Clinicopathological Correlations and Concomitant Pathologies in Rapidly Progressive Dementia: A Brain Bank Series.
CONCLUSIONS: Prion diseases were accurately identified in our series. In contrast, non-prion RPD diagnosis was poor while the patients were still alive, supporting the need for better diagnostic tools and confirmatory neuropathological studies. The presence of concomitant AD pathology in RPD should be taken into account in the interpretation of amyloid biomarkers. PMID: 26523804 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 31, 2015 Category: Neurology Authors: Grau-Rivera O, Gelpi E, Nos C, Gaig C, Ferrer I, Saiz A, Lladó A, Molinuevo JL, Graus F, Sánchez-Valle R, Neurological Tissue Bank Collaborative Group Tags: Neurodegener Dis Source Type: research

Evaluating Behavior in Mouse Models of the Behavioral Variant of Frontotemporal Dementia: Which Test for Which Symptom?
te; F Abstract The behavioral variant of frontotemporal dementia (bvFTD) is a neurodegenerative disease affecting people in their early sixties, characterized by dramatic changes in individual and social behavior. Despite the heterogeneity in the presentation of the clinical symptoms of bvFTD, some characteristic changes can be highlighted. Social disinhibition, changes in food preferences as well as loss of empathy and apathy are commonly described. This is accompanied by a characteristic and dramatic atrophy of the prefrontal cortex with the accumulation of protein aggregates in the neurons in this area. Several...
Source: Neuro-Degenerative Diseases - October 31, 2015 Category: Neurology Authors: Vernay A, Sellal F, René F Tags: Neurodegener Dis Source Type: research

Parkinson's Disease Case Ascertainment in the EPIC Cohort: The NeuroEPIC4PD Study.
CONCLUSIONS: This exercise proved that it is feasible to ascertain PD in large population-based cohort studies and offers a potential framework to be replicated in similar studies. PMID: 26375921 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - September 17, 2015 Category: Neurology Authors: Gallo V, Brayne C, Forsgren L, Barker RA, Petersson J, Hansson O, Lindqvist D, Ruffmann C, Ishihara L, Luben R, Arriola L, Bergareche A, Gavrila D, Erro ME, Vanacore N, Sacerdote C, Bueno-de-Mesquita B, Vermeulen R, Seelen M, Sieri S, Masala G, Ramat S, K Tags: Neurodegener Dis Source Type: research

Uncoupling of Protein Aggregation and Neurodegeneration in a Mouse Amyotrophic Lateral Sclerosis Model.
Abstract Aberrant accumulation of protein aggregates is a pathological hallmark of many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Although a buildup of protein aggregates frequently leads to cell death, whether it is the key pathogenic factor in driving neurodegenerative disease remains controversial. HDAC6, a cytosolic ubiquitin-binding deacetylase, has emerged as an important regulator of ubiquitin-dependent quality control autophagy, a lysosome-dependent degradative system responsible for the disposal of misfolded protein aggregates and damaged organelles. Here, we show that in ...
Source: Neuro-Degenerative Diseases - September 12, 2015 Category: Neurology Authors: Lee JY, Kawaguchi Y, Li M, Kapur M, Choi SJ, Kim HJ, Park SY, Zhu H, Yao TP Tags: Neurodegener Dis Source Type: research

Breakdown of Sensorimotor Network Communication in Leukoaraiosis.
CONCLUSIONS: These results suggest that LA impairs sensorimotor integration by interfering with the communication or coordination of these aforementioned regions related to the SMN. PMID: 26287381 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 20, 2015 Category: Neurology Authors: Wu X, Lai Y, Zhang Y, Yao L, Wen X Tags: Neurodegener Dis Source Type: research

Erratum.
Authors: PMID: 26227205 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - August 3, 2015 Category: Neurology Tags: Neurodegener Dis Source Type: research

The Big Bluff of Amyotrophic Lateral Sclerosis Diagnosis: The Role of Neurodegenerative Disease Mimics.
Abstract Neurodegenerative diseases include a significant number of pathologies affecting the nervous system. Generally, the primary cause of each disease is specific; however, recently, it was shown that they may be correlated at molecular level. This aspect, together with the exhibition of similar symptoms, renders the diagnosis of these disorders difficult. Amyotrophic lateral sclerosis is one of these pathologies. Herein, we report several cases of amyotrophic lateral sclerosis misdiagnosed as a consequence of features that are common to several neurodegenerative diseases, such as Parkinson's, Huntington's and...
Source: Neuro-Degenerative Diseases - July 30, 2015 Category: Neurology Authors: Bicchi I, Emiliani C, Vescovi A, Martino S Tags: Neurodegener Dis Source Type: research

