Subjective Cognitive Complaints in Cognitively Normal Patients With Parkinson’s Disease: A Systematic Review

Subjective cognitive complaints (SCCs) refer to self-perceived cognitive decline and are related to objective cognitive decline. SCCs in cognitively normal individuals are considered a preclinical sign of subsequent cognitive impairment due to Alzheimer’s disease, and SCCs in cognitively normal patients with Parkinson’s disease (PD) are also gaining attention. The aim of this review was to provide an overview of the current research on SCCs in cognitively normal patients with PD. A systematic search found a lack of consistency in the methodologies used to define and measure SCCs. Although the association between SCCs and objective cognitive performance in cognitively normal patients with PD is controversial, SCCs appear to be predictive of subsequent cognitive decline. These findings support the clinical value of SCCs in cognitively normal status in PD; however, further convincing evidence from biomarker studies is needed to provide a pathophysiological basis for these findings. Additionally, a consensus on the definition and assessment of SCCs is needed for further investigations.

Glycogen storage disease type Ia misdiagnosed as multiple acyl-coenzyme A dehydrogenase deficiency by mass spectrometry.

Objective: To report a case of glycogen storage disease (GSD) type Ia misdiagnosed as multiple acyl-coenzyme a dehydrogenase deficiency (MADD) by mass spectrometry. Methods: A 7 months old boy was admitted to our hospital for elevated transaminase levels lasting more than 1 month. His blood biochemistry showed hypoglycemia, metabolic acidosis, hyperlipidemia, elevated lactate and uric acid, elevated alanine amino transferase (ALT), aspartate amino transaminase (AST) and gamma-glutamyl transferase (GGT). Mass spectrometry analysis of blood and urine showed elevated blood acylcarnitines and dicarboxylic aciduria, indicating multiple acyl-coenzyme A dehydrogenase deficiency. Sanger sequencing of all exons of glucose-6-phosphatase (G6Pase) and electronic transfer flavoprotein dehydrogenase (ETFDH) was performed for the patient and his parents. Results: Coding and flanking sequences of the G6Pase gene detected two heterozygous single base substitutions in the boy. One variant was in exon 1 (c.209G > A), Which was also detected in the father. Another was in exon 5 (c.648G > T), which was detected in the mother. Coding and flanking sequences of the ETFDH gene revealed no pathogenic/likely pathogenic variants in the boy. Conclusion: GSD Ia can manifest elevated blood acyl carnitines and dicarboxylic aciduria which were the typical clinical manifestations of MADD. So the patient with clinical manifestations similar to MADD is in need of differential diagnosis for GSD Ia. Genetic testing is helpful to confirming the diagnosis of inherited metabolic diseases.

Association Between Body Mass Index and Cognitive Function in Mild Cognitive Impairment Regardless of APOE ε4 Status

Background@#and Purpose: In this study we aimed to find the association between neuropsychological performance and body mass index (BMI) in patients with mild cognitive impairment (MCI). In addition, we investigated the effects of the apolipoprotein E (APOE) genotype in the relationship between the BMI and cognition in MCI. @*Methods@#We enrolled a cohort of 3,038 subjects with MCI aged 65–90 from the Clinical Research Center for Dementia of South Korea and a dementia cohort of the Ewha Womans University Mokdong Hospital. MCI patients were classified into three subgroups according to the Asian standard of BMI. We compared cognitive performances between groups by one-way analysis of variance. To investigate the effects of the APOE genotype, we used multivariate linear regression models after adjusting for possible confounders. @*Results@#Even though normal BMI groups were younger, had more females, and had less comorbidities, the higher BMI groups had better cognitive functions. Among subjects with APOE ε4 carriers, there was a positive relationship between the BMI and the memory task alone. @*Conclusions@#Our findings suggested that higher BMI in patients with MCI were associated with better cognitive performance. The effects of the APOE ε4 genotype in the associations between BMI and cognition were distinguishing. Therefore, according to physical status, APOE ε4 genotype-specific strategies in the assessments and treatments may be necessary in elderly patients with MCI.

Reliability and Validity of the Subjective Cognitive Complaints Questionnaire for Parkinson’s Disease (SCCQ-PD)

Background@#and Purpose Subjective cognitive complaints (SCCs) are gaining attention as a self-perceived symptom for cognitive impairment in patients with Parkinson’s disease (PD), but there are few suitable tools for assessing SCCs in PD. This study aimed to develop and validate a questionnaire for assessing SCCs in PD, called the Subjective Cognitive Complaints Questionnaire for Parkinson’s Disease (SCCQ-PD). @*Methods@#The SCCQ-PD consists of 12 yeso questions on subjective cognitive function, and the questionnaire was completed by patients with PD (score-P) and their caregivers (score-C). The cognitive function of patients was examined using comprehensive neuropsychological tests. @*Results@#This study included 73 patients (38 cognitively normal, 25 with mild cognitive impairment [MCI], and 10 demented) and their caregivers. Score-P and score-C had excellent reliability (Kuder-Richardson formula 20 coefficients of 0.893 and 0.931, respectively), and the scores exhibited a strong intercorrelation. Both score-P and score-C were negatively correlated with cognitive performance, and both were excellent in discriminating demented patients from those with normal cognition or MCI (areas under the receiver operating characteristic curve of 0.83 and 0.88, respectively). @*Conclusions@#The SCCQ-PD is a reliable tool for assessing SCCs in patients with PD. SCCs measured using the SCCQ-PD are correlated with objective cognitive decline and useful for discriminating demented patients from nondemented patients.

