Background:Optic neuritis, myelitis, and neuromyelitis optica spectrum disorder (NMOSD) have been associated with antibodies against myelin oligodendrocyte glycoprotein-immunoglobulin G (anti-MOG-IgG). Furthermore, patients with radiological and demographic features atypical for multiple sclerosis (MS) with optic neuritis and myelitis also demonstrate antibodies against aquaporin-4 and anti-MOG-IgG. However, data on the diagnosis, treatment, follow-up, and prognosis in patients with anti-MOG-IgG are limited. Aims:To evaluate the clinical, radiological, and demographic characteristics of patients with anti-MOG-IgG. Study Design:Multicenter, retrospective, observational study. Methods:Patients with blood samples demonstrating anti-MOG-IgG that had been evaluated at the Neuroimmunology laboratory at Ondokuz Mayıs University’s Faculty of Medicine were included in the study. Results:Of the 104 patients with anti-MOG-IgG, 56.7% were women and 43.3% were men. Approximately 2.4% of the patients were diagnosed with MS, 15.8% with acute disseminated encephalomyelitis (ADEM), 39.4% with NMOSD, 31.3% with isolated optic neuritis, and 11.1% with isolated myelitis. Approximately 53.1% of patients with spinal involvement at clinical onset demonstrated a clinical course of NMOSD. Thereafter, 8.8% of these patients demonstrated a clinical course similar to MS and ADEM, and 28.1% demonstrated a clinical course of isolated myelitis. The response to acute attack treatment was lower and the disability was higher in patients aged > 40 years than patients aged < 40 years at clinical onset. Oligoclonal band was detected in 15.5% of the patients. Conclusion:For patients with NMOSD and without anti-NMO antibodies, the diagnosis is supported by the presence of anti-MOG-IgG. Furthermore, advanced age at clinical onset, Expanded Disability Status Scale (EDSS) score at clinical onset, spinal cord involvement, and number of attacks may be negative prognostic factors in patients with anti-MOG-IgG.
IntroductionIn this study, we aim to evaluate the treatment responses and prognostic characteristics of Myasthenia Gravis (MG) patients followed in a tertiary neuromuscular diseases center in Turkey. MethodsOne hundred seventy four MG patients (between years 2011 and 2022) in Antalya, Turkey were diagnosed, and evaluated on a classification of MG was based on Myasthenia. Gravis Foundation of America (MGFA) clinical classification. Exclusion of other possible diseases in the differential diagnosis and support by beneficial response to treatment with acetylcholinesterase inhibitors were also taken into consideration. ResultsMean age of participants was 54.86 (SD = 14.856; min-max = 22-84). Ninety (51.7%) were female. MG was more common in women under the age of 65 (58%) and in men over the age of 65 (64%). Generalized MG was seen in 75.3% of the patients. Anti-AChR positivities were detected in 52.3%, Anti-MuSK positivity in 4.6%, and seronegativity in 22.4%. Thymoma was detected in nearly 9.8% and thymectomy was performed in 28.7 percent. Most of the patients (57.5%) were using corticosteroids. Azathioprine was used by 39% and mycophenolate mofetil by 10.3% of patients. Mortality was higher and disease was more severe in late-onset (>50 years) MG patients (especially in the COVID-19 pandemic). Eight patients (four women, four men, mean age 75.5 years) died during follow-up. None of them died due to myasthenic worsening, two died due to malignancy and two due to infection. During the COVID pandemic, 16 patients (9.2%) had COVID infection. Four patients died due to COVID-19 infection, these four patients had serious comorbidities, and three of them were elderly (>75 years). ConclusionIn conclusion, MG is more common in women between the ages of 20-40 and in men over the age of 65. The use of corticosteroids was more common under the age of 50, and the use of non-steroidal immunosuppressant agents was more common over the age of 50. Thymectomy is still an important supportive treatment approach in anti-AChR positive and seronegative generalized patients under 50 years of age. IVIG and plasmapheresis are effective treatments during acute exacerbations and bridging periods of treatments. Specific treatments are needed especially for resistant group of patients.
