Shrinkage of olfactory amygdala connotes cognitive impairment in patients with Parkinson's disease.

During the caudo-rostral progression of Lewy pathology, the amygdala is involved relatively early in Parkinson's disease (PD). However, lesser is known about the volumetric differences at the amygdala subdivisions, although the evidence mainly implicates the olfactory amygdala. We aimed to investigate the volumetric differences between the amygdala's nuclear and sectoral subdivisions in the PD cognitive impairment continuum compared to healthy controls (HC). The volumes of nine nuclei of the amygdala were estimated with FreeSurfer (nuclear parcellation-NP) from T1-weighted images of PD patients with normal cognition (PD-CN), PD with mild cognitive impairment (PD-MCI), PD with dementia (PD-D), and HC. The appropriate nuclei were then merged to obtain three sectors of the amygdala (sectoral parcellation-SP). The nuclear and sectoral volumes were compared among the four groups and between the hyposmic and normosmic PD patients. There was a significant difference in the total amygdala volume among the four groups. In terms of nuclei, the bilateral cortico-amygdaloid transition area (CAT) and sectors superficial cortex-like region (sCLR) volumes of PD-MCI and PD-D were less than those of the PD-CN and HC. A linear discriminant analysis revealed that left CAT and left sCLR volumes classified the PD-CN and cognitively impaired PD (PD-CI: PD-MCI plus PD-D) with 90.7% accuracy according to NP and 85.2% accuracy to SP. Similarly, left CAT and sCLR volumes correctly identified the hyposmic and normosmic PD with 64.8% and 61.1% accuracies. Notably, the left olfactory amygdala volume successfully discriminated cognitive impairment in PD and could be used as neuroimaging-based support for PD-CI diagnosis. Supplementary Information: The online version contains supplementary material available at 10.1007/s11571-022-09887-y.

Altered learning and transfer abilities in Korsakoff's syndrome depending on task complexity.

Korsakoff's syndrome (KS) is characterized by episodic memory impairment due to damage to the medial diencephalic structures. Although commonly associated with chronic alcoholism, starvation due to the hunger strike is one of its nonalcoholic causes. Learning the stimulus-response associations and transferring the just-learned associations to novel combinations were previously tested by specific tasks in memory-impaired patients with hippocampal, basal forebrain, and basal ganglia damage. To add to this previous research, we aimed to use the same tasks in a group of patients with hunger strike-related KS presenting a stable isolated amnestic profile. Twelve patients with hunger strike-related KS and matched healthy controls were tested in two tasks varying in task complexity. Each task included two phases: the initial phase is feedback-based learning of (simple vs. complex) stimulus-response associations, and the following phase is transfer generalization (in the presence vs. absence of feedback). On a task involving simple associations, five patients with KS failed to learn the associations, while the other seven patients showed intact learning and transfer. On the other task involving more complex associations, seven patients showed slower learning and failed at transfer generalization, whereas the other five patients failed even at the acquisition phase. These findings of a task-complexity-related impairment on associative learning and transfer represent a distinct pattern from the spared learning but impaired transfer previously observed on these tasks in patients with medial temporal lobe amnesia.

Brain reserve contributes to distinguishing preclinical Alzheimer's stages 1 and 2.

BACKGROUND: In preclinical Alzheimer's disease, it is unclear why some individuals with amyloid pathologic change are asymptomatic (stage 1), whereas others experience subjective cognitive decline (SCD, stage 2). Here, we examined the association of stage 1 vs. stage 2 with structural brain reserve in memory-related brain regions. METHODS: We tested whether the volumes of hippocampal subfields and parahippocampal regions were larger in individuals at stage 1 compared to asymptomatic amyloid-negative older adults (healthy controls, HCs). We also tested whether individuals with stage 2 would show the opposite pattern, namely smaller brain volumes than in amyloid-negative individuals with SCD. Participants with cerebrospinal fluid (CSF) biomarker data and bilateral volumetric MRI data from the observational, multi-centric DZNE-Longitudinal Cognitive Impairment and Dementia Study (DELCODE) study were included. The sample comprised 95 amyloid-negative and 26 amyloid-positive asymptomatic participants as well as 104 amyloid-negative and 47 amyloid-positive individuals with SCD. Volumes were based on high-resolution T2-weighted images and automatic segmentation with manual correction according to a recently established high-resolution segmentation protocol. RESULTS: In asymptomatic individuals, brain volumes of hippocampal subfields and of the parahippocampal cortex were numerically larger in stage 1 compared to HCs, whereas the opposite was the case in individuals with SCD. MANOVAs with volumes as dependent data and age, sex, years of education, and DELCODE site as covariates showed a significant interaction between diagnosis (asymptomatic versus SCD) and amyloid status (Aß42/40 negative versus positive) for hippocampal subfields. Post hoc paired comparisons taking into account the same covariates showed that dentate gyrus and CA1 volumes in SCD were significantly smaller in amyloid-positive than negative individuals. In contrast, CA1 volumes were significantly (p = 0.014) larger in stage 1 compared with HCs. CONCLUSIONS: These data indicate that HCs and stages 1 and 2 do not correspond to linear brain volume reduction. Instead, stage 1 is associated with larger than expected volumes of hippocampal subfields in the face of amyloid pathology. This indicates a brain reserve mechanism in stage 1 that enables individuals with amyloid pathologic change to be cognitively normal and asymptomatic without subjective cognitive decline.