Imaging in Glucocerebrosidase-Associated Parkinsonism: Current Status and Implications for Pathophysiology.
CONCLUSIONS: Further imaging studies conducted in a broader population of early GBA-PD are warranted to characterize the disease and elucidate the pathophysiological mechanisms underlying GBA-PD and to understand GBA implications in iPD. © 2015 S. Karger AG, Basel. PMID: 26227893 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 28, 2015 Category: Neurology Authors: Kiferle L, Giuntini M, Bonuccelli U, Ceravolo R Tags: Neurodegener Dis Source Type: research

Cognitive Impairment and Its Structural Correlates in the Parkinsonian Subtype of Multiple System Atrophy.
CONCLUSIONS: Our data demonstrate that cortical and cerebellar atrophy and striatal degeneration are associated with cognitive impairment in patients with MSA-P. © 2015 S. Karger AG, Basel. PMID: 26202063 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 21, 2015 Category: Neurology Authors: Kim JS, Yang JJ, Lee DK, Lee JM, Youn J, Cho JW Tags: Neurodegener Dis Source Type: research

An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies.
CONCLUSION: Our findings support the hypothesis that ALS and its variants could comprise a larger neuropathological continuum. © 2015 S. Karger AG, Basel. PMID: 26183171 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 16, 2015 Category: Neurology Authors: Coan G, Mitchell CS Tags: Neurodegener Dis Source Type: research

Application of the Bead-Based Technique in Neurodegeneration: A Literature Review.
CONCLUSION: Review of literature data shows that the combined analysis of classical CSF biomarkers with novel biomarkers might increase the specificity and sensitivity of performed tests. However, we concluded that the replacement of conventional ELISA with the bead-based technique requires new reference intervals for Aβ1-42, T-tau and P-tau concentrations. © 2015 S. Karger AG, Basel. PMID: 26184922 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 16, 2015 Category: Neurology Authors: Koper OM, Kamińska J, Kemona H, Dymicka-Piekarska V Tags: Neurodegener Dis Source Type: research

Anticholinergic Activity and Alzheimer's Disease.
PMID: 26138489 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Hori K, Hachisu M, Tomioka H, Konishi K Tags: Neurodegener Dis Source Type: research

Serum Anticholinergic Activity as an Index of Anticholinergic Activity Load in Alzheimer's Disease.
We reported a procedure of serum anticholinergic activity (SAA) measurement and the reliability and reproducibility of the receptor binding assay, and we also described the usefulness of SAA measurement reflecting the anticholinergic activity (AA) in the central nervous system (CNS). According to the results of a 10 times repeated measurement of standard atropine binding, the relative error was between -5.5 and +3.7%, and we considered that measurement of SAA in our studies is accurate and validated. Downregulation of acetylcholine activates inflammation in both CNS and peripheral tissue, which causes AA in both sites. The...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Hachisu M, Konishi K, Hosoi M, Tani M, Tomioka H, Kitajima Y, Inamoto A, Hirata A, Koganemaru T, Tomita A, Akashi N, Hori K Tags: Neurodegener Dis Source Type: research

Does Anticholinergic Activity Affect Neuropathology? Implication of Neuroinflammation in Alzheimer's Disease.
Abstract One characteristic neuropathological feature of Alzheimer's disease (AD) is profound neuronal loss in the nucleus basalis of Meynert, the major source of cholinergic innervation of the cerebral cortex. Clinically, anticholinergic activity causes a decline in cognitive function and increases the risk of dementia, thus possibly enhancing AD pathologies and neurodegeneration. Until now there has been insufficient human neuropathological data to support this conclusion. Experimental studies using a tauopathy mouse model demonstrated anticholinergics enhanced tau pathology and neurodegeneration corresponding t...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Yoshiyama Y, Kojima A, Itoh K, Isose S, Koide M, Hori K, Arai K Tags: Neurodegener Dis Source Type: research

