Hippocampal Volume Loss, Brain Amyloid Accumulation, and APOE Status in Cognitively Intact Elderly Subjects.
CONCLUSION: The progressive decrement of neuropsychological performance is associated with HVL long before the emergence of clinically overt symptoms. In this cohort of healthy individuals, the presence of the APOE-ε4 allele was shown to be an independent predictor of worst hippocampal integrity in asymptomatic cases independently of amyloid positivity. PMID: 31846965 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 17, 2019 Category: Neurology Authors: Haller S, Montandon ML, Rodriguez C, Garibotto V, Herrmann FR, Giannakopoulos P Tags: Neurodegener Dis Source Type: research

Is 123I-MIBG Scintigraphy Beneficial or Excessive for the Diagnosis of Parkinson's Disease in the Early Phase?
Abstract INTRODUCTION/OBJECTIVE: In most cases, abnormal cardiac 123I-meta-iodobenzylguanidine (MIBG) scintigraphy increases the probability of a diagnosis of Parkinson's disease (PD) in patients with parkinsonian features. In our study, we validated the additional value of 123I-MIBG scintigraphy beyond providing information on neurological findings and response to dopaminergic therapy for the diagnosis of PDin the early phase. METHODS: We investigated 77 cases of PD (Hoehn and Yahr Stages I-III) and 73 cases of atypical parkinsonian disorder (APD), including 35 patients with multiple system atrophy, 19 with ...
Source: Neuro-Degenerative Diseases - November 12, 2019 Category: Neurology Authors: Ikeda T, Ikenoshita S, Sakamoto F, Shiraishi S, Nakahara K, Masuda T, Yamashita S Tags: Neurodegener Dis Source Type: research

Assessment of the Corticospinal Tract Profile in Pure Lower Motor Neuron Disease: A Diffusion Tensor Imaging Study.
CONCLUSIONS: Our study demonstrates that the CST was impaired in both ALS and pLMND patients, thus suggesting that DTI metrics are a reliable tool in detecting subtle changes of UMN in pLMND patients, also in the absence of clinical and CMCT abnormalities. PMID: 31715609 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - November 12, 2019 Category: Neurology Authors: Sarica A, Valentino P, Nisticò R, Barone S, Pucci F, Quattrone A, Cerasa A, Quattrone A Tags: Neurodegener Dis Source Type: research

Poor Diet, Stress, and Inactivity Converge to Form a "Perfect Storm" That Drives Alzheimer's Disease Pathogenesis.
Poor Diet, Stress, and Inactivity Converge to Form a "Perfect Storm" That Drives Alzheimer's Disease Pathogenesis. Neurodegener Dis. 2019 Oct 10;:1-18 Authors: Pacholko AG, Wotton CA, Bekar LK Abstract North American incidence of Alzheimer's disease (AD) is expected to more than double over the coming generation. Although genetic factors surrounding the production and clearance of amyloid-β and phosphorylated tau proteins are known to be responsible for a subset of early-onset AD cases, they do not explain the pathogenesis of the far more prevalent sporadic late-onset variant of the dis...
Source: Neuro-Degenerative Diseases - October 10, 2019 Category: Neurology Authors: Pacholko AG, Wotton CA, Bekar LK Tags: Neurodegener Dis Source Type: research

Unusual Segregation of APP Mutations in Monogenic Alzheimer Disease.
We report on a 40-year-old woman with a severe cognitive decline starting at 36 years, while her affected relatives presented symptoms onset in the 6th decade. The proband carried an APP missense variant in homozygous state (NM_000484.4: c.2032G>A; NP_000475.1: p.Asp678Asn; rs63750064) and showed a more severe clinical picture than the other AD relatives, as regards the age of onset and the rate of disease progression. This mutation behaves as a semi-dominant trait. The very rare chance of studying APP mutations in the homozygous state demonstrates they are not always dominant and other segregation models are possible. ...
Source: Neuro-Degenerative Diseases - October 2, 2019 Category: Neurology Authors: Mastromoro G, Gambardella S, Marchionni E, Campopiano R, Traversa A, Di Bonaventura C, Pizzuti A Tags: Neurodegener Dis Source Type: research

