Autoimmune encephalitis and paraneoplastic syndromes in Turkey: a multi-centre study.

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.

Is multiple sclerosis a risk factor for atherosclerosis?

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 cut-off 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.

Montreal cognitive assessment in primary sjogrens syndrome. A brief screening tool.

OBJECTIVE: To use the Montreal Cognitive Assessment (MoCA) test to assess the subclinical cognitive impairment in patients with Primary Sjogren`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 more than 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 more than 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 less than 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.

Color vision testing versus pattern visual evoked potentials and optical coherence tomography parameters in subclinical optic nerve involvement in multiple sclerosis.

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 C/D 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.

Evaluation of optical coherence tomography angiographic findings in Alzheimer's type dementia.

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.

The Three Sisters of Fate in Multiple Sclerosis: Klotho (Clotho), Fibroblast Growth Factor-23 (Lachesis), and Vitamin D (Atropos).

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.

Cognitive performance of primary open-angle glaucoma and normal-tension glaucoma patients.

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.

Choroidal Thickness in Patients with Mild Cognitive Impairment and Alzheimer's Type Dementia.

Aim. To asses both choroidal thickness differences among Alzheimer's type dementia (ATD) patients, mild cognitive impairment (MCI) patients, and healthy control (C) subjects and choroidal thickness relationships with cognitive performance. Methods. A total of 246 eyes of 123 people (41 ATD, 38 MCI, and 44 healthy C subjects) were included in this study. Complete ophthalmological and neurological examination was performed in all subjects. Choroidal thicknesses (CT) were measured at seven locations: the fovea, 500-1500-3000 µm temporal and 500-1500-3000 µm nasal to the fovea by enhanced depth imaging optical coherence tomography (EDI-OCT). Detailed neurological examination including mini mental state examination (MMSE) test which evaluates the cognitive function was applied to all participants. Results. The ages and genders of all participants were similar in all groups. Compared with healthy C subjects, the CT measurements at all regions were significantly thinner both in patients with ATD and in patients with MCI than in healthy C subjects (p < 0.05). The MMSE scores were significantly different among ATD patients, MCI patients, and healthy C subjects. They were 19.3 ± 1.8, 24.8 ± 0.9, and 27.6 ± 1.2 in ATD, MCI, and healthy controls, respectively (p < 0.001). There were also significant correlation between MMSE score and choroidal thickness at each location (p < 0.05). Conclusions. CT was reduced in ATD patients and MCI patients. Since vascular structures were affected in ATD patients and MCI patients, they had thin CT. Besides CT was correlated with degree of cognitive impairment. Therefore CT may be a new biomarker in diagnosis and follow-up of MCI and ATD patients.

Ischemia modified albumin is an indicator of oxidative stress in multiple sclerosis.

INTRODUCTION: Oligodendrocytes need iron in processes of energy generation and myelination. However, excessive levels of iron may exert iron induced oxidative stress and thus lead to tissue degeneration. Monitoring oxidative stress will be of paramount importance in follow-up of patients with many diseases including multiple sclerosis (MS). The aim of this study was to measure total anti-oxidative status (TAS), total oxidative status (TOS) and ischemia modified albumin (IMA) in stable relapse remitting MS (RRMS) patients. MATERIALS AND METHODS: Thirty-five RRMS patients (15 males and 20 females; median age 42 (20-55) years) and thirty-five age-sex matched healthy controls (13 males and 22 females; median age 37 (21-60) years) were included in this study. All patients were diagnosed with MS according to the criteria of McDonald. RESULTS: IMA levels were significantly higher in RRMS patients (P < 0.001), while TAS and TOS did not show any significant difference between groups (P = 0.870 and P = 0.460, respectively). CONCLUSIONS: Our results suggest IMA as a more efficient serum marker than TAS and TOS in detecting the oxidative stress in MS patients. Serum oxidative stress markers should be included in future study protocols besides clinical and radiological parameters.