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Stable Muscle Atrophy in Long-term Paraplegics with Complete Upper Motor Neuron Lesion from 3- to 20-year SCI

Overview
Journal Spinal Cord
Specialty Neurology
Date 2007 Oct 24
PMID 17955034
Citations 27
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Abstract

Study Design: Unrandomized trial.

Objectives: To investigate the structural and functional relationships and the progression of muscle atrophy up to 20 years of spastic paraplegia.

Setting: Clinical follow-up in Vienna, Austria; muscle biopsies analyzed by light microscopy in Padova and by electron microscopy (EM) in Chieti, Italy.

Methods: Force was measured as knee extension torque; trophism by computer tomography scan; tissue composition and fiber morphology by histopathology and EM.

Results: In the long-term group of patients (17.0+/-2.6 years), force and size of thigh muscles were only slightly different from those of mid-term subjects (2.2+/-0.5 years). Histology and ultrastructure confirm that the difference in average size of muscle fibers between long-term and mid-term paralyzed leg muscles is actually very small. In addition, muscle fibers maintain the striated appearance characteristic of normal skeletal fibers even after 14-20 years of paralysis. Ultrastructural alterations of the activating and metabolic machineries, and the presence of fibers with lower motor neuron denervation features, may explain the low-force output and the reduced endurance of paretic muscles.

Conclusion: The stable muscle atrophy that characterizes long-lasting spastic paraplegia suggests that there are no upper-time limits to begin a training program based on functional electrical stimulation.

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Deletion of Transferrin Receptor 1 in Parvalbumin Interneurons Induces a Hereditary Spastic Paraplegia-Like Phenotype.

Xiong W, Jin L, Zhao Y, Wu Y, Dong J, Guo Z J Neurosci. 2023; 43(27):5092-5113.

PMID: 37308296 PMC: 10325000. DOI: 10.1523/JNEUROSCI.2277-22.2023.