Hypothesis of Endogenous Anticholinergic Activity in Alzheimer's Disease.
Abstract In this article, we review and repropose our hypothesis of the endogenous appearance of anticholinergic activity (AA) in Alzheimer's disease (AD). First, we introduce our previous articles and speculate that, because acetylcholine (ACh) regulates both cognitive function and inflammation, downregulation of this neurotransmitter causes upregulation of the inflammatory system. AA then appears endogenously with the production of cytokines and the downregulation of ACh in AD. To support our hypothesis, we present a female AD patient whose AA was considered to occur endogenously through her AD pathology. Her se...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Konishi K, Hori K, Tani M, Tomioka H, Kitajima Y, Akashi N, Inamoto A, Kurosawa K, Yuda H, Hanashi T, Ouchi H, Hosoi M, Hachisu M Tags: Neurodegener Dis Source Type: research

Pharmacotherapy for Neurocognitive Disorders Based on the Hypothesis of Endogenous Appearance of Anticholinergic Activity.
Abstract We previously proposed the hypothesis of endogenous anticholinergic activity (AA) in Alzheimer's disease (AD). According to this hypothesis, the downregulation of acetylcholine seen in AD is associated with upregulation/hyperactivity of N-methyl-D-aspartate receptor (NMDAR). The hyperactivation of NMDAR then induces inflammation, which, in turn, causes AA to appear endogenously. Based on this hypothesis, we commented that cholinesterase inhibitors (ChEIs) are 'preventative' therapy for AD and NMDAR antagonists are the true 'treatment' for AD. We also noted that ChEIs, such as donepezil, could treat deliri...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Hosoi M, Hori K, Konishi K, Tani M, Tomioka H, Kitajima Y, Akashi N, Inamoto A, Kurosawa K, Yuda H, Hanashi T, Ouchi H, Hachisu M Tags: Neurodegener Dis Source Type: research

A Review of the Role of Anticholinergic Activity in Lewy Body Disease and Delirium.
Abstract We have previously proposed a hypothesis in which we argue that anticholinergic activity (AA) appears endogenously in Alzheimer's disease (AD). Acetylcholine (ACh) controls both cognitive function and inflammation. Consequently, when the downregulation of ACh reaches critical levels, the inflammatory system is upregulated and proinflammatory cytokines with AA appear. However, factors other than downregulation of ACh can produce AA; even if ACh downregulation does not reach critical levels, AA can still appear if one of these other AA-producing factors is added. These factors can include neurocognitive dis...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Kitajima Y, Hori K, Konishi K, Tani M, Tomioka H, Akashi N, Hosoi M, Inamoto A, Hasegawa S, Kikuchi N, Takahashi A, Hachisu M Tags: Neurodegener Dis Source Type: research

Anticholinergic Activity and Schizophrenia.
Abstract In this article, we review the downregulation of acetylcholinergic activity in schizophrenia and discuss the similarity and difference between Alzheimer's disease (AD) and schizophrenia in terms of acetylcholine (ACh) and anticholinergic activity (AA); then, we propose the use of cognition-enhancing therapy for schizophrenia. As ACh regulates an inflammatory system, when the cholinergic system is downregulated to a critical level, the inflammatory system is activated. We consider the possibility that AA appears endogenously in AD and accelerates AD pathology. This hypothesis can also be applied to schizop...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Tani M, Akashi N, Hori K, Konishi K, Kitajima Y, Tomioka H, Inamoto A, Hirata A, Tomita A, Koganemaru T, Takahashi A, Hachisu M Tags: Neurodegener Dis Source Type: research

Demonstrating the Role of Anticholinergic Activity in a Mood Disorder.
We report a case of a 54-year-old woman presenting with amnesia, apathy, work-related difficulties and mental stress. At presentation, her Mini-Mental State Examination score was 27 and her serum anticholinergic activity (SAA) was positive without medication or recent physical illnesses. In addition, magnetic resonance imaging revealed mild atrophy of the frontal and temporal lobes, with a relatively intact hippocampus. Consequently, we diagnosed mild cognitive impairment due to Alzheimer's disease and prescribed a cholinesterase inhibitor (donepezil, 10 mg/day); her SAA fully disappeared and clinical symptoms partially re...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Hori K, Konishi K, Hanashi T, Tani M, Tomioka H, Kitajima Y, Akashi N, Inamoto A, Kurosawa K, Hasegawa S, Izuno T, Kikuchi N, Hosoi M, Hachisu M Tags: Neurodegener Dis Source Type: research

