Mild Cognitive Impairment and Progression to Dementia in Progressive Supranuclear Palsy
Conclusion: Cognitive decline occurs in a great proportion of PSP-RS patients early during the disease course. In the absence of a specific phenotype, the diagnosis of MCI might identify PSP patients at greatest risk of developing dementia and should be considered further in the diagnostic assessment.Neurodegener Dis 2017;17:286-291 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - September 7, 2017 Category: Neurology Source Type: research
Two Ethnic Clusters with Huntington Disease in Israel: The Case of Mountain Jews and Karaites
Conclusion: We detected clustering of HD among the population treated at our Medical Center, which has the only specialized HD clinic in the country, with a high percentage of HD among 2 relatively small subpopulations of Jews: Mountain Jews and Karaites.Neurodegener Dis 2017;17:281-285 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - August 24, 2017 Category: Neurology Source Type: research
Gender Differences of Nonmotor Symptoms Affecting Quality of Life in Parkinson Disease
Conclusions: Gender differences of an association between HRQoL and NMSs exist in PD. We found that fatigue and depression were the main determinants of poor HRQoL in female patients even in the early stages. We suggest that a gender-specific therapeutic approach is important, and it is necessary to pay special attention to the predictors associated with causing poor HRQoL.Neurodegener Dis 2017;17:276-280 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - August 24, 2017 Category: Neurology Source Type: research
Cerebral Small Vessel Disease Is Associated with Dysregulation in the Ubiquitin Proteasome System and Other Major Cellular Pathways in Specific Brain Regions
Conclusion: This analysis deciphers brain region-specific molecular processes to increase the present knowledge of SVD pathology and determine new potential therapeutic targets.Neurodegener Dis 2017;17:261-275 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - August 15, 2017 Category: Neurology Source Type: research
Serum Growth Differentiation Factor 15 in Parkinson Disease
Conclusion: GDF15 may be a potential biomarker for the diagnosis and monitoring of motor severity in PD.Neurodegener Dis 2017;17:251-260 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - August 8, 2017 Category: Neurology Source Type: research
Lessons from Anti-Amyloid- β Immunotherapies in Alzheimer Disease: Aiming at a Moving Target
Conclusions: Despite the overall disappointing results, there is still hope that A β immunotherapy in presymptomatic patients will prevent neuronal loss and provide significant clinical benefits that can be applied to larger populations as preventive therapies. Advances with other targets may soon provide additional therapeutic options for AD with increased efficacy.Neurodegener Dis 2017;17:242-250 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - August 8, 2017 Category: Neurology Source Type: research
Frontal Cortex and Hippocampal & #x03B3;-Secretase Activating Protein Levels in Prodromal Alzheimer Disease
Conclusions: These data demonstrate that GSAP proteins are differentially dysregulated in severe AD, but only the full-length form was associated with cognitive test scores in AD.Neurodegener Dis 2017;17:235-241 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - July 25, 2017 Category: Neurology Source Type: research
Serum Interleukin-10 Levels Correlate with Cerebrospinal Fluid Amyloid Beta Deposition in Alzheimer Disease Patients
Conclusion: Our findings indicate that serum levels of IL-10 may represent a possible peripheral expression of amyloid beta deposition in AD patients.Neurodegener Dis 2017;17:227-234 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - July 18, 2017 Category: Neurology Source Type: research
Mitochondrial Metabolism in a Large-Animal Model of Huntington Disease: The Hunt for Biomarkers in the Spermatozoa of Presymptomatic Minipigs
Conclusions: Our results suggest a link between the gain of toxic function of mutated huntingtin in TgHD spermatozoa and the observed MM and/or glycolytic impairment. We determined 4 biomarkers useful for HD phenotyping and experimental therapy monitoring studies in TgHD minipigs.Neurodegener Dis 2017;17:213-226 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 20, 2017 Category: Neurology Source Type: research
SLC25A46 Mutations Associated with Autosomal Recessive Cerebellar Ataxia in North African Families
Conclusion: In this study, we report a novel variant (p.Trp160Ser) inSLC25A46 and we broaden the phenotypic spectrum associated with mutations inSLC25A46.Neurodegener Dis 2017;17:208-212 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - May 30, 2017 Category: Neurology Source Type: research
Exploring Neural Efficiency in Multiple Sclerosis Patients during the Symbol Digit Modalities Test: A Functional Magnetic Resonance Imaging Study
Conclusions: MS patients require more cognitive resources than HCs to achieve a normal SDMT performance, then revealing that they are less efficient regarding IPS capabilities.Neurodegener Dis 2017;17:199-207 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - May 24, 2017 Category: Neurology Source Type: research
A Novel Ataxic Mutant Mouse Line Having Sensory Neuropathy Shows Heavy Iron Deposition in Kidney
Conclusion: The affected mouse was characterized by heavy hind limb ataxia with gait disorder, which was first recognized at about 4 weeks of age and slowly progressed with advancing age. The phenotype was inherited in an autosomal recessive pattern. The genetic locus associated with the phenotype was namedhak and mapped to 107,305,356-108,637,615 on chromosome 2qE3, non-coding sequences in the vicinity ofBdnf gene. Many spheroids were noticed in the cerebellar medulla and the brain stem. In the peripheral nerves, some sensory ganglionic cells showed deposition of NF-200 in the perikaryon and NF-200-positive spheroids in n...
Source: Neurodegenerative Diseases - May 10, 2017 Category: Neurology Source Type: research
Elevated Levels of Selenium Species in Cerebrospinal Fluid of Amyotrophic Lateral Sclerosis Patients with Disease-Associated Gene Mutations
Conclusions: Selenium compounds can impair tubulin synthesis and the cytoskeleton structure, as do tubulin-related gene mutations. The elevated selenium species levels in the TUBA4A patient may have a genetic etiology and/or represent a pathogenic pathway through which this mutation favors disease onset, though unmeasured confounding cannot be excluded. The elevated selenomethionine levels in the other patients are also of interest due to the toxicity of this nonphysiological selenium species. Our study is the first to assess selenium exposure in genetic ALS, suggesting an interaction between this environmental factor and ...
Source: Neurodegenerative Diseases - May 5, 2017 Category: Neurology Source Type: research
Increased Risk of Bullous Pemphigoid after First-Ever Stroke: A Population-Based Study
Background: We hypothesize that autoantibodies are induced after the blood-brain barrier is damaged by stroke and the risk of bullous pemphigoid (BP) is increased after stroke. We assess the risk of BP after first-ever stroke in a nationwide population-based cohort of first-ever stroke patients.Methods: We extracted data from the Longitudinal Health Insurance Database 2005 and identified patients with first-ever stroke as well as control patients matched for age, gender, and year of enrollment. The risk of BP in first-ever stroke patients in comparison with that in control patients was analyzed using Cox regression.Results...
Source: Neurodegenerative Diseases - May 3, 2017 Category: Neurology Source Type: research
