Interferon Lambda Family along with HTLV-1 Proviral Load, Tax, and HBZ Implicated in the Pathogenesis of Myelopathy/Tropical Spastic Paraparesis
HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic neuroinflammatory disease related to human T lymphotropic virus type 1 (HTLV-1) infection. Interferon type III (IFN- λ), which includes IL28, IL29, and IL28R, and affects the outcome of viral infections, might be complicated in the progression of HAM/TSP. Here, we investigated the host-virus interactions in the manifestation of HAM/TSP, using IL28B, IL29, IL28R, HTLV-1 Tax, HTLV-1 basic leucine zipper factor (HBZ ), and proviral load (PVL). The study groups consisted of 20 patients with HAM/TSP, 20 asymptomatic HTLV-1 carriers (ACs), an...
Source: Neurodegenerative Diseases - July 10, 2018 Category: Neurology Source Type: research

Novel < b > < i > ABCD1 < /i > < /b > Gene Mutation in Adrenomyeloneuropathy with Hypoplasia and Agenesis of the Corpus Callosum
Conclusion: In addition to the typical symptoms such as spastic myelopathy, cognitive impairment, mixed neuropathy, and alopecia, AMN patients can also display hypoplasia and agenesis of the CC, which was not described in the other AMN patients reported before.Neurodegener Dis (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - July 2, 2018 Category: Neurology Source Type: research

Feasibility of Smartphone-Based Testing of Interference in Parkinson ’s Disease
Conclusions: We established that despite motor impairment, the novel approach of using smartphone technology to test interference in PD patients is feasible.Neurodegener Dis 2018;18:133 –142 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 25, 2018 Category: Neurology Source Type: research

Distinctive Olfactory Pattern in Parkinson ’s Disease and Non-Neurodegenerative Causes of Hyposmia
Conclusions: A differential pattern of hyposmia was observed in PD patients compared to other non-neurodegenerative aetiologies. Further studies with larger samples should replicate our results.Neurodegener Dis (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 25, 2018 Category: Neurology Source Type: research

Neurofilament Subunit L Levels in the Cerebrospinal Fluid and Serum of Patients with Amyotrophic Lateral Sclerosis
Conclusions: NF-L levels increased in CSF and serum of patients with ALS. NF-L may thus be a neurodegenerative biomarker for predicting ALS severity and progression, and the survival of patients with this disease.Neurodegener Dis (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 13, 2018 Category: Neurology Source Type: research

Combinations of Multiple Neuroimaging Markers using Logistic Regression for Auxiliary Diagnosis of Alzheimer Disease and Mild Cognitive Impairment
Conclusion: Using a combination of different indices, the results confirmed the initial hypothesis that different biomarkers were potentially complementary, and thus the conjoint analysis of multiple information from multiple sources would improve the capability to identify diseases such as AD and mild cognitive impairment.Neurodegener Dis 2018;18:91 –106 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 5, 2018 Category: Neurology Source Type: research

Gradual Phenotype Development in Huntington Disease Transgenic Minipig Model at 24 Months of Age
Conclusions: The gradual development of a neurodegenerative phenotype, ac ­companied with testicular degeneration, is observed in 24- month-old TgHD minipigs.Neurodegener Dis 2018;18:107 –119 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 5, 2018 Category: Neurology Source Type: research

Education-Adjusted Normality Thresholds for FDG-PET in the Diagnosis of Alzheimer Disease
Conclusion: These data show that education, as a proxy of reserve, is not a major confounder in the diagnostic accuracy of FDG-PET in AD and the adoption of education-adjusted thresholds is not required in daily practice.Neurodegener Dis 2018;18:120 –126 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 5, 2018 Category: Neurology Source Type: research

Association between White Matter Lesions and Non-Motor Symptoms in Parkinson Disease
Conclusions: Among the non-motor symptoms, fatigue, depression, anxiety, and quality of life were significantly affected by WMLs in PD. Confirmation of the possible role of WMLs in non-motor symptoms associated with PD in a prospective manner may be crucial not only for understanding non-motor symptoms but also for the development of treatment strategies.Neurodegener Dis 2018;18:127 –132 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - June 5, 2018 Category: Neurology Source Type: research