Beyond the Hypothesis of Serum Anticholinergic Activity in Alzheimer's Disease: Acetylcholine Neuronal Activity Modulates Brain-Derived Neurotrophic Factor Production and Inflammation in the Brain.
Abstract The brain of Alzheimer's disease (AD) patients is characterized by neurodegeneration, especially an acetylcholine (ACh) neuronal deficit with accumulation of β-amyloid protein, which leads to oxygen stress and inflammation. The active oxygen directly damages the neuron by increasing intracellular Ca(2+). The inflammation is due to activation of the microglia, thereby producing cytokines which inhibit the production of brain-derived neurotrophic factor (BDNF). As the BDNF acts by neuronal protection, synaptogenesis and neurogenesis, the reduction of BDNF in the brain of AD patients worsens the symptom...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Hachisu M, Konishi K, Hosoi M, Tani M, Tomioka H, Inamoto A, Minami S, Izuno T, Umezawa K, Horiuchi K, Hori K Tags: Neurodegener Dis Source Type: research

Plasma Cholinesterase Activity in Alzheimer's Disease.
Abstract Cholinesterase inhibitors (ChEIs) are not allowed to be prescribed in combination, which means that we need to select 1 of 3 ChEIs for use in a patient with Alzheimer's disease (AD). However, there is no quantitative analysis on the differences between these agents. In this article, we propose that plasma cholinesterase activity (pChE) could be used as the standard for differentiating between rivastigmine (Riv) and donepezil (Don) in the management of AD. To date, we have treated 6 patients with Riv 18 mg and 5 patients with Don 5 mg. The pChE is related to low-grade inflammation associated with AD, diabe...
Source: Neuro-Degenerative Diseases - July 5, 2015 Category: Neurology Authors: Hosoi M, Hori K, Konishi K, Tani M, Tomioka H, Kitajima Y, Akashi N, Inamoto A, Minami S, Izuno T, Umezawa K, Horiuchi K, Hachisu M Tags: Neurodegener Dis Source Type: research

Influence of Lentiviral β-Synuclein Overexpression in the Hippocampus of a Transgenic Mouse Model of Alzheimer's Disease on Amyloid Precursor Protein Metabolism and Pathology.
CONCLUSION: The initially reported beneficial effects of β-Syn could be partially reproduced, but locally elevated levels of β-Syn might also cause neurodegeneration. To enlighten the controversial pathological mechanism of β-Syn, further examinations considering the relationship between concentration and exposure time of β-Syn are needed. © 2015 S. Karger AG, Basel. PMID: 26111745 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 25, 2015 Category: Neurology Authors: Krassnig S, Schweinzer C, Taub N, Havas D, Auer E, Flunkert S, Schreibmayer W, Hutter-Paier B, Windisch M Tags: Neurodegener Dis Source Type: research

Combined Treatment of an Amyotrophic Lateral Sclerosis Rat Model with Recombinant GOT1 and Oxaloacetic Acid: A Novel Neuroprotective Treatment.
CONCLUSION: In this study we bring evidence that the administration of Glu scavengers to rats with sporadic ALS inhibited the massive death of spinal cord motor neurons, slowed the onset of motor weakness and prolonged survival. This treatment may be of high clinical significance for the future treatment of chronic neurodegenerative diseases. © 2015 S. Karger AG, Basel. PMID: 26113413 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - June 23, 2015 Category: Neurology Authors: Ruban A, Cohen-Kashi Malina K, Cooper I, Graubardt N, Babakin L, Jona G, Teichberg VI Tags: Neurodegener Dis Source Type: research

Downregulation of β-Soluble N-Ethylmaleimide-Sensitive Factor Attachment Protein: Proteomics-Based Identification in Early-Stage Prion Disease.
Downregulation of β-Soluble N-Ethylmaleimide-Sensitive Factor Attachment Protein: Proteomics-Based Identification in Early-Stage Prion Disease. Neurodegener Dis. 2015 May 22; Authors: Lin Z, Zhao D, Wang Y, Zhao W, Yin X, Zhou X, Zhang Z, Yang L Abstract Prion diseases are known as neurodegenerative diseases of the central nervous system with a long incubation period. Alzheimer's disease (AD) and prion diseases share the hallmark of severe neuronal loss, although their pathogenic mechanisms are similarly incomplete. It appears that these two neurodegenerative diseases share a complex deterioratio...
Source: Neuro-Degenerative Diseases - May 22, 2015 Category: Neurology Authors: Lin Z, Zhao D, Wang Y, Zhao W, Yin X, Zhou X, Zhang Z, Yang L Tags: Neurodegener Dis Source Type: research

