We studied motor unit changes in 20 patients with Parkinson's disease (PD) and 20 age-matched control subjects to look for evidence of motorneuron degeneration in sporadic idiopathic PD. Patients and control subjects were screened by clinical criteria and nerve conduction studies to exclude those with peripheral neuropathic processes. Changes in motor unit morphology were investigated with subjective and computerized quantitative electromyography (QEMG) of the anterior tibialis (AT) and first dorsal interosseous. Multivariate comparisons showed a significant difference in the QEMG analysis for motor unit enlargement in patients with PD versus control subjects. Some of the univariate comparisons for both the subjective and QEMG analyses of the AT were also significant. These results demonstrate that motorneuron drop-out with reinnervation occurs in sporadic idiopathic PD. In summary, our findings provide evidence that clinically silent motorneuron disease occurs in typical cases of sporadic idiopathic PD, suggesting that it may be a normal part of the pathologic picture of PD. Any hypothesis concerning the pathogenic mechanism of PD would need to take into account such a finding.
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http://dx.doi.org/10.1002/1531-8257(200003)15:2<238::aid-mds1006>3.0.co;2-j | DOI Listing |
Neuromuscul Disord
January 2025
Division of Neuropediatrics and Developmental Medicine, University Children's Hospital Basel (UKBB), University of Basel, Basel, Switzerland. Electronic address:
Most patients with Duchenne muscular dystrophy (DMD) are non-ambulant. Preserving proximal motor function is crucial, rarely studied. Tamoxifen, a selective oestrogen receptor modulator, reduced signs of muscular pathology in a DMD mouse model.
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January 2025
Department of Computer Science, University of Verona, Verona, Italy.
Background: Axial postural abnormalities (APAs) are frequent and disabling axial symptoms of Parkinson's disease (PD). Image-based measurement is considered the gold standard but may not accurately detect the true severity of APAs because these symptoms can appear or get worse under dynamic conditions.
Objective: The aim was to evaluate quantitative changes in APAs degree during prolonged standing and walking in both single- and dual-task conditions (motor + cognitive).
Eur J Neurol
February 2025
1st Department of Neurology, Eginition Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Background: The p.A53T variant in the SNCA gene was considered, until recently, to be the only SNCA variant causing familial Parkinson's disease (PD) in the Greek population. We identified a novel heterozygous p.
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January 2025
Paediatric Neurology and Neurorehabilitation Unit, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Aim: Young people with childhood-onset motor disabilities face unique challenges in understanding and managing their condition. This study explored how they learnt about their condition.
Method: A descriptive qualitative study was conducted in 2023-2024 at a Swiss paediatric neurorehabilitation unit.
Bioact Mater
May 2025
Instituto de Ciencia de Materiales de Madrid (ICMM), Consejo Superior de Investigaciones Científicas (CSIC), Calle Sor Juana Inés de la Cruz 3, 28049, Madrid, Spain.
Millions of patients and their caretakers live and deal with the devastating consequences of spinal cord injury (SCI) worldwide. Despite outstanding advances in the field to both understand and tackle these pathologies, a cure for SCI patients, with their peculiar characteristics, is still a mirage. One of the most promising therapeutic strategies to date for these patients involves the use of epidural electrical stimulation.
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