Characteristics of Early Oropharyngeal Dysphagia in Patients with Multiple System Atrophy
Conclusion: Understanding early OD characteristics in patients with MSA and the differences between MSA subtypes could be helpful in managing dysphagia in patients with MSA. Several dysphagia symptoms similar to those of Parkinson disease were frequently observed in MSA-P, but not in MSA-C. A follow-up study is needed to elucidate the natural course of OD in MSA patients and the difference between MSA subtypes.Neurodegener Dis 2018;18:84 –90 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - April 5, 2018 Category: Neurology Source Type: research

Whole-Genome Linkage Analysis with Whole-Exome Sequencing Identifies a Novel Frameshift Variant in < b > < i > NEFH < /i > < /b > in a Chinese Family with Charcot-Marie-Tooth 2: A Novel Variant in < b > < i > NEFH < /i > < /b > for Charcot-Marie-Tooth 2
Conclusions: We identified a novel stop loss variant inNEFH that is likely pathogenic for CMT2, and the results provide further evidence for the role of an aberrant assembly of neurofilament in CMT.Neurodegener Dis 2018;18:74 –83 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - March 27, 2018 Category: Neurology Source Type: research

Cognitive Reserve Hypothesis in Frontotemporal Dementia: Evidence from a Brain SPECT Study in a Series of Greek Frontotemporal Dementia Patients
Conclusion: In the present study, lifetime participation in leisure time activities was found to mitigate the burden of disease in bvFTD and PPA patients. Moreover, FTD patients with a higher educational attainment were able to cope better with greater brain damage. Determination of the most suitable activities to build an adequate level of CR is crucial for dementia prevention.Neurodegener Dis 2018;18:69 –73 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - March 8, 2018 Category: Neurology Source Type: research

Phenotypic Variability in Autosomal Dominant Familial Alzheimer Disease due to the S170F Mutation of Presenilin-1
Conclusion: The variable clinical findings associated with the S170F mutation highlight the relevance of atypical phenotypes in the context of research and under a clinical perspective. CSF sampling and detection of A β species may be essential to indicate AD pathology in unclear cases presenting with cognitive and motor symptoms at a younger age.Neurodegener Dis 2018;18:57-68 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - February 21, 2018 Category: Neurology Source Type: research

Elevated Global DNA Methylation Is Not Exclusive to Amyotrophic Lateral Sclerosis and Is Also Observed in Spinocerebellar Ataxia Types 1 and 2
We report a significant elevation in global 5-mC levels of about 2–7% on average for sALS (p #x3c; 0.01 [F(1, 243) = 9.159,p = 0.0027]) and various forms of fALS along with SCA1 (p #x3c; 0.01 [F(1, 83) = 11.285],p = 0.0012) and SCA2 (p #x3c; 0.001 [F(1, 122) = 29.996,p = 0.0001]) when compared to age- and sex-matched healthy controls.C9orf72 expansion carrier ALS patients exhibit the highest global 5-mC levels along withC9orf72 promoter hypermethylation. We failed to measure global 5-hydroxymethylcytosine (5-hmC) levels in blood, probably due to the very low levels of 5-hmC and the limitations of the commercially ava...
Source: Neurodegenerative Diseases - February 9, 2018 Category: Neurology Source Type: research

Overexpression of SNX3 Decreases Amyloid- β Peptide Production by Reducing Internalization of Amyloid Precursor Protein
Conclusion: These results provide evidence that SNX3 regulates A β production by influencing the internalization of APP.Neurodegener Dis 2018;18:26 –37 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - February 7, 2018 Category: Neurology Source Type: research

Extravascular CD3+ T Cells in Brains of Alzheimer Disease Patients Correlate with Tau but Not with Amyloid Pathology: An Immunohistochemical Study
Conclusions: Our data support the notion of T-cell occurrence in AD brains and suggest that, in advanced stages of AD, T-cell extravasation is driven by tau-related neurodegenerative changes rather than by cerebral amyloidosis. T cells could be crucial for driving the amyloid-independent phase of the AD pathology.Neurodegener Dis 2018;18:49-56 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - February 6, 2018 Category: Neurology Source Type: research

Novel Translational Research Methodology and the Prospect to a Better Understanding of Neurodegenerative Disease
Neurodegener Dis 2018;18:1 –4 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - January 16, 2018 Category: Neurology Source Type: research