A Narrative Review of Lifestyle Factors Associated with Parkinson's Disease Risk and Progression.
Abstract Parkinson's disease is a complex slowly progressive neurodegenerative disorder with motor and non-motor symptoms affecting daily living. Despite effective symptomatic treatments, with various degrees of side effects, no disease-modifying therapeutic options presently exist. Symptoms progress, with an accumulating burden, reducing the quality of life and forming the impression that medications are no longer effective. Adopting positive lifestyle behaviours can empower patients, improve the quality of life, alleviate symptoms, and potentially slow disease progression. Lifestyle behaviours including nutritio...
Source: Neuro-Degenerative Diseases - September 5, 2019 Category: Neurology Authors: Nag N, Jelinek GA Tags: Neurodegener Dis Source Type: research

Differential Changes in Functional Connectivity of Striatum-Prefrontal and Striatum-Motor Circuits in Premanifest Huntington's Disease.
Abstract BACKGROUND: Huntington's disease (HD) is a progressive neurodegenerative disorder. The striatum is one of the first brain regions that show detectable atrophy in HD. Previous studies using functional magnetic resonance imaging (fMRI) at 3 tesla (3 T) revealed reduced functional connectivity between striatum and motor cortex in the prodromal period of HD. Neuroanatomical and neurophysiological studies have suggested segregated corticostriatal pathways with distinct loops involving different cortical regions, which may be investigated using fMRI at an ultra-high field (7 T) with enhanced sensitivity compare...
Source: Neuro-Degenerative Diseases - August 14, 2019 Category: Neurology Authors: Kronenbuerger M, Hua J, Bang JYA, Ultz KE, Miao X, Zhang X, Pekar JJ, van Zijl PCM, Duan W, Margolis RL, Ross CA Tags: Neurodegener Dis Source Type: research

Associations of Neprilysin Activity in CSF with Biomarkers for Alzheimer's Disease.
CONCLUSIONS: Our results on CSF-NEP are compatible with the assumption that local degradation, amongst other mechanisms of amyloid clearance, plays a role in the development of Alzheimer's pathology. In addition, CSF-NEP is associated with the extent and the rate of neurodegeneration. PMID: 31266021 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 2, 2019 Category: Neurology Authors: Grimmer T, Goldhardt O, Yakushev I, Ortner M, Sorg C, Diehl-Schmid J, Förstl H, Kurz A, Perneczky R, Miners S Tags: Neurodegener Dis Source Type: research

Novel and Recurring NOTCH3 Mutations in Two Chinese Patients with CADASIL.
CONCLUSIONS: We identified 2 NOTCH3 mutations as likely genetic causes for CADASIL in these 2 patients. Our findings broaden the mutational spectrum of the NOTCH3 gene accountable for CADASIL. Clinical manifestations supplemented with molecular genetic analyses are critical for accurate diagnosis, the provision of genetic counseling, and the development of therapies for CADASIL. PMID: 31212292 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 18, 2019 Category: Neurology Authors: Chen X, Deng S, Xu H, Hou D, Hu P, Yang Y, Wen J, Deng H, Yuan L Tags: Neurodegener Dis Source Type: research

Age-Related Oxidative Changes in Primary Porcine Fibroblasts Expressing Mutated Huntingtin.
CONCLUSIONS: Our results unravel phenotypic alterations in primary fibroblasts isolated from the TgHD minipig model at the age of 48 months. Importantly, nDNA damage appears to precede these phenotypic alterations. Our results highlight the impact of fibroblasts from TgHD minipigs in studying the molecular mechanisms of HD pathophysiology that gradually occur with age. PMID: 31167196 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 5, 2019 Category: Neurology Authors: Smatlikova P, Askeland G, Vaskovicova M, Klima J, Motlik J, Eide L, Ellederová Z Tags: Neurodegener Dis Source Type: research

"A Battle You Would Never Choose to Fight": The Management of Neurodegenerative Diseases as a Societal Challenge.
"A Battle You Would Never Choose to Fight": The Management of Neurodegenerative Diseases as a Societal Challenge. Neurodegener Dis. 2019 Jun 04;:1-3 Authors: Steffen J PMID: 31163425 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 4, 2019 Category: Neurology Authors: Steffen J Tags: Neurodegener Dis Source Type: research

