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Progressive multifocal leukoencephalopathy (PML) is a severe, demyelinating disease of the central nervous system caused by JC virus infection. The disease can be seen in sarcoidosis patients without additional risk factors. Here, we present an individual with PML secondary to sarcoidosis treated with 8 doses of pembrolizumab, a Programmed Cell-Death-1 (PD-1) Immune Checkpoint Inhibitor who showed significant improvement. This report illustrates the objective clinical and radiological improvement in a patient with PML due to sarcoidosis, and suggests further study of immune checkpoint inhibitors as a potential treatment for sarcoidosis patients with PML.
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BACKGROUND: Previous reports indicate that juvenile Behçet's disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. OBJECTIVES: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. METHODS: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet's Disease at or before 16 years of age. RESULTS: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976-1997 and 1998-2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. CONCLUSIONS: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.
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Síndrome de Behçet , Humanos , Adulto , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/epidemiologia , Síndrome de Behçet/complicações , Estudos Retrospectivos , Seguimentos , PrevalênciaRESUMO
BACKGROUND: Backward walking training (BWT) can have a positive effect on balance, gait, and functional mobility in neurological diseases; however, the effectiveness of BWT has not been examined in multiple sclerosis (MS). Therefore, the study aimed to investigate the effects of BWT on balance, gait, and functional mobility in people with MS (PwMS). METHOD: Nineteen PwMS were randomly allocated to either the experimental group (n=10) and the control group (n=9). The experimental group received BWT in addition to conventional walking training (CWT) while the control group only received CWT. Both groups performed training three times a week for 8 weeks. Participants were assessed with the Berg Balance Scale (BBS), four square step test (FSST), activities-specific balance confidence scale (ABC), timed 25-foot walk test (T25FW), dynamic gait index (DGI), 3-meter backward walk test (3MBWT), Multiple Sclerosis Walking Scale-12 (MSWS-12), and timed up and go test (TUG) before and after training. RESULTS: After training, both groups showed significant improvements on the T25FW, and TUG (p<0.05) while only the experimental group showed significant improvements on the BBS, FSST, ABC, DGI, 3MBWT, and MSWS-12 (p<0.05). The experimental group significantly improved more than the control group in all outcomes (p<0.05) except for the T25FW (p=0.202). CONCLUSION: BWT in addition to CWT is an effective way to improve balance, gait, and functional mobility for PwMS. These results suggest that BWT may be a potentially useful treatment approach when added to CWT in the rehabilitation of MS.
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Esclerose Múltipla , Equilíbrio Postural , Humanos , Estudos de Tempo e Movimento , Marcha , CaminhadaRESUMO
In this study, the researchers aimed to investigate the effects of pelvic floor muscle training (PFMT) applied with telerehabilitation on urinary symptoms, quality of life, and subjective perception of improvement and satisfaction in multiple sclerosis (MS) patients having lower urinary tract symptoms. Patients were randomly divided into PFMT (n:21) and control (n:21) groups. The PFMT group received PFMT via telerehabilitation for 8 weeks and lifestyle advice, while the control group received only lifestyle advice. Although lifestyle advice alone was not effective, PFMT applied with telerehabilitation was an effective method in the management of lower urinary tract symptoms in MS patients. PFMT applied with telerehabilitation can be considered as an alternative method.
