Combined Central and Peripheral Demyelination: Two Case Reports.

Combined central and peripheral demyelination (CCPD) is a rare disease characterized by demyelinating lesions in both the central nervous system (CNS) and peripheral nervous system (PNS). CCPD can present with acute, subacute, or chronic onset. The initial symptom may be of CNS origin, PNS origin, or both. The clinical manifestations of CCPD are quite heterogeneous, and there are no well-defined diagnostic criteria. In MRI imaging of CCPD cases, demyelinating lesions can be seen in areas such as the brain, cerebellum, brainstem, optic nerve, and spinal cord. Common electromyography (EMG) findings in patients with CCPD include decreased motor nerve conduction velocities, decreased or absent sensory nerve action potentials, prolonged F-wave latency, and decreased amplitude of compound muscle action potentials. Neurofascin (NF) is a transmembrane protein and anti-neurofascin (anti-NF) antibodies directed against NF can be positive in cases of CCPD. Four main NF polypeptides are produced by alternative splicing: NF 186, NF 180, NF 166, and NF 155. The investigation of anti-NF in CCPD cases is therefore important for etiological considerations. Here, we discussed three cases diagnosed with CCPD based on clinical, neuroimaging, EMG, and anti-NF antibody results in light of the literature.

The effect of sleep disorders on quality of life in patients with epilepsy: A multicenter study from Turkey.

OBJECTIVE: We aimed to investigate sleep disorders in patients with epilepsy (PWE) and to investigate the effects of sleep disorders on quality of life. METHODS: In our multicenter study conducted in Turkey, 1358 PWE were evaluated. The demographic and clinical data of the patients were recorded. The Insomnia Severity Index (ISI), Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Beck Depression Inventory (BDI), and Quality of Life in Epilepsy Inventory-10 (QOLIE-10) were administered. RESULTS: The mean age of 1358 patients was 35.92 ±â€¯14.11 (range, 18-89) years. Seven hundred fifty-one (55.30 %) were women. Some 12.7 % of the patients had insomnia (ISI > 14), 9.6 % had excessive daytime sleepiness (ESS > 10), 46.5 % had poor sleep quality (PSQI > 5), and 354 patients (26.1 %) had depressive symptoms (BDI > 16). The mean QOLIE-10 score was 22.82 ±â€¯8.14 (10-48). Resistant epilepsy was evaluated as the parameter with the highest risk affecting quality of life Adjusted odds ratio (AOR = 3.714; 95 % confidence interval (CI): [2.440-5.652] < 0.001)). ISI (AOR = 1.184; 95 % CI: [1.128-1.243]; p < 0.001), ESS (AOR = 1.081; 95 % CI: [1.034-1.130]; p < 0.001), PSQI (AOR = 0.928; 95 % CI: [0.867 - 0.994]; p = 0.034), BDI (AOR = 1.106; 95 % CI: [1.084-1.129]; p < 0.001), epilepsy duration (AOR = 1.023; 95 % CI: [1.004-1.041]; p = 0.014), were determined as factors affecting quality of life. SIGNIFICANCE: Sleep disorders are common in PWE and impair their quality of life. Quality of life can be improved by controlling the factors that may cause sleep disorders such as good seizure control, avoiding polypharmacy, and correcting the underlying mood disorders in patients with epilepsy.

Validation and reliability study of the Turkish version of the everyday cognition - 12 (T- ECog) scale.