Background Autoimmune encephalitis (AIE) and paraneoplastic syndromes (PNS) are both rare groups of neurological diseases that are difficult to diagnose. Aim We aimed to determine the common and distinct aspects of these two aetiologies of encephalitis as well as the characteristics of our patient group. Methods We respectively analysed the records of the patients including symptoms, demographic features, neurological examination, cranial-magnetic-resonance-imaging (MRI), electroencephalography (EEG) findings, cerebrospinal fluid results (CSF) findings. Autoimmune/paraneoplastic autoantibodies in blood and/or CSF were all documented. Results Forty-six patients fulfilled the diagnostic criteria. Thirty-eight of them were diagnosed with AIE, and 8 of them were diagnosed with PNS. The PNS group had higher nonconvulsive status epilepticus than the AIE (2/8 vs 0/38; p=0.027). PNS patients were diagnosed with a malignancy in their follow-ups more than those in the AIE group [4/38 vs 8/8] (p<0.001). When the symptoms of antibody-positive and negative patients were compared in the AIE group, the rates of consciousness/memory problems (13/15 vs 11/23; p=0.020) and speech impairment (8/15 vs 2/23; p=0.004) were significantly higher in patients without antibodies (n: 15) than in antibody-positive patients (n: 23). In antibody-negative groups, the rates of memory problems in neurological examination (13/15 vs 12/23 p=0.028) and temporal findings on electroencephalography were more prominent than antibody-positive groups (1/23 vs 5/15; p=0.027). The number of patients with cerebellar signs was higher in antibody-positive patients (6/23 vs 0/15; p=0.038). Conclusion Although the positivity of autoantibodies is critical in the diagnosis of AIE and PNS, even minor differences in clinical and laboratory findings of patients are helpful in the diagnosis, especially in the autoantibody-negative patients. Comparing the data with other population studies has shown that several inherited and environmental factors may contribute to the pathophysiology of AIE and PNS, as well as clinical and laboratory differences.
Giriş: İzole pontin enfarktüsü (IPI), diğer beyin bölgelerinin tutulmadığı yaygın bir inme lokalizasyonudur. Metod: 1 Ağustos 2019- 1 Mart 2020 tarihleri arasında XXXX hastanesi nöroloji kliniğinde hospitalize edilen hastalar retrospektif olarak incelendi. Kumral ve ark’nın belirlediği şekilde Pons arterlerinin sulama alanlarına bağlı olarak, pons infarktları anteromediyal, anterolateral, tegmental, bilateral ve tek taraflı çoklu pons infarktları olmak üzere 5 alt tipe ayrıldı. Pons infarktları ayrıca üst, orta veya alt bölüm olacak şekilde segmental lokalizasyonuna ayrıldı. Hastaların tüm demografik özellikleri kayıt edildi. Bulgular: Retrospektif olarak incelenen 84 hasta içinden kriterlere uygun olan toplam 70 hasta çalışmaya dahil edildi. Hastaların yaş ortalaması 63,2 ±1,19 (min:33 max: 88)idi. Ortalama NIHSS’u 3,98± 2,8 ve hastaların %70’i erkekti. Risk faktörleri değerlendirildiğinde ilk sırada %78,6 oranıyla HT vardı. . İnfarkt lokalizasyonuna göre; 38 hasta anteromedial (%54,3), 13 hasta anterolateral (%18,6), 11 hastada tegmental (%15,7), 7 hastada unilateral multiple (%10) ve 1 hastada bilateral (%1,4) gözlendi. İnfarktın En sık segmental yerleşimi 31 hasta ile orta ponsta (%44,3) idi. ( 25 hasta alt pons (%35,9 )ve 14 hasta üst ponsta (%20)). Sonuç: Çalışmamızda; 5 farklı anatomik paterne göre ayrılan izole pons infarktları en sık anteromedial bölgede (%54,3) gözlenmiş ve eşlik eden en yaygın vasküler risk faktörünün HT, HPL ve DM olduğu tespit edilmiştir. Anahtar kelimeler: pons infarktı, pons, stroke, lokalizasyon