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BACKGROUND: The ability to turn while walking is essential for people's activities of daily living. Difficulties in turning while walking are commonly shown in people with multiple sclerosis (PwMS). The figure-of-eight walk test (F8W) is a clinical test assessing walking skill in a curved pathway; however, its reliability and validity have not been systematically examined for PwMS. PURPOSES: The study is aimed to investigate: (1) the test-retest reliability of the F8W in PwMS; (2) the standard error of measurement and minimum detectable change in the F8W times; (3) the concurrent and known-groups validity of the F8W times; and (4) the cut-off times that best discriminate fallers from non-fallers with MS. METHOD: This cross-sectional study included 41 PwMS and 33 healthy people. The F8W was performed along with the Timed Up and Go Test (TUG), Berg Balance Scale (BBS), Activities-specific Balance Confidence Scale (ABC), and Expanded Disability Status Scale (EDSS). To determine the test-retest reliability, the F8W was conducted twice, 7-10 days apart. The reliability was assessed using the intraclass correlation coefficient (ICC), Bland-Altman plots, standard error of measurement (SEM), and minimal detectable change (MDC). To examine validity, the correlations between the F8W and the TUG, BBS, ABC, and EDSS were assessed using correlation coefficients, and the completion times of the F8W were compared between PwMS and healthy people, and between fallers and non-fallers with MS. The receiver operating characteristic curve was constructed to determine the optimal F8W cut-off time discriminating fallers from non-fallers with MS. RESULTS: The F8W had excellent test-retest reliability with an ICC of 0.916. Bland-Altman plots showed high agreement between sessions. The SEM and MDC were found to be 0.45 and 1.25, respectively. The F8W indicated a moderate to strong correlation with other outcome measures (correlation coefficients ranged from -0.596 to 0.839, p<0.05). On the F8W, PwMS had a longer time than healthy people while fallers had a longer time than non-fallers with MS (p<0.001, and p<0.001, respectively). The cut-off time of 8.52 s best discriminated the fallers from non-fallers with MS. CONCLUSIONS: The F8W is a reliable and clinically available measurement tool for walking skill in PwMS.
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Esclerose Múltipla , Humanos , Teste de Caminhada , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Equilíbrio Postural , Reprodutibilidade dos Testes , Estudos Transversais , Atividades Cotidianas , Estudos de Tempo e Movimento , CaminhadaRESUMO
To investigate: (a) the interrater and test-retest reliability of the 3-m backward walk test (3MBW) in ambulant people with multiple sclerosis (PwMS); (b) minimal detectable change (MDC); (c) concurrent and known-groups validity; and (d) the cutoff time to best discriminate fallers from nonfallers with multiple sclerosis (MS). Forty-nine PwMS and 36 healthy people were included in this cross-sectional study. The 3MBW was administered with the timed up and go test, Berg Balance Scale, four square step test, Falls Efficacy Scale-International, and Expanded Disability Status Scale. The 3MBW was simultaneously performed by two independent raters to examine the interrater reliability while was repeated after 7-10 days to examine the test-retest reliability. The 3MBW showed good interrater reliability [intraclass correlation coefficient (ICC) = 0.987-0.989] and excellent test-retest reliability (ICC = 0.854-0.889). The MDC was found to be 1.69 s. The 3MBW had moderate-to-strong correlations with the other measures. For the 3MBW, PwMS had worse performance than healthy people ( P < 0.001), whereas fallers with MS had worse performance than nonfallers with MS ( P < 0.001). The 3MBW time of 7.86 s was determined to best discriminate fallers from nonfallers with MS. The 3MBW is a reliable, simple, and easy-to-administer tool for assessing backward walking among ambulant PwMS.
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Esclerose Múltipla , Estudos Transversais , Humanos , Equilíbrio Postural , Reprodutibilidade dos Testes , Estudos de Tempo e Movimento , Teste de Caminhada , CaminhadaRESUMO
Background: Behçet's disease (BD) is a multisystem vasculitis, which can involve the central nervous system, the gastrointestinal tract, the blood vessels, the joints, and the lungs. Neurological involvement is about 5%, but the headache is common in these patients. Objective: This study aimed to investigate whether differences exist in the onset and types of primary headaches between patients with BD without neurological involvement (BWoNI) and patients with neurological involvement, that is, neuro-Behçet's disease (NBD). Materials and Methods: A total of 136 consecutive BD patients (98 BWoNI and 38 NBD) were evaluated and compared. Results: Primary headaches beginning before and after the onset of BD were found in 27.6 and 48.1% of BWoNI patients, respectively, and in 10.5 and 34.2% of NBD patients, respectively. The start of primary headaches was more frequent after then before the onset of BD in each group and overall. The primary headache types were similar in both BWoNI and NBD patients. Conclusions: The primary headache starts more frequently after then before the onset of BD, and characteristics of primary headaches were similar in both BWoNI and NBD patients.