The Evaluation of Abnormal Voice Qualities in Patients with Amyotrophic Lateral Sclerosis.
CONCLUSIONS: Analysis of voice qualities among patients with ALS allows for the detection of various abnormalities associated with the natural progression of the disease. © 2015 S. Karger AG, Basel. PMID: 25967115 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - May 7, 2015 Category: Neurology Authors: Tomik J, Tomik B, Wiatr M, Składzień J, Stręk P, Szczudlik A Tags: Neurodegener Dis Source Type: research

Overexpression of BDNF and Full-Length TrkB Receptor Ameliorate Striatal Neural Survival in Huntington's Disease.
CONCLUSION: The results highlight the importance of BDNF-induced TrkB receptor signaling in rescuing HD-mediated apoptotic features in striatal cells. © 2015 S. Karger AG, Basel. PMID: 25896770 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 21, 2015 Category: Neurology Authors: Silva A, Naia L, Dominguez A, Ribeiro M, Rodrigues J, Vieira OV, Lessmann V, Rego AC Tags: Neurodegener Dis Source Type: research

The Epidemiology of Amyotrophic Lateral Sclerosis in New Hampshire, USA, 2004-2007.
CONCLUSION: While localized areas in N.H. with high ALS incidence rates have been reported previously, the overall incidence and mortality rates of ALS in N.H. are similar to those in other industrialized nations. © 2015 S. Karger AG, Basel. PMID: 25896575 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 16, 2015 Category: Neurology Authors: Caller TA, Andrews A, Field NC, Henegan PL, Stommel EW Tags: Neurodegener Dis Source Type: research

The Relationship between Age-Related Macular Degeneration and Olfactory Function.
CONCLUSION: This study demonstrated that AMD had significant negative effects on all orthonasal olfactory tasks, particularly in advanced stages. Similar to other neurodegenerative diseases, odor discrimination and identification seemed to be more affected than odor detection threshold tasks. © 2015 S. Karger AG, Basel. PMID: 25871947 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 11, 2015 Category: Neurology Authors: Kar T, Yildirim Y, Altundağ A, Sonmez M, Kaya A, Colakoglu K, Tekeli H, Cayonu M, Hummel T Tags: Neurodegener Dis Source Type: research

Dysfunctional Dopaminergic Neurones in Mouse Models of Huntington's Disease: A Role for SK3 Channels.
CONCLUSIONS: Dopaminergic neuronal dysfunction is a key early event in HD disease progression. The initial increase in dopamine release appears to be related to a loss of SK3 channel function, a protein containing a polyglutamine tract. Implications for polyglutamine-mediated sequestration of SK3 channels, dopamine-associated DNA damage and CAG expansion are discussed in the context of HD. © 2015 S. Karger AG, Basel. PMID: 25871323 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 9, 2015 Category: Neurology Authors: Dallérac GM, Levasseur G, Vatsavayai SC, Milnerwood AJ, Cummings DM, Kraev I, Huetz C, Evans KA, Walters SW, Rezaie P, Cho Y, Hirst MC, Murphy KP Tags: Neurodegener Dis Source Type: research

Genetic Variation of MT-ND Genes in Frontotemporal Lobar Degeneration: Biochemical Phenotype-Genotype Correlation.
CONCLUSION: To our knowledge, this is the first report of the complete sequence of the MT-ND genes in FTLD patients and correlation with MRC activity. The high number of mtDNA variations identified and a significant decrease in complex I activity suggest a possible involvement of mtDNA alterations in FTLD. Although the majority of these alterations are not primarily pathogenic, an interaction with other mutations may occur, leading to the disease, worsening its expression or influencing age of onset. © 2015 S. Karger AG, Basel. PMID: 25871488 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 3, 2015 Category: Neurology Authors: Gaspar R, Santana I, Mendes C, Fernandes AS, Duro D, Simões M, Luís D, Santos MJ, Grazina M Tags: Neurodegener Dis Source Type: research

