Function and ultrastructure of the neuromuscular junction in post-polio syndrome

Ricardo A Maselli, R. Wollmann, R. Roos

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

We performed a detailed morphological and electrophysiological analysis of the neuromuscular junction in muscle biopsies from 10 patients with post-polio syndrome (PPS). This was done to clarify the basis for the apparent neuromuscular transmission impairment in PPS. In six patients, intracellular microelectrode recordings demonstrated either reduction of amplitudes of miniature end-plate potentials (MEPPs) or decreased quantal content or both. In one patient, reduction of quantal content was only present with prolonged or high-frequency nerve stimulation. In three patients no significant abnormalities were found by the intracellular microelectrode studies. Histologically, atrophy of individual muscle fibers were present in 6 out of the 10 biopsies, but grouped atrophy was not seen. Fiber type grouping suggesting reinnervation was seen in 8 out of the 10 muscle biopsies. Fragmentation and dispersion of the end plate was present in three patients. In two of these patients dispersion of the end plate was associated with an apparent increase of the quantal content. Electron microscopy revealed either normal neuromuscular junctions or small axon termini apposed to normal postsynaptic folds. In summary, variable degrees and different types of failure of neuromuscular transmission were seen in association with histological signs of reinnervation in the muscle biopsies of affected patients. Functional and structural abnormalities of the neuromuscular junction, although very common, were not invariably present and, therefore, they do not appear to be a necessary condition to define the post-poliomyelitic syndrome.

Original languageEnglish (US)
Pages (from-to)129-137
Number of pages9
JournalAnnals of the New York Academy of Sciences
Volume753
DOIs
StatePublished - 1995

Fingerprint

Postpoliomyelitis Syndrome
Biopsy
Neuromuscular Junction
Muscle
Microelectrodes
Fibers
Muscles
Electron microscopy
Miniature Postsynaptic Potentials
Association reactions
Muscular Atrophy
Syndrome
Polio
Atrophy
Axons
Electron Microscopy

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Function and ultrastructure of the neuromuscular junction in post-polio syndrome. / Maselli, Ricardo A; Wollmann, R.; Roos, R.

In: Annals of the New York Academy of Sciences, Vol. 753, 1995, p. 129-137.

Research output: Contribution to journalArticle

@article{6c282b8b5e9e43e499edca26966c8c3c,
title = "Function and ultrastructure of the neuromuscular junction in post-polio syndrome",
abstract = "We performed a detailed morphological and electrophysiological analysis of the neuromuscular junction in muscle biopsies from 10 patients with post-polio syndrome (PPS). This was done to clarify the basis for the apparent neuromuscular transmission impairment in PPS. In six patients, intracellular microelectrode recordings demonstrated either reduction of amplitudes of miniature end-plate potentials (MEPPs) or decreased quantal content or both. In one patient, reduction of quantal content was only present with prolonged or high-frequency nerve stimulation. In three patients no significant abnormalities were found by the intracellular microelectrode studies. Histologically, atrophy of individual muscle fibers were present in 6 out of the 10 biopsies, but grouped atrophy was not seen. Fiber type grouping suggesting reinnervation was seen in 8 out of the 10 muscle biopsies. Fragmentation and dispersion of the end plate was present in three patients. In two of these patients dispersion of the end plate was associated with an apparent increase of the quantal content. Electron microscopy revealed either normal neuromuscular junctions or small axon termini apposed to normal postsynaptic folds. In summary, variable degrees and different types of failure of neuromuscular transmission were seen in association with histological signs of reinnervation in the muscle biopsies of affected patients. Functional and structural abnormalities of the neuromuscular junction, although very common, were not invariably present and, therefore, they do not appear to be a necessary condition to define the post-poliomyelitic syndrome.",
author = "Maselli, {Ricardo A} and R. Wollmann and R. Roos",
year = "1995",
doi = "10.1111/j.1749-6632.1995.tb27539.x",
language = "English (US)",
volume = "753",
pages = "129--137",
journal = "Annals of the New York Academy of Sciences",
issn = "0077-8923",
publisher = "Wiley-Blackwell",

}

TY - JOUR

T1 - Function and ultrastructure of the neuromuscular junction in post-polio syndrome

AU - Maselli, Ricardo A

AU - Wollmann, R.

AU - Roos, R.

PY - 1995

Y1 - 1995

N2 - We performed a detailed morphological and electrophysiological analysis of the neuromuscular junction in muscle biopsies from 10 patients with post-polio syndrome (PPS). This was done to clarify the basis for the apparent neuromuscular transmission impairment in PPS. In six patients, intracellular microelectrode recordings demonstrated either reduction of amplitudes of miniature end-plate potentials (MEPPs) or decreased quantal content or both. In one patient, reduction of quantal content was only present with prolonged or high-frequency nerve stimulation. In three patients no significant abnormalities were found by the intracellular microelectrode studies. Histologically, atrophy of individual muscle fibers were present in 6 out of the 10 biopsies, but grouped atrophy was not seen. Fiber type grouping suggesting reinnervation was seen in 8 out of the 10 muscle biopsies. Fragmentation and dispersion of the end plate was present in three patients. In two of these patients dispersion of the end plate was associated with an apparent increase of the quantal content. Electron microscopy revealed either normal neuromuscular junctions or small axon termini apposed to normal postsynaptic folds. In summary, variable degrees and different types of failure of neuromuscular transmission were seen in association with histological signs of reinnervation in the muscle biopsies of affected patients. Functional and structural abnormalities of the neuromuscular junction, although very common, were not invariably present and, therefore, they do not appear to be a necessary condition to define the post-poliomyelitic syndrome.

AB - We performed a detailed morphological and electrophysiological analysis of the neuromuscular junction in muscle biopsies from 10 patients with post-polio syndrome (PPS). This was done to clarify the basis for the apparent neuromuscular transmission impairment in PPS. In six patients, intracellular microelectrode recordings demonstrated either reduction of amplitudes of miniature end-plate potentials (MEPPs) or decreased quantal content or both. In one patient, reduction of quantal content was only present with prolonged or high-frequency nerve stimulation. In three patients no significant abnormalities were found by the intracellular microelectrode studies. Histologically, atrophy of individual muscle fibers were present in 6 out of the 10 biopsies, but grouped atrophy was not seen. Fiber type grouping suggesting reinnervation was seen in 8 out of the 10 muscle biopsies. Fragmentation and dispersion of the end plate was present in three patients. In two of these patients dispersion of the end plate was associated with an apparent increase of the quantal content. Electron microscopy revealed either normal neuromuscular junctions or small axon termini apposed to normal postsynaptic folds. In summary, variable degrees and different types of failure of neuromuscular transmission were seen in association with histological signs of reinnervation in the muscle biopsies of affected patients. Functional and structural abnormalities of the neuromuscular junction, although very common, were not invariably present and, therefore, they do not appear to be a necessary condition to define the post-poliomyelitic syndrome.

UR - http://www.scopus.com/inward/record.url?scp=0029051042&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0029051042&partnerID=8YFLogxK

U2 - 10.1111/j.1749-6632.1995.tb27539.x

DO - 10.1111/j.1749-6632.1995.tb27539.x

M3 - Article

VL - 753

SP - 129

EP - 137

JO - Annals of the New York Academy of Sciences

JF - Annals of the New York Academy of Sciences

SN - 0077-8923

ER -