OBJECTIVE: Assessing the activities of daily living (ADL) is important in cognitive impairment. The everyday cognition scale includes 12 items (ECog-12). It evaluates complex ADLs and executive functions. This scale can differentiate healthy elderly people from patients with mild cognitive impairment (MCI) as well as MCI from dementia patients. Our aim is to validate a Turkish version of ECog-12. METHODS: The study group consisted of 40 healthy elders, 40 patients with Alzheimer's disease (AD), and 40 patients with MCI. In addition to T-ECog-12, test - your memory- Turkish version (TYM-TR), Geriatric Dementia Scale (GDS), the Blessed orientation-memory-concentration (BOMC), and Katz ADL tests were administered to all participants for concurrent validity. RESULTS: Cronbach's alpha test showed excellent internal consistency (0.93). When T-ECog-12 was compared to the other tests, strong positive correlations were found between the GDS and BOMC; in addition, strong negative correlations were found between Katz ADL and TYM-TR scale. ECog-12 was found to be sensitive in differentiating healthy individuals from individuals with dementia (AD and MCI) (Area under the curve [AUC]=0.82, Cl=0.74-0.89). It was found to have low sensitivity in discriminating between MCI and healthy individuals (AUC=0.52, Cl=0.42-0.63). CONCLUSION: T-ECog-12 was found to be reliable and valid for Turkish population. This scale is reliable and effective in diagnostic distinguishing healthy individuals from dementia.

Decreased levels of cytokines implicate altered immune response in plasma of moderate-stage Alzheimer's disease patients.

Alzheimer's Disease (AD) is a neurodegenerative disease characterized by the accumulation of amyloid plaques and neurofibrillary tangles in the brain. However, increasing evidence suggests that the pathogenesis of the disease is associated with peripheral inflammation. Here, we aimed to determine plasma concentrations of multiple cytokines and chemokines from moderate-stage AD and age-matched controls. Changes in a total of 20 cytokines and chemokines in plasma of moderate-stage AD were evaluated by using quantitative microarray. Six of them, namely MCP-1, MIP-1a, MIP-1b, MMP-9, RANTES, and VEGF, were found to be significantly reduced in moderate-stage AD patients (n = 25) in comparison to age-matched and non-demented controls (n = 25). However, GM-CSF, GRO-α/ß/γ, IFN- γ, IL-1α, IL-1ß, IL-10, IL-12 p70, IL-13, IL-2, IL- 4, IL-5, IL-6, IL-8, and TNF-α showed no significant differences between the patient and control groups. On the contrary to previous early-stage AD studies that show increased plasma cytokine/chemokine levels, our results indicate that inflammatory plasma molecules are reduced in moderate-stage AD. This finding points out the reduced immune responsiveness, which is known to be directly correlated to the degree of AD.

Kappa/Lambda light-chain typing in Alzheimer's Disease.

BACKGROUND: Alzheimer's disease is a progressive neurodegenerative disorder characterized by memory loss and cognitive impairment. The diagnosis of Alzheimer's disease according to symptomatic events is still a puzzling task. Developing a biomarker-based, low-cost, and high-throughput test, readily applicable in clinical laboratories, dramatically impacts the rapid and reliable detection of the disease. OBJECTIVE: This study aimed to develop an accurate, sensitive, and reliable screening tool for diagnosing Alzheimer's disease, which can significantly reduce the cost and time of existing methods. METHODS: We have employed a MALDI-TOF-MS-based methodology combined with a microaffinity chromatography enrichment approach using affinity capture resins to determine serum kappa (κ) and lambda (λ) light chain levels in control and patients with AD. RESULTS: We observed a statistically significant difference in the kappa light chain over lambda light chain (κLC/λLC) ratios between patients with AD and controls (mean difference -0,409; % 95 CI:- 0.547 to -0.269; p<0.001). Our method demonstrated higher sensitivity (100.00%) and specificity (71.43%) for discrimination between AD and controls. CONCLUSION: We have developed a high-throughput screening test with a novel sample enrichment method for determining κLC/λLC ratios associated with AD diagnosis. Following further validation, we believe our test has the potential for clinical laboratories.

Neuropsychiatric Effects of COVID-19 Pandemic on Alzheimer's Disease: A Comparative Study of Total and Partial Lockdown.