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Síndrome de Behçet , Síndrome de Behçet/complicações , Cefaleia/etiologia , HumanosRESUMO
BACKGROUND: Face and facial expression recognition abilities have been frequently evaluated in the assessment of social cognition disorders in patients with MS. Investigation of the effect of new difficulties emerging in the field of face recognition with the widespread use of masks during the ongoing COVID-19 pandemic on patients with MS may make new contributions to the literature. MATERIAL AND METHODS: The study included 44 patients with relapsing-remitting MS (RRMSp) and 51 controls who were matched to the case group in terms of age and education level. The Benton face recognition test-short form (BFRT-sf), Beck Depression Inventory, a close-ended 13-item survey on face recognition difficulties due to mask use during the pandemic was administered to all groups. RESULTS: In the RRMSp, the mean disease duration was 8.2 ± 5.6, the mean EDSS score was 1.2 ± 1.0, and the mean MOCA test score was 27.23 ± 2.08. The mean BFRTsf was 19.9 ± 2.4 in the RRMSp and 21.6 ± 1.8 in the healthy controls.Twenty-five percent of RRMSp and 4% of the healthy controls required people to remove their masks to be able to recognize their faces. Improvement in face recognition difficulty over time was reported as 80% in the healthy controls and 34% in the RRMSp. CONCLUSION: RRMSp had worse performance in masked face recognition and required removal of the facial masks more often than healthy controls to recognize the faces. RRMS patients did not show as much improvement in recognizing masked faces over time according to the onset of the pandemic as healthy controls.
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COVID-19 , Reconhecimento Facial , Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Humanos , Esclerose Múltipla Recidivante-Remitente/complicações , Esclerose Múltipla Recidivante-Remitente/diagnóstico , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Pandemias , SARS-CoV-2RESUMO
OBJECTIVE: To describe the clinical characteristics of neuro-Behçet's syndrome (NBS) and to define the factors associated with relapses and poor outcome. METHODS: Among 2118 patients with Behçet's syndrome who fulfilled the international study group criteria, 208 (9.8%) patients had NBS. Retrospective data of 125 NBS patients (55.5% male; mean age 37.2 ± 11.8 years) were analysed. We divided patients into two subgroups, either parenchymal (p-NBS) or non-parenchymal (np-NBS), according to international consensus recommendations for NBS. We assessed the predictor factors associated with relapse and poor outcome-which was defined as a modified Rankin score (mRS) ≥ 3 at last follow-up and/or death-using Cox and logistic regression analyses, respectively. RESULTS: In total, 79 (63.2%) patients presented with p-NBS and 46 (36.8%) presented with np-NBS. Ocular involvement was more common in p-NBS than np-NBS (55.7% vs. 37.0%, p = 0.04), whereas vascular involvement excluding cerebral vein thrombosis was more frequent in patients with np-NBS (19.0% vs. 52.2%, p < 0.001). Forty-two patients (33.6%) experienced at least one relapse. Factors associated with relapse were BS diagnosis at a younger age and cranial nerve dysfunction (HR 0.96 95% CI 0.93-0.99 and 2.36 95% CI 1.23-4.52, respectively). After a median of 68 (Q1-Q3: 25-125) months, 23 patients (18.4%) had a poor outcome. Indicators of a poor outcome were higher initial mRS and the progressive p-NBS type (OR 8.28 95% CI 1.04-66.20 and 33.57 95% CI 5.99-188.21, respectively). CONCLUSION: Our findings indicate that clinical characteristics and prognosis differ between NBS subgroups, of which patients with p-NBS have worse outcomes.
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Síndrome de Behçet , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Estudos RetrospectivosRESUMO
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.
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Síndrome de Behçet/complicações , Desnutrição/etiologia , Estado Nutricional , Adulto , Síndrome de Behçet/epidemiologia , Progressão da Doença , Humanos , Imageamento por Ressonância Magnética , Desnutrição/epidemiologia , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
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.