Psychosocial Impact of Dysarthria: The Patient-Reported Outcome as Part of the Clinical Management.
CONCLUSIONS: All patients reported a communication complaint, attested by the DIP scores, despite the fact that not all patients, notably PD, ataxic, and PSP patients, had an intelligibility deficit. The DIP should be used in clinical practice to contribute to a holistic evaluation and management of functional communication in patients with dysarthria. PMID: 31112944 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - May 21, 2019 Category: Neurology Authors: Atkinson-Clement C, Letanneux A, Baille G, Cuartero MC, Véron-Delor L, Robieux C, Berthelot M, Robert D, Azulay JP, Defebvre L, Ferreira J, Eusebio A, Moreau C, Pinto S Tags: Neurodegener Dis Source Type: research

Physician and Surrogate Agreement with Assisted Dying and Continuous Deep Sedation in Advanced Dementia in Switzerland.
CONCLUSIONS: The surrogates were more agreeable to considering assisted dying in the setting of advanced dementia than the physicians, and about half of the participants in both groups reported CDS to be an appropriate option for this population. PMID: 31013507 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 23, 2019 Category: Neurology Authors: Loizeau AJ, Cohen SM, Mitchell SL, Theill N, Eicher S, Martin M, Riese F Tags: Neurodegener Dis Source Type: research

Role of MAPT in Pure Motor Neuron Disease: Report of a Recurrent Mutation in Italian Patients.
Abstract The aim of our study was to evaluate the role of mutations in the MAPT gene in patients with pure amyotrophic lateral sclerosis (ALS). A cohort of 120 ALS patients, both sporadic and familial, without cognitive impairment was analyzed by next-generation sequencing with a multiple-gene panel comprising 23 genes, including MAPT, known to be associated with ALS and frontotemporal dementia. The presence of the C9orf72 expansion was also investigated. Twelve patients had mutations in the SOD1, TARDBP, MATR3, and FUS genes, while 10 patients carried the C9orf72 expansion. One female patient was found to carry t...
Source: Neuro-Degenerative Diseases - March 20, 2019 Category: Neurology Authors: Origone P, Geroldi A, Lamp M, Sanguineri F, Caponnetto C, Cabona C, Gotta F, Trevisan L, Bellone E, Manganelli F, Devigili G, Mandich P Tags: Neurodegener Dis Source Type: research

Association between Primary Open-Angle Glaucoma and Cognitive Impairment as Measured by the Montreal Cognitive Assessment.
CONCLUSIONS: POAG cases and controls had similar neurocognitive function as measured by the MoCA. Among POAG cases, worse VF loss was associated with lower MoCA. Future studies are needed to further elucidate the clinical effect of neuropathy in POAG. PMID: 30893703 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - March 20, 2019 Category: Neurology Authors: McCoskey M, Addis V, Goodyear K, Sankar PS, Ying GS, Yu Y, Salowe R, Cui QN, Miller-Ellis E, Maguire M, O Apos Brien JM Tags: Neurodegener Dis Source Type: research

The Precuneus - A Witness for Excessive A β Gathering in Alzheimer's Disease Pathology.
The Precuneus - A Witness for Excessive Aβ Gathering in Alzheimer's Disease Pathology. Neurodegener Dis. 2019 Feb 27;18(5-6):302-309 Authors: Aghakhanyan G, Vergallo A, Gennaro M, Mazzarri S, Guidoccio F, Radicchi C, Ceravolo R, Tognoni G, Bonuccelli U, Volterrani D Abstract Evidence of cortical beta-amyloid (Aβ) load, assessed by Aβ positron emission tomography (Aβ-PET), is an established in vivo biomarker of Alzheimer's disease (AD)-related pathophysiology. Qualitative assessment of Aβ-PET provides binary information; meanwhile semiquantitative approaches require a parcellat...
Source: Neuro-Degenerative Diseases - February 27, 2019 Category: Neurology Authors: Aghakhanyan G, Vergallo A, Gennaro M, Mazzarri S, Guidoccio F, Radicchi C, Ceravolo R, Tognoni G, Bonuccelli U, Volterrani D Tags: Neurodegener Dis Source Type: research

