, 149 patients (20.8%) in OC, 142 (16%) in ENTC and 338 (33.1%) in NC were diagnosed with migraine. The sensitivity, specificity, and positive and negative predictive ratios of the ID migraine test were found to be similar in all clinics. An important fraction of the patients admitted to NC, as well as to OC and ENTC, for headache and/or other complaints were found out to have migraine by means of a simple screening test. This study validated the ID migraine test as a sensitive and specific tool in OC and ENTC, encouraging its use as a screening instrument.

VKORC1 and CYP2C9 polymorphisms are associated with warfarin dose requirements in Turkish patients.

OBJECTIVES: The objective of this study was to determine the quantitative influence of vitamin K epoxide reductase complex subunit 1 (VKORC1) and cytochrome P450 2C9 (CYP 2C9) polymorphisms on warfarin dose requirements in Turkish patients. METHODS: A total of 205 patients taking warfarin for >2 months were enrolled in the study. Deoxyribonucleic acid (DNA) samples from these patients were genotyped for polymorphisms in VKORC1 and CYP2C9 genes. A linear regression analysis was used to determine the independent effects of genetic and non-genetic factors on mean warfarin dose requirements. RESULTS: The VKORC1 promoter polymorphism (3673 G>A) was associated with differences in weekly mean varfarin dose: for GG genotype the dose was 43.18 mg/week, for GA genotype 33.78 mg/week and for AA genoype 25.83 mg/week (P < 0.0001). Patients who carried VKORC1 and CYP2C9 variants needed a 40% lower mean weekly warfarin dose compared to wild types. Variables associated with lower warfarin dose requirements were VKORC1 3673 AA or GA genotype (both P < 0.0001), one or two CYP2C9 variant alleles (both P < 0.0001), increasing age (P < 0.0001) and non-indication of venous thromboembolism for warfarin therapy (P = 0.002). CONCLUSION: Polymorphisms in VKORC1 and CYP2C9 genes were important determinants of warfarin dose requirements in Turkish patients.

Validity of the ID-Migraine screener in the workplace.

OBJECTIVE: The impact of migraine on physical, social, and emotional performance is considerable, yet it remains an underdiagnosed disorder. ID-Migraine is a validated migraine-screening tool developed to facilitate diagnosis. This study evaluated the validity and use of the Turkish version of the ID-Migraine screener (ID-Ms) in the workplace, and measured the impact of headache on disability, productivity, and quality of life among the workforce. METHODS: A total of 465 employees from four companies were interviewed for screening with the ID-Ms. Subjects were included in the study if they reported two or more headaches in the past 3 months and gave a positive answer to one of the two ID-Ms prescreening questions. Eligible subjects completed the ID-Ms, the Migraine Disability Assessment Questionnaire, and the Medical Outcomes Study 36-Item Short Form Health Survey. Subjects were then evaluated for confirmation of their diagnosis according to the International Classification of Headache Disorders, 2nd edition (ICHD-2) criteria. RESULTS: A total of 227 subjects (mean age 31.9 +/- 5.9 years; 65.6% women) completed the study. Migraine was diagnosed in 106 of the 227 subjects (46.7%) according to the ID-Ms and in 117 of the 227 subjects (51.5%) according to ICHD-2 criteria. The sensitivity of the ID-Ms was 70.9%, specificity was 79.1% and Cohen kappa value was 0.50. Workdays lost over the previous 3 months due to headache amounted to 8.7 +/- 9.5 days for migraine-positive and 4.9 +/- 6.6 days for migraine-negative subjects. CONCLUSION: The Turkish version of the ID-Migraine screener is a valid tool for identifying subjects with migraine in the workplace.

Investigation of neuromuscular transmission in some rare types of migraine.

