The present study describes an objective, cost- and time-efficient procedure for characterizing the ataxic effects of psychoactive drugs. Male Sprague-Dawley rats were administered an intraperitoneal injection of either saline or one of three doses (1, 5 or 10 mg/kg) of phencyclidine (PCP) 15 min prior to being placed into an empty standard operant conditioning chamber (all manipulanda were removed). The floor of the test apparatus consisted of parallel rows of metal rods spaced approximately 1.5 cm apart. During a 5-min test, a single observer counted the frequency with which each animal's paws (front or back) slipped between the rows of bars that constituted the cage floor. The data demonstrated that while saline animals exhibited no instability in their ambulation, PCP-treated animals demonstrated a highly reliable dose-dependent increase in the number of "paw slips" in a single trial. Since animals are known to develop tolerance to the ataxic response to PCP, the validity of the test as a measure of drug-induced ataxia was examined in a separate group of animals treated with the middle (5 mg/kg) dose every other day over the course of a 9-day period (i.e., resulting in five injection trials). In this experiment, each subsequent test produced a reliable reduction in the magnitude of the ataxic response, and by the fifth drug challenge, the PCP animals were performing at near-control levels. These results suggest that the "paw slip test" can serve as a simple, reliable, objective and valid measure of drug-induced ataxia. The relevance of the ataxia data for interpreting the locomotor response of animals treated with PCP is also discussed.
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http://dx.doi.org/10.1016/s0091-3057(01)00727-4 | DOI Listing |
Eur J Hum Genet
January 2025
Institute of Bioinformatics, International Technology Park, Bangalore, 560066, India.
Mitochondrial membrane protein-associated neurodegeneration (MPAN) is a rare neurodegenerative disorder characterized by spastic paraplegia, parkinsonism and psychiatric and/or behavioral symptoms caused by variants in gene encoding chromosome-19 open reading frame-12 (C19orf12). We present here seven patients from six unrelated families with detailed clinical, radiological, and genetic investigations. Childhood-onset patients predominantly had a spastic ataxic phenotype with optic atrophy, while adult-onset patients were presented with cognitive, behavioral, and parkinsonian symptoms.
View Article and Find Full Text PDFCureus
October 2024
Department of Pediatrics: Pediatric Neurology, Local Healthcare Unit Alto Ave, Guimarães, PRT.
Varicella-zoster virus is a highly contagious infection that primarily affects children. It typically presents as a mild, self-limiting illness with a distinctive rash. However, severe complications can arise, with skin and soft tissue infections being the most common.
View Article and Find Full Text PDFAnn Neurol
October 2024
Department of Neurology, Donders Institute for Brain, Cognition, and Behavior, Radboud University Medical Center, Nijmegen, Netherlands.
Objectives: Spinocerebellar ataxia type 1 (SCA1) is a rare autosomal dominant neurodegenerative disease. Objective surrogate markers sensitive to detect changes in disease severity are needed to reduce sample sizes in interventional trials and identification of predictors of faster disease progression would facilitate patient selection, enrichment, or stratification in such trials.
Methods: We performed a prospective 1-year longitudinal, multimodal study in 34 ataxic SCA1 individuals and 21 healthy controls.
Neurology
August 2024
From the Neuromuscular Diseases Unit (E.P.-G., M.C.-Á., L.Q.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona; ERN (European Reference Network) EURO-NMD (Neuromuscular Disorders); and Centro para la Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (E.P.-G., L.Q.), Madrid, Spain.
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