Non-Invasive Brain Stimulation in Dementia: A Complex Network Story.
Abstract Non-invasive brain stimulation (NIBS) is emerging as a promising rehabilitation tool for a number of neurodegenerative diseases. However, the therapeutic mechanisms of NIBS are not completely understood. In this review, we will summarize NIBS results in the context of brain imaging studies of functional connectivity and metabolites to gain insight into the possible mechanisms underlying recovery. We will briefly discuss how the clinical manifestations of common neurodegenerative disorders may be related with aberrant connectivity within large-scale neural networks. We will then focus on recent studies com...
Source: Neuro-Degenerative Diseases - January 29, 2019 Category: Neurology Authors: Pini L, Manenti R, Cotelli M, Pizzini FB, Frisoni GB, Pievani M Tags: Neurodegener Dis Source Type: research

The Role of MRI Biomarkers and Their Interactions with Cognitive Status and APOE ε4 in Nondemented Elderly Subjects.
The Role of MRI Biomarkers and Their Interactions with Cognitive Status and APOE ε4 in Nondemented Elderly Subjects. Neurodegener Dis. 2019 Jan 23;18(5-6):270-280 Authors: Liang X, Yin Z, Liu R, Zhao H, Wu S, Lu J, Qing Z, Wei Y, Yang Q, Zhu B, Xu Y, Zhang B Abstract PURPOSE: (1) To investigate atrophy patterns of hippocampal subfield volume and Alzheimer's disease (AD)-signature cortical thickness in mild cognitive impairment (MCI) patients; (2) to explore the association between the neuropsychological (NP) and the brain structure in the MCI and older normal cognition group; (3) to determine ...
Source: Neuro-Degenerative Diseases - January 23, 2019 Category: Neurology Authors: Liang X, Yin Z, Liu R, Zhao H, Wu S, Lu J, Qing Z, Wei Y, Yang Q, Zhu B, Xu Y, Zhang B Tags: Neurodegener Dis Source Type: research

Evaluation of D1/D5 Partial Agonist PF-06412562 in Parkinson's Disease following Oral Administration.
Abstract BACKGROUND: PF-06412562 is a moderately potent, highly selective oral D1/D5 dopamine receptor partial agonist. OBJECTIVE: To study the efficacy and safety of a single, oral, split dose of PF-06412562 in patients with Parkinson's disease. METHODS: Following overnight levodopa (L-dopa, Sinemet®) washout, subjects received a single dose of levodopa in open-label period 1. Periods 2 and 3 had a double-blinded, sponsor-open, randomized, 2-way cross-over, placebo-controlled design, during which subjects were randomized to PF-06412562 30 mg (+ 20 mg 4 h later) or placebo. Maximum percent improvemen...
Source: Neuro-Degenerative Diseases - November 19, 2018 Category: Neurology Authors: Papapetropoulos S, Liu W, Duvvuri S, Thayer K, Gray DL Tags: Neurodegener Dis Source Type: research

Cerebrospinal Fluid Neurofilaments May Discriminate Upper Motor Neuron Syndromes: A Pilot Study.
Abstract BACKGROUND: Patients presenting with upper motor neuron (UMN) signs may widely diverge in prognosis, ranging from amyotrophic lateral sclerosis (ALS) to primary lateral sclerosis (PLS) and hereditary spastic paraplegia (hSP). Neurofilaments are emerging as potential diagnostic and prognostic biomarkers for ALS, but the diagnosis of UMN syndromes still relies mostly on clinical long-term observation and on familiarity or genetic confirmation. OBJECTIVES: To test whether phosphorylated neurofilament heavy chain (pNfH) may discriminate different UMN syndromes at diagnosis and to test their prognostic ro...
Source: Neuro-Degenerative Diseases - November 14, 2018 Category: Neurology Authors: Zucchi E, Bedin R, Fasano A, Fini N, Gessani A, Vinceti M, Mandrioli J Tags: Neurodegener Dis Source Type: research

C9orf72 Repeat Expansion Frequency among Patients with Huntington Disease Genetic Testing.
Abstract BACKGROUND: European studies identified the C9orf72 repeat expansion as the most frequent genetic alteration in patients with Huntington disease (HD)-like phenotypes but negative HD genetic testing. OBJECTIVE: To investigate C9orf72 repeat expansion frequency in individuals tested for HD in a North American tertiary referral laboratory. METHODS: Three hundred and seventy-three cases (115 positive and 258 negative for HD) were evaluated by genotyping PCR, with follow-up Southern blot and 5' repeat methylation status assessment by combined repeat-primed and methylation-specific PCR in a subset. ...
Source: Neuro-Degenerative Diseases - October 18, 2018 Category: Neurology Authors: Ida CM, Butz ML, Lundquist PA, Dawson DB Tags: Neurodegener Dis Source Type: research