Mechanisms, Clinical Strategies, and Promising Treatments of Neurodegenerative Diseases. 12th International Conference AD/PD Nice, France, March 18-22, 2015: Abstracts.
Authors: PMID: 25824864 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - April 2, 2015 Category: Neurology Tags: Neurodegener Dis Source Type: research

Histological Bulbar Manifestations in the ALS Rat.
CONCLUSION: These findings suggest that, from a histological standpoint, the SOD1-G93A rat is a valid model of ALS bulbar symptoms. © 2015 S. Karger AG, Basel. PMID: 25825172 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - March 24, 2015 Category: Neurology Authors: Kashlan ON, Kashlan BN, Oh SS, McGinley LM, Chen KS, Kupfer R, Erman A, Sakowski SA, Feldman EL Tags: Neurodegener Dis Source Type: research

Abnormal Cochlear Potentials in Friedreich's Ataxia Point to Disordered Synchrony of Auditory Nerve Fiber Activity.
CONCLUSION: These findings suggest that underlying auditory neuropathy in FRDA is a disordered synchrony in auditory nerve fiber discharge, possibly resulting from auditory nerve fiber degeneration and inner hair cell loss. © 2015 S. Karger AG, Basel. PMID: 25791504 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - March 17, 2015 Category: Neurology Authors: Santarelli R, Cama E, Pegoraro E, Scimemi P Tags: Neurodegener Dis Source Type: research

Erratum.
Authors: PMID: 25732510 [PubMed - in process] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - March 6, 2015 Category: Neurology Tags: Neurodegener Dis Source Type: research

Antecedent Disease Is Less Prevalent in Amyotrophic Lateral Sclerosis.
Conclusions: The prevalence of antecedent disease was overall less in the ALS population. We present two potential lines of inquiry to explain these results: (1) 'Other disease as ALS protection' - antecedent diseases infer biochemical neuroprotection to ALS; (2) 'ALS as other disease protection' - the underpinnings of ALS could infer protection to other diseases, possibly via the mechanism hypervigilant regulation or 'too-high' regulatory feedback gains. © 2015 S. Karger AG, Basel. PMID: 25720304 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - February 20, 2015 Category: Neurology Authors: Mitchell CS, Hollinger SK, Goswami SD, Polak MA, Lee RH, Glass JD Tags: Neurodegener Dis Source Type: research

Natural History of Parkinson's Disease in the Province of Segovia: Disability in a 20 Years Longitudinal Study.
Conclusions: Patients with Parkinson's disease evolve gradually toward disability, and prognostic factors of this evolution were identified. © 2015 S. Karger AG, Basel. PMID: 25613012 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 22, 2015 Category: Neurology Authors: Duarte J, García Olmos LM, Mendoza A, Martínez Pueyo Á, Clavería LE Tags: Neurodegener Dis Source Type: research

An Astrocyte Regenerative Response from Vimentin-Containing Cells in the Spinal Cord of Amyotrophic Lateral Sclerosis's Disease-Like Transgenic (G93A SOD1) Mice.
Abstract The reason for regeneration in the adult spinal cord during motor neuron degeneration in amyotrophic lateral sclerosis (ALS) remains largely unknown. To this end, we studied the alteration of vimentin (a neural precursor cells marker in CNS)-containing cells (VCCs) in spinal cord during different stages of ALS used C57BL/6J G93A SOD1 transgenic mice mimicking ALS. Results showed that VCCs were mostly distributed in the ependymal zone (EZ) surrounding the central canal of spinal cord in SOD1 wild type mice; a few of VCCs were sparsely distributed in other regions. However, the number of VCCs significantly ...
Source: Neuro-Degenerative Diseases - January 15, 2015 Category: Neurology Authors: Zhou Y, Lu Y, Fang X, Zhang J, Li J, Li S, Deng X, Yu Y, Xu R Tags: Neurodegener Dis Source Type: research