The aim of this study was to delineate any dysfunction of neuromuscular transmission (NMT) by single-fibre electromyography (SFEMG) in some rare types of migraine. Recent studies have shown subclinical dysfunction of NMT in migraine with aura and cluster headache by using SFEMG, whereas another recent study has shown NMT to be normal in familial hemiplegic migraine (FHM) with CACNA1A mutations. Thirty patients with rare primary headache syndromes [18 with sporadic hemiplegic migraine (SHM), six with FHM and six with basilar-type migraine (BM)] and 15 healthy control subjects without any headache complaints underwent nerve conduction studies, EMG and SFEMG during voluntary contraction of the extensor digitorum communis muscle. Ten to 20 different potential pairs were recorded and individual jitter values calculated. The results obtained from patient groups were compared with those from the normal subjects. Of 600 individual jitter values of the patients, 27 (4.5%) were abnormally high, whereas only 3/205 (1.5%) jitter values from normal subjects were abnormal. Abnormal NMT was found in 4/30 (13.3%) patients (three SHM and one BM), but in none of the control subjects. Only in SHM patients was the number of individual abnormal jitter values slightly but significantly different from normal controls. The present study demonstrates that subclinical NMT abnormality is slightly present in only SHM and BM patients, but not in FHM patients.

Fatigue and sleep disturbance in multiple sclerosis.

Considering the association of sleep disturbance and fatigue in multiple sclerosis (MS), we investigated the presence of sleep disturbances that may be related to fatigue by using objective and subjective measures. We included 27 MS patients with fatigue, 10 MS patients without fatigue and 13 controls. The Pittsburgh sleep quality index score showed significant differences between patient groups and controls. Beck depression inventory scores were significantly higher in fatigued than non-fatigued patients. Comparison of patient groups and controls revealed significant differences for time in bed, sleep efficiency index, sleep continuity index, wake time after sleep onset, total arousal index and periodic limb movement arousal index. Our study confirms that MS causes sleep fragmentation in terms of both macro and microstructure. Fatigue in MS could be partially explained by disruption of sleep microstructure, poor subjective sleep quality and depression.

The R110C mutation in Notch3 causes variable clinical features in two Turkish families with CADASIL syndrome.

Mutations in Notch3 gene are responsible for the cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). It is a late onset neurological disorder recognized by recurrent strokes and dementia. We describe here the clinical and molecular findings of three unrelated Turkish families with CADASIL syndrome. Two of the families were identified to have the same mutation, p.R110C (c.C328T), located in exon 3 of the Notch3 gene. Interestingly, the phenotypic expression of the disease in these two families was markedly different in severity and age of onset implicating additional genetic and/or non-genetic modulating factors involved in the pathogenesis. In addition, we identified the novel p.C201R (c.T601C) mutation in exon 4 of the Notch3 gene in a proband of the third family with two consecutive stroke-like episodes and typical MRI findings. Mutations described here cause an odd number of cysteines in the N-terminal of the EGF domain of Notch3 protein, which seems to have an important functional effect in the pathophysiology of CADASIL. The phenotypic variability in families carrying the same molecular defect as presented here makes the prediction of prognosis inconceivable. Although DNA analysis is effective and valuable in diagnosing approximately 90% of the CADASIL patients, lack of genotype-phenotype correlation and prognostic parameters makes the presymptomatic genetic counseling very difficult.

Effects of multiple sclerosis on respiratory functions.

OBJECTIVES: To measure respiratory functions of ambulatory patients with multiple sclerosis and compare the results with expected values from healthy general population data. Further, to study the correlation of respiratory function impairment with the multiple sclerosis-induced disability level. SUBJECTS: Thirty-eight patients with definite diagnosis of multiple sclerosis from the multiple sclerosis outpatient clinic. METHODS: The Expanded Disability Status Scale (EDSS) scores (mean 4.34+/-1.39) were evaluated to measure disability level. Respiratory functions tested in the laboratory included spirometric (FVC, FEV1, FEV1/FVC), maximal inspiratory (MIP) and expiratory (MEP) mouth pressure measurements. Patients were clinically assessed using a pulmonary dysfunction index (PDI) and dyspnoea index; various breathing features were also recorded. RESULTS: With respect to expected values from healthy general population, important decreases were found in mouth pressures with MIP (77%+/-23%, p approximately equal to 0) and MEP (60%+/-13%, p approximately equal to 0) while spirometric measures were significantly but less affected (FVC 94%+/-12%, p < 0.01, FEV1 91%+/-16%, p < 0.001). FEV1/FVC ratios were normal. Notable increases in PDI (5.58+/-0.68, p approximately equal to 0) and dyspnoea index (0.32+/-0.47, p approximately equal to 0) were observed. Significant relationships (p < 0.01) between respiratory function impairment and the multiple sclerosis disability level could be detected for FVC, MEP and PDI. CONCLUSIONS: Multiple sclerosis clearly impairs respiratory functions; maximal mouth pressures are more severely reduced. This impairment increases with multiple sclerosis-induced disability level but is found to be independent from duration of disease.