Objectives: Coronavirus disease 2019 (COVID-19)-related lockdown may have a negative effect on the neuropsychiatric status of Alzheimer's disease (AD) cases. In this study, it was aimed to find future implications by evaluating the neuropsychiatric conditions of AD cases during total and partial lockdown periods. Methods: It is a prospective, cross-sectional, and multicenter study that includes AD cases which have been followed for at least 1 year by outpatient clinics from different regions of Turkey. Sociodemographic data, comorbidities, mobility, existence of social interactions, clinical dementia rating (CDR) scale, and neuropsychiatric inventory (NPI) for total and partial lockdown were questioned by the caregivers with the help of case files of the patients. Results: A total of 302 AD cases were enrolled to the study (mean age: 78±8 years, mean duration of education: 5.8±9 years). The total comorbidity ratio was found to be 84%, with the most frequent comorbidity being hypertension. The mean NPI score was 22.9±21 in total lockdown and 17.7±15 in partial lockdown, which is statistically significantly different. When lockdown periods were compared with the total scores of NPI scores according to gender, existence of social interactions, mobility, and comorbidities were found higher in the total lockdown than the partial lockdown. When switching from total lockdown to partial lockdown, the presence of comorbidities, mobility, and CDR were found to be factors that had a significant effect on NPI scores. In regression analysis, CDR score was found as the most effective parameter on the neuropsychiatric status of AD cases for both lockdown periods. Conclusion: When lockdown-related restrictions were reduced, the neuropsychological conditions of AD cases were significantly improved. Lockdown rules should be considered with these data in mind.

Demyelinating Disease of the Central Nervous System Concurrent With COVID-19.

Neurological diseases related to coronavirus disease-2019 (COVID-19) are increasingly reported. We report here three cases that presented with subtle neurologic findings manifesting within a range of 15 days to four months after their COVID-19 diagnoses. Magnetic resonance imaging showed acute multifocal periventricular and subcortical demyelinating lesions. Some of the lesions showed contrast enhancement and diffusion restriction. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) PCR was found in the cerebrospinal fluid of just one patient. All patients received intravenous methylprednisolone therapy. In this report, we aim to discuss the aspects of possible COVID-19-related demyelination that support a diagnosis of multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM).

The Impact of the COVID-19 Lockdown on the Quality of Life in Chronic Neurological Diseases: The Results of a COVQoL-CND Study.

BACKGROUND: Coronavirus disease 2019 (COVID-19) pandemic and lockdown period may induce an impairment in quality of life (QoL), disruption in treatment (DIT), and posttraumatic stress disorder (PTSD) in chronic neurological diseases (CNDs). To reach this information, a multicenter, cross-sectional study (COVQoL-CND) was planned. Parkinson's disease (PD), headache (HA), multiple sclerosis (MS), epilepsy (EP), polyneuropathy (PNP), and cerebrovascular disease (CVD) were selected as the CND. METHODS: The COVQoL-CND study includes demographic data, the World Health Organization Quality of Life short form (WHOQOL-BREF), and Impact of Event Scale-Revised (IES-R) forms. RESULTS: The mean age of a total of 577 patients was 49 ± 17 (19-87 years), and the ratio of female/male was 352/225. The mean age of patients with PD, HA, MS, EP, PNP, and CVD were 65 ± 11, 39 ± 12, 38 ± 10, 47 ± 17, 61 ± 12, and 60 ± 15 years, respectively. The IES-R scores were found to be higher in the younger group, those with comorbid disease, contacted with CO-VID-19 patients, or diagnosed with COVID-19. In the group with a high IES-R score, the rate of DIT was found to be high. IES-R scores were negatively correlated with QoL. IES-R total scores were found highest in the CVD group and lowest in the PD group. The ratio of DIT was found highest in the PNP group and the lowest in the EP group. Contact with CO-VID-19 patients was high in the EP and HA group. CONCLUSIONS: The results of the COVQoL-CND study showed that lockdown causes posttraumatic stress and deterioration in the QoL in CND.

Superior Optic Vein Thrombosis Related to Orbital Cellulitis Secondary to Aquatic Injury.