Background. ? Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system. We aimed to discuss possible predisposing factors to atherosclerosis such as carotid intima-media thickness (CIMT) and high-sensitivity C-reactive protein (Hs-CRP) levels in MS. Methods. ? Thirty-five ambulatory patients with relapsing-remitting MS (RRMS) (22 females and 13 males) and 34 healthy controls (21 females and 13 males) with similar demographic variables were included. Blood cell counts, cholesterol levels, vitamin D and B12, Hs-CRP levels, body mass index (BMI), history of smoking, and CIMT of both groups, Expanded Disability Status Scale (EDSS) scores, and disease duration of patients were recorded. Patients with a history of other vascular diseases such as hypertension, diabetes mellitus, peripheral artery disease, and acute relapses were excluded. Results. ? Sixty-nine participants were included. The mean age of the study population was 35.8 ? 7.1 years. Right CIMT was significantly greater in the patient population (P < 0.001). Spearman?s correlation coefficient between age and right CIMT was r = 0.41, P = 0.01. When we compared the Hs-CRP with a cutoff value of ? 3, the right, left, and mean CIMT levels were not statistically significant (P = 0.17; P = 0.22; P = 0.15). The mean serum vitamin D levels were higher in the patient group and this was statistically significant (P < 0.001). The statistically significant factors identified with univariate analysis with P < 0.2 were further entered into multivariate modelling. Conclusion. ? CIMT seems to be affected in patients with MS by means of the disease itself and age. Thus, CIMT might reflect the predisposition to subclinical atherosclerosis more than Hs-CRP. Further investigation in a large MS population is still needed. ? 2019 Elsevier Masson SAS. All rights reserved.
Objectives: To use the Montreal Cognitive Assessment (MoCA) test to assess the subclinical cognitive impairment in patients with Primary Sjögren’s Syndrome (PSS) and assess the correlation of MoCA results with magnetic resonance imaging (MRI) findings in these patients. Methods: The MoCA test was prospectively administered to 32 consecutive patients (31 females, 1 male) diagnosed with PSS and 30 healthy controls (29 females, 1 male) at Antalya Education and Research Hospital between June 2014 and October 2015. Twenty PSS patients underwent a brain MRI (T1, T2, and T2- FLAIR-weighted sequences). Results: The mean age was 45.84 (range 24-63) in the PSS group, and the mean duration of disease was 3.5 years (4 months - 18 years). There were 22 patients (68.80%) with 5-8 years of education and 10 patients (31.30%) with >8 years of education. The mean age was 42.8 (28-64) in the control group. There were 20 controls (66.70%) with 5-8 years of education and 10 controls (33.3%) with >8 years of education. The delayed recall rate of the patient group with 5-8 years of education was significantly lower than that of the control group, and the recall rate with multiple choice cues for the same patient group was significantly higher than that of the control group (p<0.05). There was no correlation between the number of lesions and total MoCA score or subgroups. Conclusion: We suggest that the MoCA test is a single-page, easy-to-administer test, can be used to assess cognition in patients with PSS especially in large groups.
Amac: Bu calismanin amaci Saglik Bilimleri Universitesi Antalya Egitim ve Arastirma Hastanesi multipl skleroz (MS) polikliniginin oral tedavi deneyimini paylasmak, hastalar icin uygun tedaviyi secmenin onemini vurgulamaktir. Gerec ve yontem: 2012-2018 yillari arasinda MS polikliniginde en az 6 aydir duzenli takip edilen 550 hastanin dosyasi retrospektif olarak incelendi. Demografik veriler, daha oncesinde kullanmakta oldugu tedaviler ve hangi sebeple (relaps sikliginda artis, progresyon, MR aktivasyonu, hasta istegi, onceki ilaca bagli yan etki) oral tedavi tercih edildigi, hastalik sureleri ve ne kadar suredir oral tedavi kullanmakta olduklari, hastalik dizabilitesini olcen Expanded Disability Status Scale ( EDSS) skorlari kaydedildi. Bulgular: 2010 McDonald kriterlerine gore kesin MS tanisi alan 550 hasta calismaya dahil edildi. 108 (%19,6) hasta oral tedavilerden (fingolimod, dimetilfumarat ya da teriflunomid) birini kullanmaktaydi. 53 hasta (%49,1) fingolimod, 34 hasta (%31,5) dimetilfumarat, 21 hasta (%19,4) teriflunomid kullanmaktaydi. En fazla atak sikliginda artis nedeniyle oral tedavi tercih edildigi saptandi. Sonuc: Oral tedavi kulanim oranimiz Turkiye ortalamasinin altinda olsa da bulgularimiz, tedavi degisimi yaptigimizda tedavi penceresi icinde kaldigimizi gostermektedir.
