Conduction of the central motor pathways (CMCT) by magnetic stimulation of the motor cortex (TMS) was performed in 17 patients with hereditary motor sensory neuropathy (HMSN) and 2 siblings with hereditary spastic paraplegia (HSP). CMCT was prolonged in two patients with HMSN I with associated pyramidal features and in two subjects with HMSN II without clinical pyramidal signs. CMCT may be abnormal in HMSN due to central motor pathways involvement or altered spinal excitability with increased synaptic delay. CMCT was normal in the upper limbs in patients with HSP but increased in the legs. Diagnostic yield of TMS increased in less disabled cases with HSP when selective conduction at the spinal level (C7-S1) was calculated. Abnormal spinal conduction in HSP is consistent with degeneration of the crossed corticospinal tracts at the thoracic level found in neuropathologic observations.
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Neurol Int
January 2025
Department of Medicine, Faculty of Medicine, Srinakharinwirot University, Nakhon Nayok 26120, Thailand.
Background: Central facial palsy (CFP), resulting from upper motor neuron lesions in the corticofacial pathway, is traditionally characterized by the sparing of the upper facial muscles. However, reports of upper facial weakness in CFP due to acute ischemic stroke have challenged this long-held assumption. This study aimed to determine the prevalence of upper facial weakness in CFP and identify its associated clinical factors.
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January 2025
Department of Neuroscience, "Giovanni Paolo II" Hospital, Lamezia Terme, 88046 Catanzaro, Italy.
Stage 1 Plus is defined here as a naïve, previously untreated, status epilepticus (SE) that is probably refractory to Benzodiazepines (BDZ). These cases include not only prolonged SE as previously proposed by the author (SE lasting > 10 min) but also other cases notoriously associated with BDZ refractoriness such as the absence of prominent motor phenomena and acute etiology (especially primary central nervous system etiology). Interestingly, the absence of prominent motor phenomena as is the case of non convulsive SE might implicitly fall in the category of prolonged SE due to the delay in recognition and treatment.
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January 2025
State Key Laboratory of Cardiology and Medical Innovation Center, Shanghai East Hospital, Clinical Center for Brain and Spinal Cord Research, School of Medicine, Tongji University, 200331, Shanghai, China.
Amyotrophic lateral sclerosis (ALS) is a severe motor neuron disease, with most sporadic cases lacking clear genetic causes. Abnormal pre-mRNA splicing is a fundamental mechanism in neurodegenerative diseases. For example, TAR DNA-binding protein 43 (TDP-43) loss-of-function (LOF) causes widespread RNA mis-splicing events in ALS.
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December 2024
Department of Speech, Hearing and Phonetic Sciences, Division of Psychology and Language Sciences, University College London, Chandler House 2 Wakefield Street, London WC1N 1PF, UK.
Speech is a highly skilled motor activity that shares a core problem with other motor skills: how to reduce the massive degrees of freedom (DOF) to the extent that the central nervous control and learning of complex motor movements become possible. It is hypothesized in this paper that a key solution to the DOF problem is to eliminate most of the temporal degrees of freedom by synchronizing concurrent movements, and that this is performed in speech through the syllable-a mechanism that synchronizes consonantal, vocalic, and laryngeal gestures. Under this hypothesis, syllable articulation is enabled by three basic mechanisms: target approximation, edge-synchronization, and tactile anchoring.
View Article and Find Full Text PDFBioengineering (Basel)
January 2025
School of Engineering, Grand Valley State University, Grand Rapids, MI 49504, USA.
In quiet standing, the central nervous system implements a pre-programmed ankle strategy of postural control to maintain upright balance and stability. This strategy comprises a synchronized common neural drive delivered to synergistically grouped muscles. This study evaluated connectivity between EMG signals of the unilateral and bilateral homologous muscle pairs of the lower legs during various standing balance conditions using magnitude-squared coherence (MSC).
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