Motor unit number estimation in infants and children with spinal muscular atrophy

Spinal muscular atrophy (SMA) is a disease of lower motor neurons. Motor unit number estimation (MUNE) is an electrophysiologic method to estimate the number of motor neurons innervating a muscle group. We applied the multiple point stimulation technique to the ulnar nerve–hypothenar muscle group to...

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Bibliographic Details
Published in:Muscle & nerve 2002-03, Vol.25 (3), p.445-447
Main Authors: Bromberg, Mark B., Swoboda, Kathryn J.
Format: Article
Language:English
Subjects:
Action Potentials
Adolescent
Adult
Biological and medical sciences
Cell Count
Child
Child, Preschool
Electrodiagnosis. Electric activity recording
Electrophysiology - methods
Humans
Infant
Infant, Newborn
Investigative techniques, diagnostic techniques (general aspects)
Medical sciences
Middle Aged
motor neuron disease
Motor Neurons - pathology
motor unit number estimation (MUNE)
Muscle, Skeletal - innervation
Muscle, Skeletal - pathology
Nervous system
Spinal Muscular Atrophies of Childhood - pathology
spinal muscular atrophy (SMA)
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Summary:Spinal muscular atrophy (SMA) is a disease of lower motor neurons. Motor unit number estimation (MUNE) is an electrophysiologic method to estimate the number of motor neurons innervating a muscle group. We applied the multiple point stimulation technique to the ulnar nerve–hypothenar muscle group to study lower motor neuron loss in 14 SMA subjects, including those presymptomatic, and varying from newborn through 45 years of age. Preliminary data support the value of MUNE to help understand the time course of motor neuron loss in SMA. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.10050