Eight infants with an acute reversible motor unit disorder are described, including two infants from whom Clostridum botulinum type A was isolated from stool specimens. The clinical spectrum includes constipation, cranial nerve deficits, pupillary involvement, and generalized hypotonic weakness. There were no deaths, and all infants have had complete clinical recovery. A characteristic electromyographic (EMG) pattern was present in part until clinical recovery. This distinctive pattern consisted of brief, small, abundant for power exerted motor unit potentials. This EMG pattern in the context of the clinical syndrome may well be diagnostic for acute infantile motor unit disorder.
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http://dx.doi.org/10.1001/archneur.1977.00500160050009 | DOI Listing |
JBJS Rev
November 2024
Division of Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, Colorado.
Background: Modern nerve-to-nerve transfers are a significant advancement in peripheral nerve surgery. Nerve transfers involve transferring donor nerves or branches to recipient nerves close to the motor end unit, leading to earlier reinnervation and preservation of the musculotendinous units in proximal nerve injuries. After nerve reinnervation, function may be superior to traditional tendon transfer techniques in terms of strength and independent motion.
View Article and Find Full Text PDFJ Appl Physiol (1985)
January 2025
Department of Electrical Engineering and Automation, Aalto University, Espoo, Finland.
Disruption of the blood supply to a limb in conjunction with active movement boosts muscle growth, aids in rehabilitation, and allows controlled exploration of the sensorimotor system. Yet, the underlying neuromechanical changes have not been observed in great detail. This study aims to report the acute neuromuscular effects of temporary blood flow restriction (BFR) through behavioral changes at the level of motor units (MUs) using high-density surface electromyography on the abductor digiti minimi muscle during 20 trapezoidal and sinusoidal isometric force tracking tasks (5 pre-BFR, 5 during BFR, and 10 post-BFR).
View Article and Find Full Text PDFJ Neurol
January 2025
Sleep Unit, Neurology Service, Hospital Clínic Barcelona, IDIBAPS, CIBERNED, Universitat de Barcelona, Villarroel 170, 08036, Barcelona, Spain.
Background: The diagnosis of isolated REM sleep behavior disorder (IRBD) requires video polysomnography (V-PSG) showing increased muscle activity and abnormal behaviors in REM sleep.
Objective: To describe in IRBD the behavioral manifestations occurring during REM sleep in the diagnostic V-PSG.
Methods: This is a systematic audiovisual V-PSG analysis of consecutive IRBD patients.
J Neurol
January 2025
Laboratory of Clinical Pharmacology and Therapeutics, Faculdade de Medicina, Universidade de Lisboa, Av. Prof. Egas Moniz, 1649-028, Lisbon, Portugal.
Background: Drooling, defined as the unintentional loss of saliva from the anterior oral cavity, remains poorly understood in terms of the underlying clinical factors in people with Parkinson's disease (PwP). This study aims to clarify these factors by analyzing predictors and secondarily the correlates with the severity of drooling in PwP.
Methods: We conducted a cross-sectional study involving 42 PwP with drooling and 59 without drooling.
Epilepsia
January 2025
Department of Epilepsy Genetics and Personalized Medicine, Danish Epilepsy Center, Dianalund, Denmark.
Objectives: Developmental and epileptic encephalopathies (DEEs) caused by pathogenic variants in SCN8A are associated with difficult-to-treat and early-onset seizures, developmental delay/intellectual disability, impaired quality of life, and increased risk of early mortality. High doses of sodium channel blockers are typically used to treat SCN8A-DEE caused by gain-of-function (GoF) variants. However, seizures are often drug resistant, and only a few patients achieve seizure freedom.
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