A 52-year-old woman presented with orbital cellulitis and sixth cranial nerve palsy as a result of striking the tail of a stingray while swimming. Her ophthalmologic and neurologic examination showed injury of the conjunctiva, corneal abrasion without mention of foreign body, contusion of the eyelid, and isolated lateral gaze palsy and ptosis in the right eye. Orbital magnetic resonance (MR) imaging and MR venography showed orbital cellulitis, superior and lateral rectus edema, and thrombosis of the superior ophthalmic vein on the right eye. She was treated appropriately, and her physical examination showed significant improvement within three months.

Validation and reliability study of the Turkish version of the Neuroquality of Life (Neuro-QoL)-Stigma Scale for neurological disorders

Background/aim: Stigma can be defined as a negative perception of chronically ill patients by their relatives or by society, or a similar self-perception by the patients themselves. We aimed to validate the Turkish version of the Neuroquality of Life (Neuro-QoL)-Stigma Scale for neurologic diseases. Materials and methods: Forms were filled out by a total of 152 randomized patients under regular follow-up in the outpatient clinic (29 polyneuropathy, 25 epilepsy, 23 stroke, 24 tension-type headache, 28 multiple sclerosis, 27 Parkinson disease). The forms consisted of the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), WHOQOL-BREF quality of life scale, the Multidimensional Scale of Perceived Social Support (MSPSS), the General Self-Efficacy (GSE) scale, and the Neuro-QoL-Stigma scale. Results: The internal consistency of the Neuro-QoL-Stigma scale showed Cronbach's α coefficients of 0.95 for all groups. The mean scores of the stigma scales were 33.42 ± 13.91 (min­max: 24­87). There were strong negative correlations between high stigma scores and GSE-T, MSPSS-T, and WHOQOL-BREF, and a positive correlation with the BDI and BAI. Conclusion: The Turkish version of Neuro-QoL-Stigma has satisfactory content validity and high internal consistency. Neuro-QoL-Stigma is suitable for understanding stigmatization in different neurological disorders in the Turkish population. The scale is available for use at http://www.healthmeasures.net/explore-measurement-systems/neuro-qol.

Distinguishing Depressive Pseudodementia from Alzheimer Disease: A Comparative Study of Hippocampal Volumetry and Cognitive Tests.

BACKGROUND AND AIM: Depressive pseudodementia (DPD) is a condition which may develop secondary to depression. The aim of this study was to contribute to the differential diagnosis between Alzheimer disease (AD) and DPD by comparing the neurocognitive tests and hippocampal volume. MATERIALS AND METHODS: Patients who met criteria of AD/DPD were enrolled in the study. All patients were assessed using the Wechsler Memory Scale (WMS), clock-drawing test, Stroop test, Benton Facial Recognition Test (BFRT), Boston Naming Test, Mini-Mental State Examination (MMSE), and Geriatric Depression Scale (GDS). Hippocampal volume was measured by importing the coronal T1-weighted magnetic resonance images to the Vitrea 2 workstation. RESULTS: A significant difference was found between the AD and DPD groups on the WMS test, clock-drawing test, Stroop test, Boston Naming Test, MMSE, GDS, and left hippocampal volume. A significant correlation between BFRT and bilateral hippocampal volumes was found in the AD group. No correlation was found among parameters in DPD patients. CONCLUSIONS: Our results suggest that evaluation of facial recognition and left hippocampal volume may provide more reliable evidence for distinguishing DPD from AD. Further investigations combined with functional imaging techniques including more patients are needed.

Are Cerebrospinal Fluid Protein Levels and Plasma Neutrophil/Lymphocyte Ratio Associated with Prognosis of Guillain Barré Syndrome?