Cortical Thinning Associated with Age and CSF Biomarkers in Early Parkinson's Disease Is Modified by the SNCA rs356181 Polymorphism.
ulisevsky J Abstract The role of cerebrospinal fluid (CSF) biomarkers such as CSF α-synuclein and CSF tau in predicting cognitive decline in Parkinson's disease (PD) continues to be inconsistent. Here, using a cohort of de novo PD patients with preserved cognition from the Parkinson's Progression Markers Initiative (PPMI), we show that the SNCA rs356181 single nucleotide polymorphism (SNP) modulates the effect of these CSF biomarkers on cortical thinning. Depending on this SNP's genotype, cortical atrophy was associated with either higher or lower CSF biomarker levels. Additionally, this SNP modified age-rel...
Source: Neuro-Degenerative Diseases - October 18, 2018 Category: Neurology Authors: Sampedro F, Marín-Lahoz J, Martínez-Horta S, Pagonabarraga J, Kulisevsky J Tags: Neurodegener Dis Source Type: research

Critical Review of Complementary and Alternative Medicine Use in Amyotrophic Lateral Sclerosis: Prevalence and Users' Profile, Decision-Making, Information Seeking, and Disclosure in the Face of a Lack of Efficacy.
CONCLUSION: A proportion of ALS patients report utilizing CAM concurrently with conventional treatments. Such use, set amidst a dearth of evidence for the efficacy of CAM in ALS, poses potential direct and indirect risks to patient care, and medical providers should be mindful of and enquire about CAM use when treating ALS patients. PMID: 30248676 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 24, 2018 Category: Neurology Authors: Adams J, Lee M, Peng W Tags: Neurodegener Dis Source Type: research

Prevalence of Apolipoprotein E Polymorphisms in Alzheimer's Disease, Mild Cognitive Impairment, and Healthy Elderly: A Northern Greece Study.
CONCLUSION: In Greece, APOE ε4 presence is related to an increased risk for AD in a dose-related manner. Contrary to long-standing views, men and women with the APOE ε4 genotype have nearly the same odds of developing MCI and AD. PMID: 30205398 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 11, 2018 Category: Neurology Authors: Tsolaki AC, Gatzima O, Daniilidou M, Lazarou E, Bamidis PD, Verykouki E, Iakovidou-Kritsi Z, Tsolaki M Tags: Neurodegener Dis Source Type: research

Evaluation of Chitotriosidase and CC-Chemokine Ligand 18 as Biomarkers of Microglia Activation in Amyotrophic Lateral Sclerosis.
CONCLUSIONS: High ChT activity in CSF of patients with ALS may reflect microglia activation and could be a potential biomarker of the disease. We did not find any significant difference regarding CCL-18, another specific marker of microglia activation that is related with M2-like microglia phenotype. Deepening the understanding of the activation state of microglia (M1 and M2) may contribute to the knowledge about the specific role of neuroinflammation in ALS and future therapeutic strategies. PMID: 30134252 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 22, 2018 Category: Neurology Authors: Martinez-Merino L, Iridoy M, Galbete A, Roldán M, Rivero A, Acha B, Irún P, Canosa C, Pocoví M, Mendioroz M, Jericó I Tags: Neurodegener Dis Source Type: research

Relationship between Liver Pathology and Disease Progression in a Murine Model of Amyotrophic Lateral Sclerosis.
CONCLUSIONS: Taken together, these findings suggest that liver dysfunction in hSOD1G93A transgenic mice is mediated by increased inflammation and oxidative stress as well as the upregulation of fibrosis-related proteins. PMID: 30130789 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 21, 2018 Category: Neurology Authors: Lee SH, Yang EJ Tags: Neurodegener Dis Source Type: research

Estimating the Evolution of Disease in the Parkinson's Progression Markers Initiative.
In this study, we apply a latent time joint mixed-effects model to study biomarker progression and disease dynamics in the Parkinson's Progression Markers Initiative (PPMI) and examine which markers might be most informative in the earliest phases of disease. The results reveal that, even though diagnostic category was not included in the model, it seems to accurately reflect the temporal ordering of the disease state consistent with diagnosis categorization at baseline. In addition, results indicated that the specific binding ratio on striatum and the total Unified Parkinson's Disease Rating Scale (UPDRS) show high discri...
Source: Neuro-Degenerative Diseases - August 8, 2018 Category: Neurology Authors: Iddi S, Li D, Aisen PS, Rafii MS, Litvan I, Thompson WK, Donohue MC Tags: Neurodegener Dis Source Type: research