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Síndrome de Behçet , Qualidade de Vida , Síndrome de Behçet/complicações , Estudos Transversais , HumanosRESUMO
The aim of the study was evaluation of sexual dysfunction in neuro-Behcet's disease (NBD). A total of 25 patients with NBD, 22 patients with Behçet's disease (BD) without neurologic involvement (BDWONI) and 19 persons in control group were prospectively included in the study. We collected all demographic data. A semi-structured interview guided by a Arizona sexual experience scale (ASEX) questionnaire, Beck Depression Inventory and Beck Anxiety Inventory was applied to all of the patients. The sexual dysfunction was significantly higher in patients with NBD (47.9%) compared to patients with BDWONI (35.4%) and control group (16.7%) (p = 0.001). The score of total ASEX, drive, aurosal, vaginal lubrication/penil erection and satisfaction from orgasm were significantly higher in patients with NBD than healthy control group, statistically. Just arousal and lubrication/penile erection scores were significantly higher in NBD group than BDWONI group. Female/Male ratio regarding sexual dysfunction was 43.5/56.5, 29.4/70.6, 62.5/37.5 in NBD, BDWONI and healthy control group, respectively (0 = 0.027). NBD had a negative effect on sexuality in all but especially on arousal and vaginal lubrication/penil erection function based on ASEX questionnaire. Moreover, male sexuality was more affected by BD than female sexuality. Further researches using larger sample sizes are needed to clarify the issues.
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Síndrome de Behçet/complicações , Disfunções Sexuais Fisiológicas/etiologia , Adulto , Nível de Alerta , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Satisfação Pessoal , Escalas de Graduação Psiquiátrica , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: Neuro-Behçet's disease (NBD) is a rare manifestation of Behçet's disease (BD) and may cause severe disability. The aim of this study was to evaluate the treatment response in patients with NBD and to investigate the parameters that may influence the prognosis of the disease in patients with severe to mild-moderate disability. METHODS: The files of 60 patients admitted to our outpatient clinic for NBD between January 2007 and June 2014 were retrospectively reviewed. We compared the BD duration, time to NBD, NBD type and course, clinical findings of BD, functional neurological system involvement, localization of lesions on brain MRI, and all the medications between the severe and mild-moderate disability groups. RESULTS: The mean time to the onset of NBD was significantly longer (17.8±4.6 years) and the mean age was significantly higher (50.25±9.1 years) in patients with severe disability than in those with mild-moderate disability (7.5±8.0 years and 37.5±10.9 years; p=0.01 and p=0.03, respectively). Moreover, hemispheric involvement was significantly associated with severe disability (p=0.006). No difference was found with regard toother investigated parameters between the groups. CONCLUSION: We believe that severe neurological disability may be associated with older age at the onset of NBD or longer time to NBD and hemispheric lesions on brain MRI. However, our results should be cautiously evaluated with further research.
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BACKGROUND: Cognitive impairment (CI) is a common problem in multiple sclerosis (MS), may occur either in early or late phase of the disease, and impairs quality of life. OBJECTIVES: This study aimed to determine the prevalence of CI and related risk factors in relapsing-remitting MS (RRMS) patients in Turkey. METHODS: The present cross-sectional, multi-center, and nationally representative study included RRMS patients. Sociodemographic characteristics, cognitive functions and additional outcomes were compared between patients with and without CI. RESULTS: The analyses included 487 RRMS patients. According to the BRB-N battery results, CI prevalence was 53.7%. There was a negative significant correlation of BRB-N subtests with age, disease duration, and EDSS and MSNQ-patient rated scores. On the logistic regression analysis, increased age, living in village/rural area, high income level, and high EDSS score were significant increasing risk factors in the development of CI. CONCLUSIONS: This is the first national cognitive data obtained from MS in Turkey, which is a country between Europe and Asia and thus has characteristics of both continents. The similarity of the results of the present study obtained from Turkey to the Western-based data indicates that CI is universal in MS and the main factors affecting CI have not changed.
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Disfunção Cognitiva/epidemiologia , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Adulto , Fatores Etários , Disfunção Cognitiva/etiologia , Estudos Transversais , Avaliação da Deficiência , Escolaridade , Feminino , Humanos , Masculino , Esclerose Múltipla Recidivante-Remitente/complicações , Esclerose Múltipla Recidivante-Remitente/psicologia , Testes Neuropsicológicos , Prevalência , Fatores de Risco , Fatores de Tempo , Turquia/epidemiologiaRESUMO
This study was aimed at the development of an immunosensor for the simultaneous quantification of Myelin Basic Protein (MBP) and Tau proteins in cerebrospinal fluid (CSF) and serum, obtained from Multiple Sclerosis (MS) patients. The newly developed GO/pPG/anti-MBP/anti-Tau nanoimmunosensor has been established by immobilization of MBP and Tau antibodies. The newly developed nanoimmunosensor was tested, optimized and characterized using differential pulse voltammetry (DPV) and electrochemical impedance spectroscopy (EIS). The developed nanoimmunosensor was seen to have detection limits of 0.30nM for MBP and 0.15nM for Tau proteins which were sufficient for the levels to be analysed in neuro-clinic. The clinical study performed using CSF and serum of MS patients showed that the designed nanoimmunosensor was capable of detecting the proteins properly, that were essentially proven by ELISA.
