Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease

Akito Ikemoto, Asao Hirano, Sadayuki Matsumoto, Ichiro Akiguchi, Jun Kimura

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

This report concerns the study of synaptophysin (SP) expression in the anterior horn in four cases of Werdnig-Hoffmann disease (WHD). All patients had distinct anterior horn cell degeneration, and died before the age of one year. Normal spinal cords from five age-matched children served as controls. Five cases of sporadic amyotrophic lateral sclerosis (S-ALS), three cases of lower motor neuron disease (L-MND), three cases of peripheral neuropathy with axonal reaction, and six adult cases with normal spinal cords were included for comparison. Immunohistochemical techniques were used throughout. The results show that normal spinal cords of children have similar SP immunoreactivity patterns as those of normal adults. We also found that despite relatively preserved or slightly increased SP immunoreactivity on the surface of the cell body and proximal processes of the remaining neurons, there was a diffuse decrease of immunoreaction product deposits in the anterior horn neuropil of the WHD cases. The ballooned neurons in the anterior hems of patients with WHD, S-ALS, L-MND, and axonal reaction had few SP immunoreactive dots or granules around the cell bodies and proximal processes. The perikarya of some ballooned neurons of the children with WHD was diffusely stained for SP. There was no SP immunoreactive structures within the empty cell beds of these patients. The observed decrease in SP expression around ballooned neurons in these disorders is indicative of a disconnection of presynaptic terminals of afferent fibers from the proximal portion of the swollen degenerated anterior horn cells.

Original languageEnglish (US)
Pages (from-to)94-100
Number of pages7
JournalJournal of the Neurological Sciences
Volume136
Issue number1-2
DOIs
StatePublished - 1996

Fingerprint

Spinal Muscular Atrophies of Childhood
Synaptophysin
Horns
Retrograde Degeneration
Anterior Horn Cells
Neurons
Spinal Cord
Motor Neuron Disease
Neuropil
Presynaptic Terminals
Amyotrophic Lateral Sclerosis
Peripheral Nervous System Diseases

Keywords

  • Anterior horn cell
  • Axonal reaction
  • Ballooned neuron
  • Immunohistochemistry
  • Motor neuron disease
  • Presynaptic terminal
  • Synaptophysin
  • Werdnig-Hoffmann disease

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Developmental Neuroscience
  • Neurology
  • Neuroscience(all)

Cite this

Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease. / Ikemoto, Akito; Hirano, Asao; Matsumoto, Sadayuki; Akiguchi, Ichiro; Kimura, Jun.

In: Journal of the Neurological Sciences, Vol. 136, No. 1-2, 1996, p. 94-100.

Research output: Contribution to journalArticle

Ikemoto, A, Hirano, A, Matsumoto, S, Akiguchi, I & Kimura, J 1996, 'Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease', Journal of the Neurological Sciences, vol. 136, no. 1-2, pp. 94-100. https://doi.org/10.1016/0022-510X(95)00297-F
Ikemoto, Akito ; Hirano, Asao ; Matsumoto, Sadayuki ; Akiguchi, Ichiro ; Kimura, Jun. / Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease. In: Journal of the Neurological Sciences. 1996 ; Vol. 136, No. 1-2. pp. 94-100.
@article{c461e24373e34a9fba7fec809ac35af4,
title = "Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease",
abstract = "This report concerns the study of synaptophysin (SP) expression in the anterior horn in four cases of Werdnig-Hoffmann disease (WHD). All patients had distinct anterior horn cell degeneration, and died before the age of one year. Normal spinal cords from five age-matched children served as controls. Five cases of sporadic amyotrophic lateral sclerosis (S-ALS), three cases of lower motor neuron disease (L-MND), three cases of peripheral neuropathy with axonal reaction, and six adult cases with normal spinal cords were included for comparison. Immunohistochemical techniques were used throughout. The results show that normal spinal cords of children have similar SP immunoreactivity patterns as those of normal adults. We also found that despite relatively preserved or slightly increased SP immunoreactivity on the surface of the cell body and proximal processes of the remaining neurons, there was a diffuse decrease of immunoreaction product deposits in the anterior horn neuropil of the WHD cases. The ballooned neurons in the anterior hems of patients with WHD, S-ALS, L-MND, and axonal reaction had few SP immunoreactive dots or granules around the cell bodies and proximal processes. The perikarya of some ballooned neurons of the children with WHD was diffusely stained for SP. There was no SP immunoreactive structures within the empty cell beds of these patients. The observed decrease in SP expression around ballooned neurons in these disorders is indicative of a disconnection of presynaptic terminals of afferent fibers from the proximal portion of the swollen degenerated anterior horn cells.",
keywords = "Anterior horn cell, Axonal reaction, Ballooned neuron, Immunohistochemistry, Motor neuron disease, Presynaptic terminal, Synaptophysin, Werdnig-Hoffmann disease",
author = "Akito Ikemoto and Asao Hirano and Sadayuki Matsumoto and Ichiro Akiguchi and Jun Kimura",
year = "1996",
doi = "10.1016/0022-510X(95)00297-F",
language = "English (US)",
volume = "136",
pages = "94--100",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "1-2",