Guillain Barré syndrome (GBS) is a post-infectious acute autoimmune polyradiculopathy. Cerebrospinal fluid (CSF) total protein level and plasma neutrophil/lymphocyte ratio (NLR) are related with autoimmune response. We aimed to reach a prognostic indicator for GBS by using electrophysiological findings, protein level of CSF, and plasma NLR based on Medical Research Council (MRC) sum score data. Cases who met diagnostic criteria of GBS and followed at least six months were enrolled in the study. Nerve conduction study (NCS) and lumbar puncture were performed one week after symptom onset. Routine CSF findings and complete blood count were recorded. Plasma NLR was calculated as the ratio of neutrophil cell count to lymphocyte cell count. All patients received intravenous immunoglobulin. MRC sum scores were calculated on administration time (1st) and six months later (2nd) for evaluation of recovery. Mean values of baseline CSF protein level, NCS parameters and NLR were compared with mean scores of MRC1st and MRC2nd. Increased CSF protein levels showed negative correlation with MRC2nd scores but no correlation with NCS. Increased NLR levels were positively correlated with age, MRC2nd scores and NCS. Facial diplegia was observed in 42% of patients. A positive correlation was found between high level of NLR and MRC1st, and there was no relationship with MRC2nd. Regression analyses showed that only CSF protein level was an independent factor on both MRC1st and MRC2nd. A positive association was found between baseline data included young age high plasma NLR, low level of CSF protein and good prognosis in our study. Also a positive correlation was found between high level of NLR and baseline disability in GBS cases with facial diplegia. Calculation of NLR is an easy and inexpensive method. On the other hand it may be influenced by age and immunotherapy. Our results showed that CSF protein level is still a liable parameter for prognosis. NLR could be a candidate prognostic marker of GBS cases. Further investigations including more cases are needed.

Distinguishing age-related cognitive decline from dementias: A study based on machine learning algorithms.

BACKGROUND AND AIM: This study aims to examine the distinguishability of age-related cognitive decline (ARCD) from dementias based on some neurocognitive tests using machine learning. MATERIALS AND METHODS: 106 subjects were divided into four groups: ARCD (n=30), probable Alzheimer's disease (AD) (n=20), vascular dementia (VD) (n=21) and amnestic mild cognitive impairment (MCI) (n=35). The following tests were applied to all subjects: The Wechsler memory scale-revised, a clock-drawing, the dual similarities, interpretation of proverbs, word fluency, the Stroop, the Boston naming (BNT), the Benton face recognition, a copying-drawings and Öktem verbal memory processes (Ö-VMPT) tests. A multilayer perceptron, a support vector machine and a classification via regression with M5-model trees were employed for classification. RESULTS: The pairwise classification results show that ARCD is completely separable from AD with a success rate of 100% and highly separable from MCI and VD with success rates of 95.4% and 86.30%, respectively. The neurocognitive tests with the higher merit values were Ö-VMPT recognition (ARCD vs. AD), Ö-VMPT total learning (ARCD vs. MCI) and semantic fluency, proverbs, Stroop interference and naming BNT (ARCD vs. VD). CONCLUSION: The findings show that machine learning can be successfully utilized for distinguishing ARCD from dementias based on neurocognitive tests.

The connection between MCI and Alzheimer disease: neurocognitive clues.

BACKGROUND/AIM: Mild cognitive impairment (MCI) is defined as a pathological stage between 'healthy aging' and 'dementia' In this study, cases of MCI were compared with early-stage Alzheimer disease (AD) and age-related cognitive decline (ARCD) in terms of cognitive profiles in order to find a connection between MCI and AD. MATERIALS AND METHODS: Patients who were comparable in terms of age and sex and who met the criteria of MCI, ARCD, or early-stage AD were included in the study retrospectively. Wechsler memory scale, executive function, visuospatial, language, and memory tests were applied to all subjects. Additionally, all patients completed a mini-mental state examination test, geriatric depression scale, and activities of daily living scale. RESULTS: Complex attention tests and long-term memory tests were more impaired in MCI patients when compared with ARCD. However, there were no significant differences between the MCI and ARCD cases in activities of daily living. Memory and executive functions were more deteriorated in patients with AD in comparison to MCI. CONCLUSION: During the follow-up period of ARCD, impairment in orientation, complex attention, and long-term memory should suggest the diagnosis of MCI. When personal information and executive functions are affected in MCI, AD should be carefully considered.