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Técnicas Biossensoriais/métodos , Esclerose Múltipla/sangue , Esclerose Múltipla/líquido cefalorraquidiano , Proteína Básica da Mielina/sangue , Proteína Básica da Mielina/líquido cefalorraquidiano , Proteínas tau/sangue , Proteínas tau/líquido cefalorraquidiano , Anticorpos Imobilizados/química , Compostos de Cádmio/química , Dendrímeros/química , Espectroscopia Dielétrica/métodos , Técnicas Eletroquímicas/métodos , Grafite/química , Humanos , Imunoensaio/métodos , Chumbo/química , Limite de Detecção , Nanopartículas/química , Nanopartículas/ultraestrutura , Óxidos/química , Sulfetos/químicaRESUMO
OBJECTIVE: To determine the frequency and severity as well as the diagnosis and treatment of overactive bladder problems in patients with multiple sclerosis (MS) followed up at five centers in Turkey. DESIGN: Survey study. SETTING: Outpatient tertiary clinics of physical medicine and rehabilitation and neurology. PARTICIPANTS: Consecutive MS patients scheduled for outpatient follow-up (n = 309). INTERVENTION: MS patients were asked to complete a questionnaire regarding the frequency and severity, as well as the diagnosis and treatment of their overactive bladder problems. RESULTS: The mean age ± SD was 39.3 ± 10.6 years. Urinary urgency was the most common urinary symptom (62%), followed by frequency (50.4%), urge incontinence (44.7%) and nocturia (33%). Residual urine volume was measured using a portable ultrasound instrument in 13.3% of the patients and by catheterization in 16.2% of them. Urodynamic investigations and urinary tract ultrasound were performed on 26.5% and 35.3% of the patients, respectively. Anticholinergic medications were prescribed for 27.5% of the patients. Intermittent catheterization and indwelling catheterization were used on 8.1% and 1.9% of the patients, respectively. The overactive bladder symptom score (OABSS) was significantly higher in patients who had had residual urine measurement (P < 0.001), upper urinary tract assessment by ultrasound (P < 0.001), urodynamic assessment (P < 0.001), admitted to a doctor for urinary symptoms (P < 0.001), and current or past catheter use (P = 0.002). CONCLUSION: Urgency was the most common urinary symptom followed by frequency, urge incontinence and nocturia in MS patients. The patients with lower OABSS had detailed urological assessments less frequently than the patients with higher OABSS.
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Esclerose Múltipla/complicações , Bexiga Urinária Hiperativa/diagnóstico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/epidemiologia , Bexiga Urinária Hiperativa/epidemiologia , Bexiga Urinária Hiperativa/etiologia , Bexiga Urinária Hiperativa/terapia , Cateterismo Urinário/métodos , UrodinâmicaRESUMO
OBJECTIVES: This study aims to report the outcomes of patients with Behçet's disease (BD) with cerebral venous thrombosis (CVT) due to BD compared to patients with CVT due to other causes and to discuss the treatment options. PATIENTS AND METHODS: Files of 47 patients admitted to our outpatient clinic for CVT between January 2007 and November 2014 were retrospectively reviewed. Patients were divided into two groups; group 1 included 21 CVT patients with BD (9 males, 12 females; mean age 47±12 years; range 27 to 69 years) and group 2 included 26 CVT patients without BD (11 males, 15 females; mean age 45±16 years; range 25 to 79 years). We collected data for diagnosis for BD and CVT, duration of all medications, functional system involvement, baseline Expanded Disability Status Scale scores, modified Rankin Scale scores at follow-up, and localizations of lesions in brain magnetic resonance imaging and magnetic resonance venography. RESULTS: Mean follow-up duration was four years in group 1 and two years in group 2. There was no significant difference between the groups regarding age, sex, neurological symptoms/findings and baseline Expanded Disability Status Scale scores. Localizations of occluded sinus in group 1 and group 2 were similar. For acute CVT, patients were treated with intravenous high dose prednisolone in group 1 and with anticoagulant in group 2. Follow-up modified Rankin Scale score was 0 in both groups. CONCLUSION: Our study revealed similar clinical and radiological findings in CVT patients with or without BD. Although medications used for treatment were different between the groups, treatment responses were good in both groups. Treatment with prednisolone may be sufficient and anticoagulation therapy may not be necessary for acute CVT attacks in BD.