Mild Encephalitis/Encephalopathy with a Reversible Lesion in The Splenium.

Mild encephalitis/encephalopathy with a reversible lesion in the splenium (MERS) is a clinico-radiological syndrome with mild central nervous system symptoms and a reversible lesion in the splenium of the corpus callosum. It is mainly associated with a number of viral and bacterial infections, including Coronavirus disease 2019 (COVID-19). In this paper, we report four MERS patients. One had a mumps infection, the second had aseptic meningitis, the third had Marchiafava-Bignami disease, and the fourth had atypical pneumonia associated with COVID-19 infection.

Senior Moments Are Never-ending Times When You Are Old (Are They?): First Step of Turquoise Project.

Introduction: The number of dementia patients in Turkey is increasing, as well as all over the world. However, we do not know how much society knows about dementia. The aim of this study is to evaluate people's concept of dementia, their awareness of dementia research and treatment, whether dementia and forgetfulness are considered normal in old age, and whether having dementia is associated with a lack of mental abilities. Methods: A Dementia Awareness Questionnaire was created in the form of a self-report questionnaire, consisting of 20 questions and using a five-point Likert-type answering method in order to question participants' information about dementia. In addition, we asked for demographic information such as age, gender, occupation, education level of the participants, as well as whether they have had relatives diagnosed with a neurodegenerative disease. The surveys were administered online. Results: A total of 1551 participants from 53 cities were included in the study. Approximately half of the participants did not know the definition of dementia, 20.9% thought that dementia and Alzheimer's disease were the same; 50.4% considered forgetfulness, and 55.2% considered dementia as a natural consequence of aging. While 34.5% of the participants thought that dementia patients could be dangerous, 10.3% thought they could not continue living as a part of society. While 38.5% of healthcare professionals do not know the definition of dementia, 18.5% of them say that dementia and Alzheimer's disease are the same, 58.5% think that dementia patients are not fit to make their own decisions, 40.6% believe that dementia patients have criminal liability. 15.8% of healthcare professionals thought that dementia is only seen in elderly people; 21.4% thought that dementia, and 49.2% thought that forgetfulness was a result of normal aging. Conclusion: Our study confirms that dementia is still an unknown concept in society and among healthcare professionals. It is widely believed that forgetfulness and dementia are part of normal aging, and there is no cure for dementia. This study, which we have done in order to understand the level of dementia awareness in Turkish society, reveals the necessity for research on dementia and studies on how to increase dementia awareness.

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.

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.

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN.

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report - A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro-logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.

The Right Temporal Variant of Frontotemporal Dementia Is Not Genetically Sporadic: A Case Series.

BACKGROUND: Right temporal variant frontotemporal dementia (rtvFTD) has been generally considered as a right sided variant of semantic variant primary progressive aphasia (svPPA), which is a genetically sporadic disorder. Recently, we have shown that rtvFTD has a unique clinical syndrome compared to svPPA and behavioral variant frontotemporal dementia. OBJECTIVE: We challenge the assumption that rtvFTD is a sporadic, non-familial variant of FTD by identifying potential autosomal dominant inheritance and related genes in rtvFTD. METHODS: We collected all subjects with a diagnosis of FTD or primary progressive aphasia who had undergone genetic screening (n = 284) and subsequently who had a genetic variant (n = 48) with a diagnosis of rtvFTD (n = 6) in 2 specialized memory clinics. RESULTS: Genetic variants in FTD related genes were found in 33% of genetically screened rtvFTD cases; including MAPT (n = 4), GRN (n = 1), and TARDBP (n = 1) genes, whereas only one svPPA case had a genetic variant in our combined cohorts. Additionally, 4 out of 6 rtvFTD subjects had a strong family history for dementia. CONCLUSION: Our results demonstrate that rtvFTD, unlike svPPA, is not a pure sporadic, but a heterogeneous potential genetic variant of FTD, and screening for genetic causes for FTD should be performed in patients with rtvFTD.