}

TY - JOUR

T1 - Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease

AU - Ikemoto, Akito

AU - Hirano, Asao

AU - Matsumoto, Sadayuki

AU - Akiguchi, Ichiro

AU - Kimura, Jun

PY - 1996

Y1 - 1996

N2 - This report concerns the study of synaptophysin (SP) expression in the anterior horn in four cases of Werdnig-Hoffmann disease (WHD). All patients had distinct anterior horn cell degeneration, and died before the age of one year. Normal spinal cords from five age-matched children served as controls. Five cases of sporadic amyotrophic lateral sclerosis (S-ALS), three cases of lower motor neuron disease (L-MND), three cases of peripheral neuropathy with axonal reaction, and six adult cases with normal spinal cords were included for comparison. Immunohistochemical techniques were used throughout. The results show that normal spinal cords of children have similar SP immunoreactivity patterns as those of normal adults. We also found that despite relatively preserved or slightly increased SP immunoreactivity on the surface of the cell body and proximal processes of the remaining neurons, there was a diffuse decrease of immunoreaction product deposits in the anterior horn neuropil of the WHD cases. The ballooned neurons in the anterior hems of patients with WHD, S-ALS, L-MND, and axonal reaction had few SP immunoreactive dots or granules around the cell bodies and proximal processes. The perikarya of some ballooned neurons of the children with WHD was diffusely stained for SP. There was no SP immunoreactive structures within the empty cell beds of these patients. The observed decrease in SP expression around ballooned neurons in these disorders is indicative of a disconnection of presynaptic terminals of afferent fibers from the proximal portion of the swollen degenerated anterior horn cells.

AB - This report concerns the study of synaptophysin (SP) expression in the anterior horn in four cases of Werdnig-Hoffmann disease (WHD). All patients had distinct anterior horn cell degeneration, and died before the age of one year. Normal spinal cords from five age-matched children served as controls. Five cases of sporadic amyotrophic lateral sclerosis (S-ALS), three cases of lower motor neuron disease (L-MND), three cases of peripheral neuropathy with axonal reaction, and six adult cases with normal spinal cords were included for comparison. Immunohistochemical techniques were used throughout. The results show that normal spinal cords of children have similar SP immunoreactivity patterns as those of normal adults. We also found that despite relatively preserved or slightly increased SP immunoreactivity on the surface of the cell body and proximal processes of the remaining neurons, there was a diffuse decrease of immunoreaction product deposits in the anterior horn neuropil of the WHD cases. The ballooned neurons in the anterior hems of patients with WHD, S-ALS, L-MND, and axonal reaction had few SP immunoreactive dots or granules around the cell bodies and proximal processes. The perikarya of some ballooned neurons of the children with WHD was diffusely stained for SP. There was no SP immunoreactive structures within the empty cell beds of these patients. The observed decrease in SP expression around ballooned neurons in these disorders is indicative of a disconnection of presynaptic terminals of afferent fibers from the proximal portion of the swollen degenerated anterior horn cells.

KW - Anterior horn cell

KW - Axonal reaction

KW - Ballooned neuron

KW - Immunohistochemistry

KW - Motor neuron disease

KW - Presynaptic terminal

KW - Synaptophysin

KW - Werdnig-Hoffmann disease

UR - http://www.scopus.com/inward/record.url?scp=0029985237&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0029985237&partnerID=8YFLogxK

U2 - 10.1016/0022-510X(95)00297-F

DO - 10.1016/0022-510X(95)00297-F

M3 - Article

VL - 136

SP - 94

EP - 100

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

IS - 1-2

ER -