Acute idiopathic demyelinating optic neuritis is frequently the initial manifestation of multiple sclerosis (MS). We aimed to discuss the value of color vision testing to detect possible optic nerve involvement in patients with MS who had no history of optic neuritis. We evaluated color vision with Farnsworth-Munsell 100 (FM-100) hue test. Total error scores (TES), partial error scores for the red-green axis (RGS) and blue-yellow axis (BYS) were calculated. Topographic optic disc parameters (RNFL, RA, DA, CV, RV, and vertical CAD ratio), total macular volume (TMV), central macular thickness (CMT), and retinal ganglion cell layer (RGCL) were determined using spectral domain optical coherence tomography (SD-OCT). Choroidal thickness (CT) was measured using enhanced depth imaging optical coherence tomography (EDI-OCT). Pattern visual evoked potentials (PVEP) were also performed. Twenty-eight patients with RRMS (56 eyes) and 25 healthy controls (50 eyes) were included. P100 latencies were significantly delayed and P100 amplitudes were significantly reduced in the patient group compared with the controls (p <= 0.05). Statistically significant thinning was found in temporal quadrant in the patient group compared with the controls (p = 0.002). TES RGS, and BYS were all increased in the patient group but this was not statistically significant. We found no correlation between TES, RGS, BYS, and P100 latencies or OCT parameters. In our investigation as to whether color vision testing could be a simple biomarker for showing neurodegeneration of the anterior visual pathway regardless of optic neuritis, PVEP and OCT-assessed RNFL thickness seemed to be a more valuable biomarker than color vision testing. (C) 2018 Elsevier Ltd. All rights reserved.
Objective: We aimed to report our nine Neuromyelitis Optica (NMO) and NMO Spectrum Disorders (NMOSD) cases which have different presentations. Patients and Methods: In this observational retrospective study, conducted between September 2011 and September 2015 in Antalya Education and Research Hospital Neurology Department, we enrolled 9 patients who were diagnosed as NMO/NMOSD. Results: We had 9 patients (8 female, 1 male). The mean age at onset was 49 years (36-67). While 7 patients were diagnosed with NMO, 2 patients’ diagnoses were NMOSD. The Case 6 was admitted to our clinic with an LETM (longitudinally extensive transverse myelitis) attack after 4 months of chemotherapy and radiotherapy treatment for gallbladder adenocancer. The Case 8 experienced LETM after the diagnosis of pulmonary tuberculosis and under anti-tuberculosis treatment, and the Case 9 was presented with LETM after post-vaccination. Optic nerve and spinal cord involvement occurred simultaneously in three patients. In three cases, optic neuritis developed 4 months, 15 months and 5 years after the myelitis attack. The Case 7 was referred to the hospital owing to cranial involvement, following a history of optic neuritis attacks six months earlier. The Case 1 had three recurrent optic neuritis attacks before the last attack of admission which included brain stem involvement and LETM. The Case 4 was receiving low dose interferone beta-1a with diagnosis of MS. NMO-IgG seropositivity was detected in 6 patients. Conclusion: Most of our patients were female and have relapsing course. Patients who experienced relapses, mostly were seropositive for NMO- IgG.