Aberrant Connectivity in Mild Cognitive Impairment and Alzheimer Disease Revealed by Multimodal Neuroimaging Data
In this study, 116 MCI, 116 NC and 116 Alzheimer disease (AD) subjects from the Alzheimer's Disease Neuroimaging Initiative were included for the evaluation of the brain covariance graphic model. Sparse inverse covariance estimation was utilized to get the graphic model.Results: The connections among different brain regions were quite different between NC and MCI or between MCI and AD subjects (p (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - January 15, 2018 Category: Neurology Source Type: research

Characteristic Motor and Nonmotor Symptoms Related to Quality of Life in Drug-Na ïve Patients with Late-Onset Parkinson Disease
Background/Aims: Unlike young-onset Parkinson disease (YOPD), characteristics of late-onset PD (LOPD) have not yet been clearly elucidated. We investigated characteristic features and symptoms related to quality of life (QoL) in LOPD patients.Methods: We recruited drug-na ïve, early PD patients. The patient cohort was divided into 3 subgroups based on patient age at onset (AAO): the YOPD group (AAO (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - January 12, 2018 Category: Neurology Source Type: research

Acknowledgement to Reviewers
Neurodegener Dis 2017;17:338 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - December 14, 2017 Category: Neurology Source Type: research

Contents Vol. 17, 2017
Neurodegener Dis 2017;17:I-IV (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - December 14, 2017 Category: Neurology Source Type: research

Subjective Assessment of Sleep in Huntington Disease: Reliability of Sleep Questionnaires Compared to Polysomnography
Discussion: All comparisons between scale scores and PSG results showed poor or totally absent concordance between subjective and objective measures.Significance: The subjective evaluation of sleep in HD patients shows a poor correlation with PSG results.Neurodegener Dis 2017;17:330-337 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 23, 2017 Category: Neurology Source Type: research

Genetic and Pathological Assessment of hnRNPA1, hnRNPA2/B1, and hnRNPA3 in Familial and Sporadic Amyotrophic Lateral Sclerosis
Conclusions: hnRNPA3 pathology was identified in motor neurons of ALS patients with C9orf72 repeat expansions, implicating hnRNPA3 in the pathogenesis ofC9orf72-linked ALS. hnRNPA3 warrants further investigation into the pathogenesis of ALS linked toC9orf72. This study also determined thatHNRNP mutations are not a common cause of FALS and SALS in Australia.Neurodegener Dis 2017;17:304-312 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 10, 2017 Category: Neurology Source Type: research

Health Status Perspectives in Amyotrophic Lateral Sclerosis
Background/Aims: The global perception of the health status (HS) of amyotrophic lateral sclerosis (ALS) patients before the initial diagnosis has not been addressed previously.Methods: We recorded the following at the first visit, before diagnostic information: (1) visual analog scale (VAS) of the EQ-5D; (2) the revised ALS functional rating scale (ALSFRS- R), bulbar (ALSFRSb), upper limb (ALSFRSul), lower limb (ALSFRSll), and respiratory (RofALSFRS-R) subscores; and (3) forced and slow vital capacities. Correlations were tested by the Pearson correlation test. Variables were compared between groups defined by the VAS medi...
Source: Neurodegenerative Diseases - October 30, 2017 Category: Neurology Source Type: research

Huntington's Disease: Premotor Phase
Huntington's disease (HD) is an incurable, neurodegenerative disease, which manifests via a triad of progressive symptoms: motor impairment, psychiatric disorders, and cognitive decline. Conventionally, the HD diagnosis is based on the presence of involuntary choreiform movements and a positive genetic test for the CAG-expanded allele gene. Although the diagnosis focuses on the motor part of the triad, there is increasing evidence that both cognitive and neuropsychiatric symptoms can, and often do, present decades before the onset of motor symptoms. In this paper, we review the evidence regarding the symptoms in the HD pre...
Source: Neurodegenerative Diseases - October 26, 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 - October 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 2017 Category: Neurology Source Type: research

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 - October 25, 2017 Category: Neurology Source Type: research

ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers
Conclusions: We show that in fibroblasts ofFUS P525L mutation carriers, FUS mislocalized to the cytoplasm where it redistributed into stress granules with likely a dose effect, i.e. a higher number of cells with granules, which persist longer, than in controls and ALS cases. These data represent an early molecular change occurring before ALS onset, suggesting a transient preaggregative state.Neurodegener Dis 2017;17:292-303 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - October 25, 2017 Category: Neurology Source Type: research

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