Voltage-Gated Potassium Channel Antibodies in Slow-Progression Motor Neuron Disease
Conclusion: Although VGKC-complex Abs are not likely to be pathogenic, these results could reflect the coexistence of an immunological activation in patients with slow disease progression.Neurodegener Dis 2017;17:59-62 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - October 6, 2016 Category: Neurology Source Type: research
Clinical Impact of 123I-Ioflupane SPECT (DaTscan) in a Movement Disorder Center
Background/Aims: The clinical diagnosis of degenerative forms of parkinsonism is imperfect, with past studies reporting a high rate of misdiagnosis by neurologists and movement disorder specialists, particularly early in the disease course.123I-ioflupane SPECT (DaTscan) is a diagnostic neuroimaging tool used to distinguish essential tremor from tremor due to degenerative parkinsonisms. The present study expands upon prior studies of the clinical impact of DaTscan imaging in movement disorder centers by assessing quantitative estimates of diagnostic certainty, the impact on subsequent clinical decisions, and the degree to w...
Source: Neurodegenerative Diseases - September 12, 2016 Category: Neurology Source Type: research
Brain-Derived Neurotrophic Factor Facilitates Functional Recovery from ALS-Cerebral Spinal Fluid-Induced Neurodegenerative Changes in the NSC-34 Motor Neuron Cell Line
Conclusion: Our study provides evidence that BDNF supplementation ameliorates most but not all degenerative changes. The incomplete revival at the ultrastructural level signifies the requirement of factors other than BDNF for near-total protection of motor neurons, and, to an extent, it explains why only a partial success is achieved in clinical trials with BDNF in ALS patients.Neurodegener Dis 2017;17:44-58 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - September 12, 2016 Category: Neurology Source Type: research
Clinical Impact of < sup > 123 < /sup > I-Ioflupane SPECT (DaTscan) in a Movement Disorder Center
Background/Aims: The clinical diagnosis of degenerative forms of parkinsonism is imperfect, with past studies reporting a high rate of misdiagnosis by neurologists and movement disorder specialists, particularly early in the disease course.123I-ioflupane SPECT (DaTscan) is a diagnostic neuroimaging tool used to distinguish essential tremor from tremor due to degenerative parkinsonisms. The present study expands upon prior studies of the clinical impact of DaTscan imaging in movement disorder centers by assessing quantitative estimates of diagnostic certainty, the impact on subsequent clinical decisions, and the degree to w...
Source: Neurodegenerative Diseases - September 9, 2016 Category: Neurology Source Type: research
Differential Progression of Midbrain Atrophy in Parkinsonism: Longitudinal MRI Study
Conclusions: Midbrain atrophy progresses differentially in patients with PSP-RS, PSP-P, and PD. Longitudinal measurements of midbrain atrophy using MRI-based visual rating indexes can help distinguish patients with PSP-P from those with PSP-RS and PD.Neurodegener Dis 2017;17:31-37 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - September 9, 2016 Category: Neurology Source Type: research
Novel Blood Biomarkers Are Associated with White Matter Lesions in Fragile X- Associated Tremor/Ataxia Syndrome
Conclusion: The dramatic increase in mitochondrial activity in lymphoblasts from PM carriers may represent either the early stages of disease (specific alterations in short-lived blood cells) or an activation of the lymphocytes under pathological situations. These changes may provide early, convenient blood biomarkers of clinical involvements.Neurodegener Dis 2017;17:22-30 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - September 7, 2016 Category: Neurology Source Type: research
The Prognostic Role of Obstructive Sleep Apnea at the Onset of Amyotrophic Lateral Sclerosis
Background/Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive loss of central and peripheral motor neurons. Some studies have found discordant data in the presence of sleep apnea in ALS patients. An obstructive component also occurs with upper airways hypotonia and muscle weakness that may result in an excessive reduction of airway lumen, leading to obstructive sleep apnea (OSA). The aim of this study was to assess the role of obstructive apneic events at disease onset in the ALS prognosis.Methods: A longitudinal retrospective study was conducted on 42 clinically ...