Role of Immunity and Inflammation in the Pathophysiology of Neurodegenerative Diseases.
Abstract Neurodegenerative diseases are the result of progressive loss of neurons and axons in the central nervous system (CNS), which can lead to cognition and motor dysfunction. It is well known that CNS inflammation and immune activation play a major role in the pathophysiology of neurodegenerative diseases. Although the blood-brain barrier (BBB) is able to protect the CNS from immune activation, it becomes more permeable during inflammation, which renders the brain vulnerable to infections. A better understanding of the interaction between inflammatory mediators, such as cytokines, and the activated immune res...
Source: Neuro-Degenerative Diseases - January 15, 2015 Category: Neurology Authors: Fakhoury M Tags: Neurodegener Dis Source Type: research

Is There a Role for DAT-SPECT Imaging in a Specialty Movement Disorders Practice?
Conclusions: Diagnostic uncertainty in cases of parkinsonism exists even in a tertiary referral center. DAT-SPECT has significant impact on clinical diagnosis and management even in the hands of movement disorders specialists. © 2015 S. Karger AG, Basel. PMID: 25592727 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 15, 2015 Category: Neurology Authors: Bega D, Gonzalez-Latapi P, Zadikoff C, Spies W, Simuni T Tags: Neurodegener Dis Source Type: research

Souvenaid Reduces Behavioral Deficits and Improves Social Cognition Skills in Frontotemporal Dementia: A Proof-of-Concept Study.
Conclusions: Our results provide evidence of the potential of Souvenaid™ therapy for the treatment of behavioral disturbances and social cognition skills in FTD. © 2015 S. Karger AG, Basel. PMID: 25592742 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 15, 2015 Category: Neurology Authors: Pardini M, Serrati C, Guida S, Mattei C, Abate L, Massucco D, Sassos D, Amore M, Krueger F, Cocito L, Emberti Gialloreti L Tags: Neurodegener Dis Source Type: research

Brain Amyloid-Beta Fragment Signatures in Pathological Ageing and Alzheimer's Disease by Hybrid Immunoprecipitation Mass Spectrometry.
Conclusion: Senile plaques in AD may have an Aβ fragment composition distinct from PA with more N-terminally and pyroglutamate-modified Aβ peptides that may be linked to neurotoxicity. © 2015 S. Karger AG, Basel. PMID: 25591542 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - January 13, 2015 Category: Neurology Authors: Portelius E, Lashley T, Westerlund A, Persson R, Fox NC, Blennow K, Revesz T, Zetterberg H Tags: Neurodegener Dis Source Type: research

Amyloid-β Immunotherapy Reduces Amyloid Plaques and Astroglial Reaction in Aged Domestic Dogs.
Conclusion: The anti-Aβ immunotherapy developed in our laboratory modifies the equilibrium between soluble and insoluble Aβ in aged dogs in close correlation with S100B-negative astrocytosis and microglial reaction. © 2014 S. Karger AG, Basel. PMID: 25531153 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 20, 2014 Category: Neurology Authors: Neus Bosch M, Pugliese M, Andrade C, Gimeno-Bayón J, Mahy N, Rodriguez MJ Tags: Neurodegener Dis Source Type: research

Is Schizophrenia a Neurodegenerative Disease? Evidence from Age-Related Decline of Brain-Derived Neurotrophic Factor in the Brains of Schizophrenia Patients and Matched Nonpsychiatric Controls.
Conclusion: PFC BDNF does not follow a normative linear age effect in schizophrenia patients as they grow older, which may represent a 'floor effect' due to earlier decline or a survivor cohort of older patient donors who are less susceptible to a schizophrenia-related pathological aging process. © 2014 S. Karger AG, Basel. PMID: 25531449 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 17, 2014 Category: Neurology Authors: Rao J, Chiappelli J, Kochunov P, Regenold WT, Rapoport SI, Hong LE Tags: Neurodegener Dis Source Type: research

The Parkinson's Disease-Related Protein DJ-1 Protects Dopaminergic Neurons in vivo and Cultured Cells from Alpha-Synuclein and 6-Hydroxydopamine Toxicity.
Conclusion: DJ-1 appears to have a protective role against dopaminergic degeneration triggered by α-syn or 6-OHDA, reinforcing the possible therapeutic importance of this protein in PD. © 2014 S. Karger AG, Basel. PMID: 25500798 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 11, 2014 Category: Neurology Authors: Batelli S, Invernizzi RW, Negro A, Calcagno E, Rodilossi S, Forloni G, Albani D Tags: Neurodegener Dis Source Type: research