Validation of the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale

Background@#and PurposeImpulse-control disorder is an important nonmotor symptom of Parkinson's disease (PD) that can lead to financial and social problems, and be related to a poor quality of life. A nationwide multicenter prospective study was performed with the aim of validating the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale (K-QUIP-RS). @*Methods@#The K-QUIP-RS was constructed using forward and backward translation, and pretesting of the prefinal version. PD patients on stable medical condition were recruited from 27 movement-disorder clinics. Participants were assessed using the K-QUIP-RS and evaluated for parkinsonian motor and nonmotor statuses and for PD-related quality of life using a predefined evaluation battery. The test–retest reliability of the K-QUIP-RS was assessed over an interval of 10–14 days, and correlations between the KQUIP-RS and other clinical scales were analyzed. @*Results@#This study enrolled 136 patients. The internal consistency of the K-QUIP-RS was indicated by a Cronbach's α coefficient of 0.846, as was the test–retest reliability by a Guttman split-half coefficient of 0.808. The total K-QUIP-RS score was positively correlated with the scores for depression and motivation items on the Unified PD Rating Scale (UPDRS), Montgomery-Asberg Depression Scale, and Rapid-Eye-Movement Sleep-Behavior-Disorders Questionnaire. The total K-QUIP-RS score was also correlated with the scores on part II of the UPDRS and the PD Quality of Life-39 questionnaire, and the dopaminergic medication dose. @*Conclusions@#The K-QUIP-RS appears to be a reliable assessment tool for impulse-control and related behavioral disturbances in the Korean PD population.

Subtypes of Sleep Disturbance in Parkinson's Disease Based on the Cross-Culturally Validated Korean Version of Parkinson's Disease Sleep Scale-2

Association of Nutritional Status with Cognitive Stage in the Elderly Korean Population: The Korean Brain Aging Study for the Early Diagnosis and Prediction of Alzheimer's Disease

BACKGROUND AND PURPOSE: Epidemiological studies have suggested the presence of strong correlations among diet, lifestyle, and dementia onset. However, these studies have unfortunately had major limitations due to their inability to fully control the various potential confounders affecting the nutritional status. The purpose of the current study was to determine the nutritional status of participants in the Korean Brain Aging Study for the Early Diagnosis and Prediction of Alzheimer's Disease (KBASE) and to identify clinical risk factors for being at risk of malnutrition or being malnourished. METHODS: Baseline data from 212 participants [119 cognitively unimpaired (CU), 56 with mild cognitive impairment (MCI), and 37 with dementia] included in the KBASE database were analyzed. All participants underwent a comprehensive cognitive test and MRI at baseline. The presence of malnutrition at baseline was measured by the Mini Nutritional Assessment score. We examined the cross-sectional relationships of clinical findings with nutritional status using multiple logistic regression applied to variables for which p<0.2 in the univariate analysis. Differences in cortical thickness according to the nutritional status were also investigated. RESULTS: After adjustment for demographic, nutritional, and neuropsychological factors, participants with dementia had a significantly higher odds ratio (OR) for being at risk of malnutrition or being malnourished than CU participants [OR=5.98, 95% CI=1.20–32.97] whereas participants with MCI did not (OR=0.62, 95% CI=0.20–1.83). Cortical thinning in the at-risk/malnutrition group was observed in the left temporal area. CONCLUSIONS: Dementia was found to be an independent predictor for the risk of malnutrition compared with CU participants. Our findings further suggest that cortical thinning in left temporal regions is related to the nutritional status.

Reduced Uptake on Dopamine Transporter Imaging by Methylphenidate

No abstract available.

Paraneoplastic Chorea Associated with Anti-Hu Antibody and Small Cell Lung Carcinoma

No abstract available.

Validation of the Korean Version of the Scales for Outcomes in Parkinson's Disease-Sleep

BACKGROUND: Sleep problems commonly occur in patients with Parkinson's disease (PD), and are associated with a lower quality of life. The aim of the current study was to translate the English version of the Scales for Outcomes in Parkinson's Disease-Sleep (SCOPA-S) into the Korean version of SCOPA-S (K-SCOPA-S), and to evaluate its reliability and validity for use by Korean-speaking patients with PD. METHODS: In total, 136 patients with PD from 27 movement disorder centres of university-affiliated hospitals in Korea were enrolled in this study. They were assessed using SCOPA, Hoehn and Yahr Scale (HYS), Unified Parkinson's Disease Rating Scale (UPDRS), Parkinson's Disease Sleep Scale 2nd version (PDSS-2), Non-motor Symptoms Scale (NMSS), Montgomery Asberg Depression Scale (MADS), 39-item Parkinson's Disease Questionnaire (PDQ39), Neurogenic Orthostatic Hypotension Questionnaire (NOHQ), and Rapid Eye Movement Sleep Behaviour Disorder Questionnaire (RBDQ). The test-retest reliability was assessed over a time interval of 10–14 days. RESULTS: The internal consistency (Cronbach's α-coefficients) of K-SCOPA-S was 0.88 for nighttime sleep (NS) and 0.75 for daytime sleepiness (DS). Test-retest reliability was 0.88 and 0.85 for the NS and DS, respectively. There was a moderate correlation between the NS sub-score and PDSS-2 total score. The NS and DS sub-scores of K-SCOPA-S were correlated with motor scale such as HYS, and non-motor scales such as UPDRS I, UPDRS II, MADS, NMSS, PDQ39, and NOHQ while the DS sub-score was with RBDQ. CONCLUSION: The K-SCOPA-S exhibited good reliability and validity for the assessment of sleep problems in the Korean patients with PD.

Abnormal Dopamine Transporter Positron Emission Tomography Image Caused by Lesion on Nigrostriatal Tract

No abstract available.

Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans

OBJECTIVE: Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea. METHODS: We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN). RESULTS: Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN. CONCLUSIONS: We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.

Traumatic Basilar Artery Dissection With Acute Pontine Infarction

No abstract available.