Background/Aims To identify the retinal vascular pathologies in patients with Alzheimer's type dementia (ATD) through optical coherence tomography angiography (OCTA) imaging. Methods Our study included 26 patients in the patient group, and age-matched and sex-matched 26 subjects in the control group. A detailed ophthalmological and neurological examination was performed for all subjects included in the study. The retinal, choroidal vascular structures and choroidal thickness (CT) of all subjects were analysed in a detailed way with a commercial spectral domain OCTA. Moreover, all participants underwent detailed neurological examination including Mini Mental State Examination (MMSE) test to evaluate cognitive function. Results In the group of patients with ATD, the MMSE score was significantly lower than that of the control group (p<0.001). The retinal vascular density was significantly lower than that of the control group in all zones (p<0.05). Foveal avascular zone (FAZ) was significantly enlarged compared with the control group (p=0.001). CT was significantly lower in the group of patients with ATD (p<0.001). Outer retinal and choroidal flow rates were lower in the group of patients with ATD, while the difference was not significant (p>0.05). Furthermore, significant correlation was found between the MMSE and all vascular density parameters, CT parameter and FAZ tested with OCTA imaging (p<0.05). Conclusions In patients with ATD, retinal and choroidal vascular pathologies detected through OCTA imaging can be used as a new biomarker in the early diagnosis of the disease, follow-up of its progression and in investigating the efficacy of the drugs.
Karotikokavernoz fistuller (KKF) internal karotid arter (IKA) ile kavernoz sinus (KS) arasindaki anormal arteriyovenoz anastomozlardir. Karotikokavernoz fistullerin direkt ve indirekt seklinde farkli klinik sunumu olan iki genis kategorisi vardir. Direkt ya da “yuksek akimli” KKF’de internal karotis arter ile kavernoz sinus arasinda; indirekt ya da “dusuk akimli” olanlarda ise internal veya external karotis arterin dallari arasinda anormal baglanti vardir. Ilki 87 yasinda erkek hasta akut baslayan sagda agrili komplet pupil tutulumu olan okulomotor paralizi ile digeri ise 65 yasinda kadin hasta iki yildir olan sag gozde kizariklik ile basvurdu. Kateter anjiografiyle dusuk akimli (dural) KKF saptandi. Dural KKF’ler klinik pratikde ender rastlanmalari nedeniyle bu tur olgularin hatirlanarak ayirici tanilarda yer almasi ve aciklanamayan ilgili klinigi olanlarda ileri tetkik acisindan israrci olunmasi gerekir.
PURPOSE:To assess cognitive performance differences among primary open-angle glaucoma (POAG) patients, normal-tension glaucoma (NTG) patients, and healthy control (C) subjects.METHODS:A total of 60 participants (20 POAG, 20 NTG, and 20 C subjects) were included in this study. A detailed ophthalmologic examination was performed on all participants. A spectral domain-optical coherence tomography (SD-OCT) system was used to measure the ganglion cell-inner plexiform layer (GC-IPL) and retinal nerve fiber layer (RNFL) thicknesses. To assess the cognitive performance of all participants, detailed neurological examinations, including the mini-mental state examination (MMSE), were performed by the same neurologist.RESULTS:There were no significant differences among the groups in terms of age (p =0.348) or gender (p =0.935). The mean RNFL thicknesses were significantly different among the groups (85.2 ± 14.7, 76.8 ± 10.3, and 91.4 ± 7.7 µm in the POAG, NTG, and C subjects, respectively; p <0.001). The mean GC-IPL thicknesses were 77.5 ± 9.7 µm in the POAG group, 73.4 ± 7.8 µm in the NTG group, and 78.8 ± 3.8 µm in the C group. Differences among the groups were not statistically significant (p =0.085). MMSE scores were 26.1 ± 1.4, 25.7 ± 2.3, and 28.8 ± 0.9 in the POAG, NTG, and C groups, respectively. There were significant differences among the three groups (p <0.001). Specifically, there were significant differences between the NTG and C groups (p <0.001), and between the POAG and C groups (p =0.001). There was no significant difference between the POAG and NTG groups (p =0.595).CONCLUSIONS:There appear to be similar risk factors in glaucoma and neurodegenerative disorders that cause deterioration in cognitive performance. Comparing the low MMSE scores of the POAG and NTG patients with the scores of healthy C participants supports our hypothesis. Consequently, it is recommended that a neurologist should also examine glaucoma patients.