Cerebral venous thrombosis is associated with major vessel disease in Behçet's syndrome.

OBJECTIVE: To examine the association between the type of central nervous system (CNS) involvement (parenchymal disease and cerebral venous thrombosis (CVT)) and extra-cranial large vessel events, mainly venous thrombosis, in Behcet's syndrome (BS). METHODS: Conventional venous angiograms, Doppler ultrasonography, computed tomography, and MR angiography were used to study 88 patients with BS, with (n = 88) and without (n = 80) CNS disease for the presence of major vessel disease. RESULTS: Major vessel involvement among the male patients with and without CNS disease (21/73 (29%) v 18/80 (23%), respectively) showed no significant differences (p = 0.374). When patients with CNS disease were stratified according to the type of CNS involvement, 7/11 (64%) patients with CVT had major vessel disease compared with 15/77 (19%) patients with parenchymal disease (p = 0.004). The mean (SD) age of onset of CVT (23.1 (8.8) years) among the male patients was significantly earlier than among the men with parenchymal disease (32.0 (7.5); p = 0.002). CONCLUSIONS: CVT in BS was strongly associated with peripheral major vessel disease and occurred earlier in the disease course than the parenchymal type of CNS disease. As superficial thrombophlebitis also occurs more frequently in patients with major vessel disease in BS, this may suggest a common pathogenic mechanism.

MRI findings in pediatric neuro-Behçet's disease.

Behçet's disease (BD), a systemic vasculitis of unknown cause, affects many organs and systems. Neurological involvement is seen in 5-15% of the patients, and the two major forms of neurological disease seen in BD are central nervous system (CNS) parenchymal involvement and cerebral venous sinus thrombosis. We report a 14-year-old boy with BD who had neuro-parenchymal involvement. The diagnosis of the systemic disease was not made until the onset of the neurological manifestations, which led to an MRI study that revealed findings suggestive of CNS involvement of BD. We therefore emphasize the importance of the localization and appearance of other characteristics of the lesions on MRI in the differential diagnosis of parenchymal neuro-Behçet syndrome.

Two cases of neuro-Behcet's disease mimicking cerebral tumor.

Two cases of neuro-Behcet's disease with isolated, solitary fronto-temporal and mesencephalic lesions respectively are reported. The cases were misdiagnosed as cerebral tumor. The postoperative outcome of the first patient was not satisfactory as he developed hemispheric edema. The second patient, with mesencephalic lesion, was treated only with corticosteroids, and the patient improved significantly. The lesion in this case resolved completely at six-month follow-up. We conclude that distinguishing the isolated solitary cerebral lesion of the Behcet's disease from a tumor may prevent surgical intervention.

Behçet's disease: diagnostic and prognostic aspects of neurological involvement.

This study was conducted to describe clinical and prognostic aspects of neurological involvement in Behçet's disease (BD). Patients referred for neurological evaluation fulfilled the criteria of the International Study Group for Behçet's Disease. We analyzed disability and survival by the Kaplan-Meier method, using Kurtzke's Extended Disability Status Scale (modified for BD) and the prognostic effect of demographic and clinical factors by Cox regression analysis. We studied 164 patients; of the 107 diagnostic neuroimaging studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thrombosis (VST) and the others were normal. CSF studies were performed in 47 patients; all with inflammatory CSF findings (n = 18) had parenchymal involvement. An isolated increase in pressure was compatible with either VST or normal imaging. The final diagnoses were VST (12.2%), neuro-Behçet syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet syndrome (0.6%), and indefinite (11%). VST and NBS were never diagnosed together. Ten years from onset of BD 45.1% (all NBS) reached a disability level of EDSS 6 or higher, and 95.7 +/- 2.1% of the patients were still alive. Having accompanying cerebellar symptoms at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurological diagnoses in BD are NBS and VST. These are distinct in clinical, radiological, and prognostic aspects, hence suggesting a difference in pathogenesis.