Anti-Neuronal Antibodies in Patients with Fragile X Syndrome: Is there a Role of Autoimmunity in Its Pathogenesis?
Conclusion: Serum anti-neuronal antibodies were found in a subgroup of FXS patients. Autistic symptoms in FXS may, in part, be caused by auto-immune factors. Further studies in larger patient and control groups are necessary to elucidate the aetiopathogenic role of anti-neuronal antibodies in FXS patients. © 2014 S. Karger AG, Basel. PMID: 25500855 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 11, 2014 Category: Neurology Authors: Lisik MZ, Gutmajster E, Sieroń AL Tags: Neurodegener Dis Source Type: research

The Effect of TOMM40 Poly-T Repeat Lengths on Age of Onset and Cerebrospinal Fluid Biomarkers in Finnish Alzheimer's Disease Patients.
Conclusion: We conclude that the poly-T repeat associations of rs10524523 in TOMM40 reflect the APOE ε4-dependent association in LOAD. © 2014 S. Karger AG, Basel. PMID: 25500937 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - December 4, 2014 Category: Neurology Authors: Helisalmi S, Hall A, Meriläinen EH, Väisänen V, Koivisto AM, Herukka SK, Laitinen M, Soininen H, Hiltunen M Tags: Neurodegener Dis Source Type: research

UCH-L1 S18Y Variant and Risk of Parkinson's Disease in Asian Populations: An Updated Meta-Analysis.
Abstract The ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) gene has been implicated in the etiology of Parkinson's disease (PD). Several studies have evaluated the association between the UCH-L1 S18Y variant and the risk of PD. However, conflicting results have been reported. Therefore, we performed a meta-analysis to explore whether the UCH-L1 S18Y variant is associated with susceptibility to PD in Asians. A database search in Web of Science, MEDLINE, Embase and PubMed up to March 2014 identified 10 studies comprising 4,897 PD patients and 4,446 controls in total, which were included in this meta-analysis. In ...
Source: Neuro-Degenerative Diseases - December 2, 2014 Category: Neurology Authors: Zhu R, Zhu Y, Liu X, He Z Tags: Neurodegener Dis Source Type: research

Failure of Ubiquitin Proteasome System: Risk for Neurodegenerative Diseases.
Abstract The ubiquitin proteasome system (UPS) is the primary proteolytic quality control system in cells and has an essential function in the nervous system. UPS dysfunction has been linked to neurodegenerative conditions, including Alzheimer's, Parkinson's and Huntington's diseases. The pathology of neurodegenerative diseases is characterized by the abnormal accumulation of insoluble protein aggregates or inclusion bodies within neurons. The failure or dysregulation of the UPS prevents the degradation of misfolded/aberrant proteins, leading to deficient synaptic function that eventually affects the nervous syste...
Source: Neuro-Degenerative Diseases - November 20, 2014 Category: Neurology Authors: Zheng C, Geetha T, Babu JR Tags: Neurodegener Dis Source Type: research

Frontal Corpus Callosum Alterations in Progressive Supranuclear Palsy but Not in Parkinson's Disease.
Conclusion: DTI identified a PSP-associated microstructural alteration pattern in the frontal lobes and in the CC area II including the corresponding bilateral callosal radiation tracts that could not be identified in both control samples, supporting the prominent PSP-associated frontal involvement as a potential neuroimaging marker. © 2014 S. Karger AG, Basel. PMID: 25377379 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - November 1, 2014 Category: Neurology Authors: Rosskopf J, Müller HP, Huppertz HJ, Ludolph AC, Pinkhardt EH, Kassubek J Tags: Neurodegener Dis Source Type: research

Mutation and Clinical Characteristics of Autosomal-Dominant Hereditary Spastic Paraplegias in China.
Conclusions: Our data confirm that hereditary spastic paraplegias in China represent a heterogeneous group of genetic neurodegenerative disorders in autosomal-dominant and apparently sporadic forms. Novel genotype-phenotype correlations were established. © 2014 S. Karger AG, Basel. PMID: 25341883 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
Source: Neuro-Degenerative Diseases - October 22, 2014 Category: Neurology Authors: Luo Y, Chen C, Zhan Z, Wang Y, Du J, Hu Z, Liao X, Zhao G, Wang J, Yan X, Jiang H, Pan Q, Xia K, Tang B, Shen L Tags: Neurodegener Dis Source Type: research