The cerebral blood flow deficits in Parkinson's disease with mild cognitive impairment using arterial spin labeling MRI.

Parkinson's disease (PD) with mild cognitive impairment (PD-MCI) is currently diagnosed based on an arbitrarily predefined standard deviation of neuropsychological test scores, and more objective biomarkers for PD-MCI diagnosis are needed. The purpose of this study was to define possible brain perfusion-based biomarkers of not only mild cognitive impairment, but also risky gene carriers in PD using arterial spin labeling magnetic resonance imaging (ASL-MRI). Fifteen healthy controls (HC), 26 cognitively normal PD (PD-CN), and 27 PD-MCI subjects participated in this study. ASL-MRI data were acquired by signal targeting with alternating radio-frequency labeling with Look-Locker sequence at 3 T. Single nucleotide polymorphism genotyping for rs9468 [microtubule-associated protein tau (MAPT) H1/H1 versus H1/H2 haplotype] was performed using a Stratagene Mx3005p real-time polymerase chain-reaction system (Agilent Technologies, USA). There were 15 subjects with MAPT H1/H1 and 11 subjects with MAPT H1/H2 within PD-MCI, and 33 subjects with MAPT H1/H1 and 19 subjects with MAPT H1/H2 within all PD. Voxel-wise differences of cerebral blood flow (CBF) values between HC, PD-CN and PD-MCI were assessed by one-way analysis of variance followed by pairwise post hoc comparisons. Further, the subgroup of PD patients carrying the risky MAPT H1/H1 haplotype was compared with noncarriers (MAPT H1/H2 haplotype) in terms of CBF by a two-sample t test. A pattern that could be summarized as "posterior hypoperfusion" (PH) differentiated the PD-MCI group from the HC group with an accuracy of 92.6% (sensitivity = 93%, specificity = 93%). Additionally, the PD patients with MAPT H1/H1 haplotype had decreased perfusion than the ones with H1/H2 haplotype at the posterior areas of the visual network (VN), default mode network (DMN), and dorsal attention network (DAN). The PH-type pattern in ASL-MRI could be employed as a biomarker of both current cognitive impairment and future cognitive decline in PD.

Who is the bigger stigmatizor?: The loved one or the society.

OBJECTIVE: Epilepsy has long been considered by the society as a dangerous and frightening spiritual possession or even a contagious disease. This results in an unavoidable embarrassment for both the patient and the family leading to social isolation, seclusion, and secretiveness about the diagnosis. The aim of this study was to determine the stigmatizing level of the general Turkish population and to compare these results with the stigmatizing level of the patients' relatives group (PRG). METHODS: This is a substudy of our main study which aimed to develop two scales for the quantification of the stigma level in patients with epilepsy (PWE) and their relatives [Stigma Scale for Epilepsy (SSE) -Self Report (SR) and -Informant Report (IR)]. First-degree relatives of the patients had been included in the main study. For the present study, 202 healthy people who were caregivers and relatives of neurologic patients other than epilepsy were recruited for the control group (CG). A sociodemographic and clinical data form and SSE-IR scale were administered to the CG as well. The relationship between sociodemographic characteristics and SSE-IR scores was evaluated, and a regression analysis was performed in order to analyze sociodemographic factors contributing to SSE-IR scores. Stigmatizing levels were compared between PRG and CG. Statistical analysis was performed using Statistical Package for the Social Sciences version 22.0 software. RESULTS: Comparison of stigma scores among different sociodemographic strata of the CG showed that there was a statistically significant difference in terms of educational status and occupation (p < 0.01). Multivariate linear regression analysis revealed that education accounted for 10.8% and 8.9% of the variance in the SSE-IR scale respectively in the PRG and the CG. Prejudgment scores and total scores of the PRG were significantly higher than those of the CG. There was no statistically significant difference between two groups in terms of discrimination and false beliefs subscales scores. The proportion of highly stigmatizor participants in the PRG was statistically significantly higher than that of the CG. DISCUSSION/CONCLUSIONS: This study showed us that the stigmatization levels in a group of subjects drawn from general population without acquaintance of epilepsy were lower than the relatives of the patients. This result may be partially explained by the ambivalent attitudes of the relatives, as those high scores may stem from not only enacted but also the felt stigma that they were experiencing themselves. It must be a warning sign for both of all the clinicians treating epilepsy and national association against epilepsy, as well as public health officials to increase efforts for awareness raising.

Stigma accounts for depression in patients with epilepsy.

