Corticobasal degeneration (CBD) exhibits distinct features of akinesia, the 'alien limb' sign and cortical myoclonus. We report a 63-year old woman with a history of CBD for 18 months who was studied twice using all-night polysomnography with an interval of 13 months. Both recordings revealed frequent periodic arm and leg movements predominantly during non-REM sleep. To our knowledge this has not been described in a patient with CBD so far. Similar to a previous report we found REM sleep without atonia (RWA) in all REM episodes. However, the patient showed RWA and some non violent movements that fulfilled the diagnosis of subclinical REM sleep behavior disorder only in the second investigation. These observations may be due to the underlying degenerative process that involves not only cortical but also thalamic and brainstem structures.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/s1389-9457(01)00097-1 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Advanced Magnetic Resonance Imaging for Early Diagnosis and Monitoring of Movement Disorders.
Brain Sci
January 2025
Unidad de Trastornos del Movimiento y Sueño, Hospital General Dr. Manuel Gea González, Calzada de Tlalpan 4800, Mexico City 14080, Mexico.
Advanced magnetic resonance imaging (MRI) techniques are transforming the study of movement disorders by providing valuable insights into disease mechanisms. This narrative review presents a comprehensive overview of their applications in this field, offering an updated perspective on their potential for early diagnosis, disease monitoring, and therapeutic evaluation. Emerging MRI modalities such as neuromelanin-sensitive imaging, diffusion-weighted imaging, magnetization transfer imaging, and relaxometry provide sensitive biomarkers that can detect early microstructural degeneration, iron deposition, and connectivity disruptions in key regions like the substantia nigra.
View Article and Find Full Text PDFRespiratory failure as main presentation sign of MAPT-related disorder.
J Neurol
January 2025
Centre de Génétique Humaine, Centre Hospitalier Universitaire de Besançon, Besançon, France.
Introduction: The MAPT gene encodes Tau, a protein mainly expressed by neurons. Tau protein plays an important role in cerebral microtubule polymerization and stabilization, in axonal transport and synaptic plasticity. Heterozygous pathogenic variation in MAPT are involved in a spectrum of autosomal dominant neurodegenerative diseases known as taupathies, including Alzheimer's disease, Pick's disease, fronto-temporal dementia, cortico-basal degeneration and progressive supranuclear palsy.
View Article and Find Full Text PDFBrain aging rejuvenation factors in adults with genetic and sporadic neurodegenerative disease.
Brain Commun
January 2025
Department of Neurology, Memory and Aging Center, University of California, San Francisco, CA 94158, USA.
The largest risk factor for dementia is age. Heterochronic blood exchange studies have uncovered age-related blood factors that demonstrate 'pro-aging' or 'pro-youthful' effects on the mouse brain. The clinical relevance and combined effects of these factors for humans is unclear.
View Article and Find Full Text PDFAltered cortical network dynamics during observing and preparing action in patients with corticobasal syndrome.
Neurobiol Dis
February 2025
Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich Heine University, 40225 Düsseldorf, Germany. Electronic address:
Corticobasal syndrome (CBS) is characterized not only by parkinsonism but also by higher-order cortical dysfunctions, such as apraxia. However, the electrophysiological mechanisms underlying these symptoms remain poorly understood. To explore the pathophysiology of CBS, we recorded magnetoencephalographic (MEG) data from 17 CBS patients and 20 age-matched controls during an observe-to-imitate task.
View Article and Find Full Text PDFEssential tremor with tau pathology features seeds indistinguishable in conformation from Alzheimer's disease and primary age-related tauopathy.
Acta Neuropathol
January 2025
Center for Alzheimer's and Neurodegenerative Diseases, Peter O'Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Neurodegenerative tauopathies are characterized by the deposition of distinct fibrillar tau assemblies, whose rigid core structures correlate with defined neuropathological phenotypes. Essential tremor (ET) is a progressive neurological disorder that, in some cases, is associated with cognitive impairment and tau accumulation. In this study, we explored tau assembly conformation in ET patients with tau pathology using cytometry-based tau biosensor assays.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!