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Articles Tagged With: neurodegenerative

  • Medications for Progressive Supranuclear Palsy: A Note of Caution

    In this post hoc secondary analysis of a Phase II/III randomized clinical trial of davunetide for progressive supranuclear palsy (PSP), investigators found that patients with PSP prescribed benzodiazepine derivatives experienced more rapid worsening of their PSP Rating Scale scores over time.

  • Repetitive Head Impacts and Chronic Traumatic Encephalopathy in Young Contact Sport Athletes

    In a first of its kind study, researchers found neuropathological markers suggestive of chronic traumatic encephalopathy (CTE) pathology in 41% (n = 152) of young contact sport athletes (< 30 years of age) at autopsy. Clinical measures (obtained retrospectively via informant report) assessing for cognitive and psychiatric/behavioral dysfunction, while elevated across most measures for the entire group, did not significantly differ between brain donors with and without a CTE diagnosis. Functional impairments also were not commonly found.

  • Lovastatin for Reducing Risk and Delaying Onset of ALS

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease without available treatments that significantly alter the disease course. Using Medicare databases and pharmacy records, with multiple logistic regression models, researchers studied three candidate drugs (lovastatin, sulfasalazine, and telmisartan) that were identified as possible therapies for ALS and their effects on SOD1G93A transgenic mice. Animal testing showed a delay in disease onset and prolonged survival in mice treated with a mouse-equivalent dose of lovastatin 40 mg, but showed no benefit from the other two candidate drugs.

  • Viral Exposure and Risk of Neurodegenerative Disease

    Twenty-two pairs of viral exposures associated with an increased risk of neurodegenerative diseases were identified and replicated across two national European biobanks.

  • Early Respiratory Decline in Amyotrophic Lateral Sclerosis

    Hypercapnia, a manifestation of early respiratory dysfunction, can be challenging to detect in amyotrophic lateral sclerosis (ALS) patients. Pulmonary function tests are helpful, but their specificity in detecting hypercapnia is low and their use is limited in patients with bulbar weakness. Specific symptoms, such as dyspnea at rest, dyspnea while talking, and use of medications for sleep, can be more reliable in detecting hypercapnia among ALS patients.

  • Lipid Pathway Dysfunction in Parkinson’s Disease

    In this large-scale study, the authors used a comprehensive untargeted lipidomic approach to determine the extent to which lipid dysregulation occurs in patients with Parkinson’s disease generally and in mutation carriers of one of the most common Parkinson’s disease risk genes, LRRK2. Further pathway analysis reveals sphingolipid metabolism, insulin signaling, and mitochondrial function as major metabolic pathways dysregulated in Parkinson’s disease.

  • The Natural History of Leigh Syndrome

    The natural history of Leigh syndrome is characterized by quantifiable disease progression in fewer than three years, with the poorest outcomes predicted by surfeit locus protein 1 (SURF1) pathogenic variants, bilateral caudate involvement on magnetic resonance imaging, and rapid increases in yearly Newcastle Paediatric Mitochondrial Disease Scale scores.

  • Plasma Biomarkers for Neurodegenerative Dementias: We Are Getting Closer

    Two recent multicenter studies suggest that plasma biomarkers, including phosphorylated-tau181, could be used as cost-effective and more readily accessible biomarkers for the diagnosis and management of individuals with neurodegenerative dementias.

  • Neurodegenerative Ataxia: Improvement in Motor and Cognitive Outcomes with Cerebello-Spinal Stimulation

    Concurrent cerebellar and spinal stimulation with a transcranial direct current stimulation device resulted in improvement of both motor and cognitive functions in patients with several different forms of neurodegenerative ataxia.

  • Diagnostic Utility of CSF Alpha-Synuclein

    This observational study investigated whether the cerebrospinal fluid (CSF) α-synuclein (α-syn) real-time quaking-induced conversion (RT-QuIC) assay, applied to 289 CSF samples, accurately identified patients with mild cognitive impairment (MCI) caused by probable Lewy body (LB) disease. RT-QuIC identified patients with MCI-LB against cognitively unimpaired controls with 95% sensitivity, 97% specificity, and 96% accuracy and showed 98% specificity in neuropathologic controls, indicating that CSF α-syn RT-QuIC is a robust biomarker for prodromal dementia with Lewy bodies.