Gene Co-Expression Network Analysis Implicates microRNA Processing in Parkinson's Disease Pathogenesis.
CONCLUSION: These results suggest that microRNA processing and function may play a role in the pathogenesis of PD, and thus may represent a useful target for future drug development. PMID: 30089309 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 8, 2018 Category: Neurology Authors: Chen JA Tags: Neurodegener Dis Source Type: research

Interferon Lambda Family along with HTLV-1 Proviral Load, Tax, and HBZ Implicated in the Pathogenesis of Myelopathy/Tropical Spastic Paraparesis.
Abstract 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 ca...
Source: Neuro-Degenerative Diseases - July 10, 2018 Category: Neurology Authors: Mozhgani SH, Jahantigh HR, Rafatpanah H, Valizadeh N, Mohammadi A, Basharkhah S, Rezaee SA Tags: Neurodegener Dis Source Type: research

Novel ABCD1 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. PMID: 29966135 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 2, 2018 Category: Neurology Authors: Qiu Y, Xin L, Wang Y, Yu Y, Zou K, Zhou Q, Chen Y, Chen S, Zhu M, Hong D Tags: Neurodegener Dis Source Type: research

Distinctive Olfactory Pattern in Parkinson's Disease and Non-Neurodegenerative Causes of Hyposmia.
Abstract BACKGROUND: Olfactory dysfunction is common in Parkinson's disease (PD). The characteristics of the hyposmia in PD have not been well defined. OBJECTIVE: To characterize the pattern of the olfactory deficit in PD and in other non-neurodegenerative aetiologies of hyposmia. METHODS: We evaluated 36 PD patients, 20 patients with hyposmia secondary to acute respiratory infection (ARI), and 19 patients with hyposmia secondary to traumatic brain injury (TBI). For comparison purposes, we included a group of 15 controls age and sex matched with PD patients. PD patients were classified based on disease d...
Source: Neuro-Degenerative Diseases - June 25, 2018 Category: Neurology Authors: Crespo Cuevas AM, Ispierto L, Vilas D, Planas A, Planas A, Isern I, Sanchez J, De Haro J, Alvarez R Tags: Neurodegener Dis Source Type: research

Feasibility of Smartphone-Based Testing of Interference in Parkinson's Disease.
Abstract BACKGROUND: Interference refers to learned associations and established behaviors "interfering" with response to new material. It forms a core pillar of executive functions, which are commonly affected in Parkinson's disease (PD). Cognitive interference test (CIT) forms part of a smartphone application designed for ambulatory assessment in PD. OBJECTIVE: The aims of this study were to establish that CIT could effectively demonstrate interference and would perform comparably to the Stroop Color-Word Test Victoria version (VST) despite PD-related motor impairment. METHODS: Ninety-nine pa...
Source: Neuro-Degenerative Diseases - June 25, 2018 Category: Neurology Authors: Lee W, Williams DR, Evans A Tags: Neurodegener Dis 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. PMID: 29898446 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 13, 2018 Category: Neurology Authors: Gong ZY, Lv GP, Gao LN, Lu Y, Guo J, Zang DW Tags: Neurodegener Dis 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. PMID: 29870975 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 5, 2018 Category: Neurology Authors: Lee JY, Kim JS, Jang W, Park J, Oh E, Youn J, Park S, Cho JW Tags: Neurodegener Dis 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. PMID: 29870978 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 5, 2018 Category: Neurology Authors: Mao N, Liu Y, Chen K, Yao L, Wu X Tags: Neurodegener Dis 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. PMID: 29870995 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 5, 2018 Category: Neurology Authors: Vidinská D, Vochozková P, Šmatlíková P, Ardan T, Klíma J, Juhás Š, Juhásová J, Bohuslavová B, Baxa M, Valeková I, Motlík J, Ellederová Z Tags: Neurodegener Dis 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. PMID: 29870998 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 5, 2018 Category: Neurology Authors: Mainta IC, Trombella S, Morbelli S, Frisoni GB, Garibotto V, Alzheimer Disease Neuroimaging Initiative (ADNI) Tags: Neurodegener Dis 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. PMID: 29621788 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 5, 2018 Category: Neurology Authors: Lee HH, Seo HG, Kim KD, Lee SH, Lee WH, Oh BM, Lee WW, Kim Y, Kim A, Kim HJ, Jeon B, Han TR Tags: Neurodegener Dis Source Type: research

