Heart Rate Variability in Huntington Disease. A Long Term Longitudinal Study
Introduction: The aim of the study was to follow tonic and phasic autonomic nervous activity in Huntington disease (HD) mutation carriers and patients. Methods: Evaluation of motor functions and total functional capacity was performed in 30 HD mutation carriers or patients at the beginning and in 22 subjects after 8-10 years. Continuous arterial blood pressure, heart rate (HR), ECG at rest were measured and HR variability analysis was performed in four different ways. A group of matched controls was also evaluated. Results: Eighteen subjects were assorted in three groups: 6 HD mutation carriers without motor symptoms (PH...
Source: Neurodegenerative Diseases - June 29, 2022 Category: Neurology Source Type: research

Juvenile-Onset Kufs Disease in a Chinese Consanguineous Family due to CLN6 Mutation
Conclusions: A novel homozygous mutation of the CLN6 gene was identified, and patients with the same mutation showed different ages of onset and different levels of severity under MRI. Significance: Our study established that the same CLN6 mutation could produce different phenotypes in patients, and it has expanded the mutational and phenotypical spectrum of the CLN6 gene. (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - May 24, 2022 Category: Neurology Source Type: research

Diagnostic accuracy of noun- and verb-naming tasks in detecting cognitive impairment in Parkinson ’s disease
Conclusions. In accordance with the Movement Disorders Society guidelines, noun-naming tasks are diagnostically sound psychometric instruments to discriminate PD patients with vs. without CI. However, these findings need replication by (1) employing a gold standard different from the MMSE, which does not capture the full range of cognitive impairment in this population and (2) sub-dividing PD patients into those with mild cognitive impairment and dementia. (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - May 23, 2022 Category: Neurology Source Type: research

Distribution Changes of Neural Precursor Cells in the Brain Stem of Tg(SOD1*G93A)1Gur Mice
Conclusions: The data suggest that NPCs widely distribute in the brain stem of adult TG mice can differentiate into astrocytes and migrate into damaged brain regions. This response might be a potential mechanism to repair degenerated motor neurons and restore dysfunctional neural circuitry in ALS. (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - May 18, 2022 Category: Neurology Source Type: research

Plasma Cystatin C correlates with plasma NfL levels and predicts disease progression in Parkinson ’s disease
Conclusions: Cys-C was associated with higher NfL levels and a remarkably faster motor progression in PD independently from peripheral inflammation. Further studies are needed in order to understand the mechanisms underpinning the association of Cys-C with higher neuronal damage markers in neurodegenerative diseases. (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - March 14, 2022 Category: Neurology Source Type: research

Neuroprotective and Therapeutic Effects of Tocovid and Twendee-X on A β Oligomer-Induced Damage in the SH-SY5Y Cell Line
Conclusion: These findings suggest the neuroprotective and therapeutic potential of Tocovid and Twendee-X for AD treatment.Neurodegener Dis (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - March 10, 2022 Category: Neurology Source Type: research

Neuroprotective and therapeutic effects of Tocovid and Twendee-X on A β oligomers-induced damage in the SH-SY5Y cell line
Background: Alzheimer ’s disease (AD) is the most frequent cause of dementia among the elderly. The accumulation of amyloid beta (Aβ) and its downstream pathological events such as oxidative stress play central roles in AD. Recent studies revealed that Aβ oligomers (AβOs) induced strong neurotoxicity in SH-SY5Y cell s via the induction of oxidative stress. Objective: In the present study, we investigated the effect of two antioxidants, Tocovid and Twendee-X, on AβO-induced SH-SY5Y cell damage. Methods: AβOs (2.5 μM) were applied to induce cellular damage in the SH-SY5Y cell line. Cell viability following Aβ O toxi...
Source: Neurodegenerative Diseases - March 10, 2022 Category: Neurology Source Type: research

Inflammatory cytokine levels in patients with sporadic amyotrophic lateral sclerosis
Conclusion Elevated levels of inflammatory cytokines support the hypothesis of an inflammatory response in ALS, and IL-2 and IL-6 may be used as an inflammation-related biomarke r for disease severity. (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - February 4, 2022 Category: Neurology Source Type: research

Sexuality and Neurodegenerative Disease: An Unmet Challenge for Patients, Caregivers, and Treatment
This study gives a general overview about the relationship between sexuality and cognitive impairment. It starts with a general discussion of sexual aspects in the elderly. This is followed by research studies in this field including effects of dementia on sexual life, sexuality issues related to cognitive decline, inappropriate sexual behaviors in dementia patients, and sexuality in healthcare institutions. We discuss also ethical aspects in relation with sexuality and dementia. Finally, we show different approaches to treat inappropriate sexual behaviors.Key Messages: The discussion of sexuality in dementia raises many m...
Source: Neurodegenerative Diseases - January 18, 2022 Category: Neurology Source Type: research

Sexuality and neurodegenerative disease: An unmet challenge for patients, care-givers and treatment.
This study gives a general overview about the relationship between sexuality and cognitive impairment. It starts with a general discussion of sexual aspects in the elderly. This is followed by research studies in this field including effects of dementia on sexual life, sexuality issues related to cognitive decline, inappropriate sexual behaviors in dementia patients and sexuality in healthcare institutions. We discuss also ethical aspects in relation with sexuality and dementia. Finally, we show different approaches to treat inappropriate sexual behaviors. Key messages: The discussion of sexuality in dementia raises many...
Source: Neurodegenerative Diseases - January 18, 2022 Category: Neurology Source Type: research

Homoharringtonine inhibits Alzheimer ’s disease progression by reducing neuroinflammation via STAT3 signaling in APP/PS1 mice
(Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 22, 2021 Category: Neurology Source Type: research

Contents Vol. 20, 2020
Neurodegener Dis 2020;20:I –VI (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 11, 2021 Category: Neurology Source Type: research

Acknowledgement to Reviewers
Neurodegener Dis 2020;20:217 (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 11, 2021 Category: Neurology Source Type: research

Increased Intake of Fast-Acting Carbohydrates in Patients with Parkinson ’s Disease
Conclusion: Our study confirmed changes in intake of fast-acting carbohydrates derived from sweets in PD. Future studies should address the observed association with disease progression to understand underlying pathophysiological mechanisms leading to this behavioral change. (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 9, 2021 Category: Neurology Source Type: research

Increased intake of fast acting carbohydrates in patients with Parkinson ’s Disease
This study investigated the consumption of high sugar-content food products in 221 PD patients compared with 184 healthy controls using a self-administered questionnaire. Results: Male PD patients reported significant more often a high consumption of chocolate (p=0.005) and other sweets (p (Source: Neurodegenerative Diseases)
Source: Neurodegenerative Diseases - November 9, 2021 Category: Neurology Source Type: research