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Bilateral optic neuritis has been reported very rarely as a manifestation of neuro-Behçet's disease. We present a 50 year old woman who had 20-year history of Behçet's disease presented with acutely blurred vision associated with orbital pain in both eyes. Visual acuity was 0.4 in the right eye and light perception in the left eye; afferent pupillary defect was detected in the left eye. Bilateral swelling of the optic disk was found. The cerebrospinal fluid sample tests were within normal limits. Brain magnetic resonance imaging, magnetic resonance venography and fundus fluorescein angiography were normal. She was diagnosed with bilateral optic neuritis and treated with intravenous methyl prednisolone for 10 days. As there was no response to the treatment, therapeutic plasma exchange was started and the patient's visual acuities improved moderately. We suggest that when high dose steroid is failed to treat ON in BD, treatment with TPE may be considered.
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BACKGROUND: Neuromyelitis optica (NMO) is an immune-mediated, chronic relapsing, inflammatory disease characterized by severe attacks of optic neuritis and myelitis. OBJECTIVE: To determine the demographic, clinical, and laboratory features; antibody status; and treatment modalities of patients with NMO and neuromyelitis optica spectrum disorders in a Turkish cohort from 11 centers. METHODS: A total of 182 patients were included in this study. Data on age at disease onset, sex, type of attacks, clinical presentation, analysis of cerebrospinal fluid, serum antiaquaporin-4 antibody status, annual progression index, and medical and family histories were collected. RESULTS: Mean age was 38.43±12.40 years (range, 13 to 75 y), and mean age at disease onset was 31.29±12.40 years (median, 29 y; range, 10 to 74 y). In NMO group, the rate of NMO immunoglobulin (Ig)G positivity was 62.5%. The annual progression index was significantly higher in the longitudinally extending spinal cord lesion. The mean Expanded Disability Status Scale score was higher in the late than early-onset NMO group. CONCLUSION: Our results revealed a lower rate of NMO IgG positivity, more severe disability in patients with NMO/neuromyelitis optica spectrum disorders presenting with either transverse myelitis or late-onset NMO, and no correlation between disability and NMO IgG status.
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Demografia/estatística & dados numéricos , Neuromielite Óptica , Adolescente , Adulto , Idade de Início , Idoso , Anti-Inflamatórios/uso terapêutico , Aquaporina 4/imunologia , Estudos de Coortes , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Imunoglobulina G/sangue , Imageamento por Ressonância Magnética , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/epidemiologia , Neuromielite Óptica/terapia , Turquia/epidemiologia , Adulto JovemRESUMO
BACKGROUND/AIM: There have been conflicting results in the literature regarding the relationship between functional system involvement, the expanded disability status scale (EDSS), and the presence of restless legs syndrome (RLS) in patients with multiple sclerosis (MS). MATERIALS AND METHODS: Ninety-one patients with MS and 40 patients in a control group (headache, essential tremor, and benign positional paroxysmal vertigo) were studied. The patients underwent a complete neurological examination and Kurtzke functional system scores were calculated. In order to assess the temporal relation between the onset of RLS and MS, a semistructured interview guided by a questionnaire about RLS was applied to all of the patients. RESULTS: Sixteen (17.6%) of the patients with MS and 1 (2.5%) patient in the control group had RLS. The prevalence of RLS was higher in patients with MS, compared to the control group (P = 0.018). Among the patients with MS, none of them suffered from RLS before the onset of MS, whereas sixteen patients (16%) suffered RLS after the onset of MS. There was no significant relationship between functional system involvement and the presence of RLS. CONCLUSION: The prevalence of RLS was higher in MS patients than it was in the control group. No association was found between RLS and functional system involvement in MS patients.