Whole-Genome Linkage Analysis with Whole-Exome Sequencing Identifies a Novel Frameshift Variant in NEFH in a Chinese Family with Charcot-Marie-Tooth 2: A Novel Variant in NEFH for Charcot-Marie-Tooth 2.
CONCLUSIONS: We identified a novel stop loss variant in NEFH that is likely pathogenic for CMT2, and the results provide further evidence for the role of an aberrant assembly of neurofilament in CMT. PMID: 29587262 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - March 27, 2018 Category: Neurology Authors: Bian X, Lin P, Li J, Long F, Duan R, Yuan Q, Li Y, Gao F, Gao S, Wei S, Li X, Sun W, Gong Y, Yan C, Liu Q Tags: Neurodegener Dis 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. PMID: 29514157 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - March 7, 2018 Category: Neurology Authors: Maiovis P, Ioannidis P, Gerasimou G, Gotzamani-Psarrakou A, Karacostas D Tags: Neurodegener Dis 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 Aβ species may be essential to indicate AD pathology in unclear cases presenting with cognitive and motor symptoms at a younger age. PMID: 29466804 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 22, 2018 Category: Neurology Authors: Tiedt HO, Benjamin B, Niedeggen M, Lueschow A Tags: Neurodegener Dis 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
Source: Neuro-Degenerative Diseases - February 9, 2018 Category: Neurology Authors: Hamzeiy H, Savaş D, Tunca C, Şen NE, Gündoğdu Eken A, Şahbaz I, Calini D, Tiloca C, Ticozzi N, Ratti A, Silani V, Başak AN Tags: Neurodegener Dis 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 Aβ production by influencing the internalization of APP. PMID: 29414832 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 7, 2018 Category: Neurology Authors: Xu S, Nigam SM, Brodin L Tags: Neurodegener Dis 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. PMID: 29402847 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 7, 2018 Category: Neurology Authors: Merlini M, Kirabali T, Kulic L, Nitsch RM, Ferretti MT Tags: Neurodegener Dis Source Type: research

Novel Translational Research Methodology and the Prospect to a Better Understanding of Neurodegenerative Disease.
PMID: 29339665 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 16, 2018 Category: Neurology Authors: Unschuld PG Tags: Neurodegener Dis 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: Neuro-Degenerative Diseases - January 13, 2018 Category: Neurology Authors: Li Q, Wu X, Xie F, Chen K, Yao L, Zhang J, Guo X, Li R, and Alzheimer's Disease Neuroimaging Initiative Tags: Neurodegener Dis Source Type: research

Characteristic Motor and Nonmotor Symptoms Related to Quality of Life in Drug-Na ïve Patients with Late-Onset Parkinson Disease.
Characteristic Motor and Nonmotor Symptoms Related to Quality of Life in Drug-Naïve Patients with Late-Onset Parkinson Disease. Neurodegener Dis. 2018 Jan 12;18(1):19-25 Authors: Park HR, Youn J, Cho JW, Oh ES, Kim JS, Park S, Jang W, Park JS Abstract 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-naïve, early PD patients. The patient cohort was divided into 3 subgro...
Source: Neuro-Degenerative Diseases - January 12, 2018 Category: Neurology Authors: Park HR, Youn J, Cho JW, Oh ES, Kim JS, Park S, Jang W, Park JS Tags: Neurodegener Dis Source Type: research

Contents Vol. 17, 2017.
Authors: PMID: 29241187 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 15, 2017 Category: Neurology Tags: Neurodegener Dis Source Type: research

Acknowledgement to Reviewers.
Authors: PMID: 29241188 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 15, 2017 Category: Neurology Tags: Neurodegener Dis 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. PMID: 29169178 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - November 24, 2017 Category: Neurology Authors: Piano C, Della Marca G, Losurdo A, Imperatori C, Solito M, Calandra-Buonaura G, Provini F, Cortelli P, Bentivoglio AR Tags: Neurodegener Dis Source Type: research