Source: Neurodegenerative Diseases - September 5, 2016 Category: Neurology Source Type: research
< b > < i > C9orf72 < /i > < /b > Hexanucleotide Repeat Analysis in Cases with Pathologically Confirmed Dementia with Lewy Bodies
Conclusion: Our data illustrate thatC9orf72 screening of clinically diagnosed DLB patients should only be considered in cases with a family history of motor neuron disease or frontotemporal dementia to distinguish between mimic diseases.Neurodegener Dis 2016;16:370-372 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - September 2, 2016 Category: Neurology Source Type: research
Novel Compound Heterozygous < b > < i > Spatacsin < /i > < /b > Mutations in a Greek Kindred with Hereditary Spastic Paraplegia SPG11 and Dementia
SPG11 belongs to the autosomal recessive hereditary spastic paraplegias (HSP) and presents during childhood or puberty with a complex clinical phenotype encompassing learning difficulties, ataxia, peripheral neuropathy, amyotrophy, and mental retardation. We hereby present the case of a 30-year-old female patient with complex autosomal recessive HSP with thinning of the corpus callosum (TCC) and dementia that was compound heterozygous with two novel mutations in theSPG11 gene. Sequence analysis of theSPG11gene revealed two novel mutations in a compound heterozygous state in the index patient (c.2431C>T/p.Gln811Ter and c.67...
Source: Neurodegenerative Diseases - September 2, 2016 Category: Neurology Source Type: research
Contents Vol. 16, 2016
Neurodegener Dis 2016;16:I-IV (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - September 2, 2016 Category: Neurology Source Type: research
Granulocyte Colony-Stimulating Factor Ameliorates Skeletal Muscle Dysfunction in Amyotrophic Lateral Sclerosis Mice and Improves Proliferation of SOD1-G93A Myoblasts in vitro
Conclusion: Our results support the beneficial role of the G-CSF analog PEGF in a SOD1-G93A model of ALS. Thus, G-CSF andits analogs may be directly beneficial in diseases where the SKM function is compromised.Neurodegener Dis 2017;17:1-13 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - August 19, 2016 Category: Neurology Source Type: research
Exploring Olfactory Function and Its Relation with Behavioral and Cognitive Impairment in Amyotrophic Lateral Sclerosis Patients: A Cross-Sectional Study
Background: Behavioral and cognitive impairment are common in amyotrophic lateral sclerosis (ALS) and represent a continuum with frontotemporal dementia (FTD). Olfactory dysfunction has been described in a subset of ALS patients and might be associated with frontotemporal and insular cortex dysfunction.Objective: To evaluate olfaction dysfunction in ALS patients and its relationship with either cognition or behavioral impairment.Methods: 28 consecutive ALS patients underwent an extensive cognitive and behavioral battery and were classified as patients with normal cognition (ALS-N, n = 11) or with part of the ALS-FTD spectr...
Source: Neurodegenerative Diseases - August 5, 2016 Category: Neurology Source Type: research
Using High-Field Magnetic Resonance Imaging to Estimate Distensibility of the Middle Cerebral Artery
Conclusion: < /i > < /b > These results lead to the first noninvasive image-based estimation of distensibility of the MCA (approx. 5.8 × 10 < sup > -4 < /sup > mm Hg < sup > -1 < /sup > ) and demonstrate that ultra-high-field MRI could be a promising tool for investigating distensibility of intracranial arteries in relation to cerebrovascular pathology. < br / > Neurodegener Dis 2016;16:407-410 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - July 22, 2016 Category: Neurology Source Type: research
Human Basic Fibroblast Growth Factor Inhibits Tau Phosphorylation via the PI3K/Akt-GSK3 β Signaling Pathway in a 6-Hydroxydopamine-Induced Model of Parkinson's Disease
Conclusion: < /i > < /b > Liposomes markedly assisted in the delivery of bFGF to the brain and enhanced the neuroprotective effects of bFGF by inhibiting the phosphorylation of tau. bFGF down-regulated the phosphorylation of tau by increasing the phosphorylation of GSK3 β via the PI3K/Akt signaling pathway. These findings provide a new vision of bFGF as a potential therapy for PD. < br / > Neurodegener Dis 2016;16:357-369 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - July 22, 2016 Category: Neurology Source Type: research
A Study of A β Oligomers in the Temporal Cortex and Cerebellum of Patients with Neuropathologically Confirmed Alzheimer's Disease Compared to Aged Controls
Conclusion: < /i > < /b > These results suggest that a putative dodecamer, near 55 kDa, may contribute to AD vulnerability of the TC. < br / > Neurodegener Dis 2016;16:398-406 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - July 22, 2016 Category: Neurology Source Type: research