Background: The klotho (Klt)-fibroblast growth factor-23 (FGF-23)-vitamin D axis is the main component of calcium (Ca) and phosphorus (P) metabolisms; on the contrary, it is also secreted from the choroid plexus (CP). Purpose: This study is aimed at evaluating serum soluble Klt (sKlt), FGF-23, and 25-(OH)-vitamin D levels in multiple sclerosis (MS) patients. Methods: Thirty-two relapsing-remitting MS patients (11 males and 21 females; mean age 38.3 years) and 31 age-sex matched healthy controls (12 males and 19 females; median age 38.5 years) were included in this study. All patients were diagnosed with MS according to the criteria of McDonald. Results: Serum sKlt, FGF-23, and P levels were significantly higher in MS patients compared to the control group (p < 0.01, p < 0.01, and p = 0.02, respectively). Serum 25-(OH)-vitamin D and Ca levels were significantly lower in MS patients (p < 0.01 and p = 0.04, respectively). Conclusion: Klt, which is secreted from CP, could be a response to the inflammatory condition in MS. Elevated FGF-23 levels suppress 1α-hydroxylase and upregulates 24α-hydroxylase, which results in a decrease in 1,25-(OH)2D3 levels. Thus, the neuroprotective and immunomodulatory effects of vitamin D might not be seen in MS patients.
Introduction - Human serum paraoxonase (PON1) and arylesterase (ARE) are lipophilic antioxidant enzymes. PON-1 serum activity diverges in individuals and populations, which might be due to polymorphisms in the PON-1 gene. The PON1 activity phenotyping method, based on the ratio of the stimulated PON activity and the ARE activity, could determine the low-activity homozygotes (QQ), intermediate activity heterozygotes (QR), and high-activity homozygotes (RR) regardless of the genotype. The aim of the present study was to determine the PON1 phenotype distribution and enzymatic activity of PON1 and ARE in multiple sclerosis (MS) patients. Materials and methods - Thirty-four relapsing remitting MS (RRMS) patients (22 females and 12 males; median age 42 (range 20-55) years) in the remission phase and thirty-four age-sex matched healthy controls (19 females and 15 males; median age 37 (21-60) years) were included in this study. All patients had clinically definite MS according to McDonald’s criteria. Results - Serum PON1 and ARE enzyme activities, as well as salt-stimulated PON1, were not significantly different between the patient and control groups. Phenotype distributions were as follows: QQ 58.8%, QR 38.2%, and RR 3% in MS patients (n=34); QQ 44.1%, QR 50%, and RR 5.9% in the control group (n=34). QQ (low activity) phenotypic distribution was more common in MS patients than controls, but this difference was not significant (p=0.14). Conclusions - Our results did not reveal meaningful relationships between PON1 activity or PON1 phenotypes and MS. More studies in larger samples and in all phases of the disease are needed in the future.
As anti-oxidative therapies come into consideration, finding feasible markers of oxidative stress becomes mandatory. We here in this study investigated the levels of major internal antioxidant uric acid (UA), oxidative stress parameters total antioxidant status (TAS), total oxidative status (TOS) in multiple sclerosis (MS) patients compared to controls. Thirty-five stable relapse remitting MS patients (15 males and 20 females; mean age 38 ± 11 years) and thirty-five age-sex matched healthy controls (13 males and 22 females; mean age 38 ± 10 years) were included in this study. All patients were diagnosed with MS according to the criteria of McDonald. Serum UA levels were significantly lower in the MS group (p = 0.03). When MS patients were sub-classified according to gender, female patients showed lower UA levels compared to male patients (p = 0.01). We did not find statistically significant differences in TAS and TOS between patients and controls. Serum UA correlated well with serum TAS as expected. However the correlation was more powerful in MS group (p < 0.0001 in MS group versus p = 0.02 in controls). Interpreting sole measurements of oxidative stress parameters may be deceptive as their values depend on many factors including serum levels of each other. We believe in the superiority of using TAS and TOS as markers of oxidative stress in MS patients as they inform about totals, independent of levels of individual parameters.