OBJECTIVE: Epilepsy is a chronic disease with an increased risk of stigmatization due to psychosocial consequences of the seizures. Intuitively, one may well conclude that stigmatization would lead to depression in patients with epilepsy as well as in other patient groups with increased risk of stigmatization. Indeed, there are a few studies in support of this intuition. In this study, we aimed to investigate the relationship between level of stigmatization and the severity of the depressive symptoms in our patients with epilepsy. METHODS: This is a substudy of our main study, which aimed to develop a scale for the quantification of the stigma level in patients with epilepsy. The study included a total of 302 patients with epilepsy, who had at least a literacy level education and one-week-seizure-freedom. Beck Depression Inventory (BDI) was used to quantify depressive symptoms. The correlation between BDI scores and the Stigma Scale for Epilepsy-Self Report (SSE-SR) scores was evaluated. A regression analysis was done in order to parse out significant sociodemographic and clinical factors contributing to depressive symptoms. Statistical analyses were done using the Statistics Package for the Social Sciences software 24.0 package program. RESULTS: We saw that 46.9% (n=139) of this population rated themselves as having at least mildly depressive symptoms with BDI (BDI>9). There was a moderate positive correlation between stigma scores and BDI scores (p=0.000, r=0.504), and 96.3% of highly stigmatized patients had at least mildly depressive scores, 73.9% of the nonstigmatized group had none or minimal depressive scores. Stigma scores (ß=.51), gender, educational level, seizure frequency, and income level were the variables significantly affecting the BDI scores. Stigma score accounted for 26.2% of the variance in the BDI score. CONCLUSION: This study shows that stigmatization of the patients with epilepsy leads to depression in those patients. Therefore, protection of the patients with epilepsy against stigmatization may also help to protect them from a concomitant disabling condition. On the other hand, detection for depressive symptoms in already stigmatized patients with epilepsy may unearth a treatable condition.

Development and validation of the stigma scale for epilepsy in Turkey.

OBJECTIVE: Epilepsy is a chronic disease with an increased risk of stigma. The aim of this study was to investigate the efficacy of a scale developed by the authors to determine the level of stigma in Turkish patients with epilepsy and their relatives. METHODS: In this pilot study, two scales were developed, one consisting of 32 questions for the patients and one of 20 questions for the patients' relatives. Initially, a total of 30 patients with epilepsy and 30 relatives of the patients were included. The Cronbach's alpha coefficient was calculated in a reliability analysis of validity applying the scales to 302 patients and 201 relatives of the patients. The Pearson correlation coefficient was used for the reliability analysis of the test-retest. The t-test was used in paired series, and factor analysis was conducted. The correlation between the clinical and demographical data and the stigma scores was evaluated. RESULTS: The scales were applied to participants twice under the same conditions in one-week interval. In the test-retest analysis, the internal consistency of the scales was high and reliable. In the analysis of the patients, the Cronbach's alpha value of the scale was found to be 0.915. In the factor analysis, the questions were grouped into five factors including social isolation, discrimination, insufficiency, false beliefs, and stigma resistance. The factors with the highest contribution to the stigma level were social isolation and discrimination. In the stigma scores, a significant correlation was found between the age of the patient, frequency of seizures, education status, level of income, and the amount of antiepileptic drugs used. In the analysis of the patients' relatives, the Cronbach's alpha value of the scale was found to be 0.892. In the factor analysis, the questions were classified as discrimination, prejudgments, and false beliefs. The factor which most contributed to the stigma level was discrimination. A significant correlation was found in the stigma scores between sex, education status, marital status, and income distribution. CONCLUSION: According to our study results, it is clearly seen that both patients and their relatives suffer from epilepsy-associated stigma. Patients with epilepsy and their relatives are faced with discrimination in society, resulting in social isolation. We, therefore, believe that both patients and their relatives should be informed in detail about discrimination to overcome this challenge.

Self-esteem in Turkish diabetic children.

Self-esteem is the state of appreciation arising from the ratification of the ego concept that has resulted in self-assessment of their own skills, preferences, and psychologic traits. If people evaluate themselves in positive terms, we say they have high self-esteem. Self-esteem can be evaluated in relation to factors such as family, parents, level of education, age, occupation, and economic status. Children with diabetes mellitus type 1 live with physical, personal, and social developments that occur rapidly and intensively during adolescence. They also must cope with a chronic disease that may have a negative effect on their lives. This study was designed to evaluate the self-esteem of the children who have a diagnosis of diabetes mellitus type 1 and uses a prospective design. The sample consisted of Turkish children between the ages 10 and 14 years old. The study group was selected from the patients admitted to the diabetes outpatient clinics of the hospitals of the university and the Social Insurance Association, and had a diagnosis of diabetes mellitus type 1. The control group was selected from healthy children who were students in a primary school.