TY - JOUR
T1 - Skein-like inclusions in the anterior horn cells in motor neuron disease
AU - Mizusawa, Hidehiro
AU - Nakamura, Haruomi
AU - Wakayama, Ikuro
AU - Yen, Shu Hui C.
AU - Hirano, Asao
N1 - Funding Information:
Acknowledgements A part of this study was presented at the llth International Congress of Neuropathology, Kyoto, Japan, September 1990. We thank Dr. S. Kuroda for permitting us to examine an individual case and Dr. T. Arai for providing anti-tubulin antibodies. This work was supported in part by a grant-in-aid from the Ministry of Education (Japan) and the Amyotrophic Lateral Sclerosis Association (U.S.A.).
PY - 1991/9
Y1 - 1991/9
N2 - Skein-like inclusions (SLIs) in the anterior horn cells of patients with motor neuron diseases, including familial amyotrophic lateral sclerosis with posterior column degeneration, sporadic lower motor neuron disease and classical amyotrophic lateral sclerosis, were investigated morphologically with hematoxylin and eosin preparations, immunostaining for ubiquitin and immunoelectron microscopy. The SLIs were thready linear or tubular structures which immunostained with antiubiquitin antibodies. They were detected on hematoxylin and eosin preparations as eosinophilic thread-like structures often surrounded by pale areas. SLIs were occasionally present as networks of threads or tubules. Sometimes, they were aggregated and formed larger pale inclusions. Ultrastructurally, the SLIs were bundles of filaments which appeared thicker than neurofilaments. The SLIs tended to have central hollow spaces which were devoid of filaments. When the SLIs were clustered, fuzzy thick filaments were randomly and loosely arranged among the individual SLIs. The SLIs were histologically and ultrastructurally distinct from other inclusions such as Bunina bodies and hyaline inclusions. This unique morphology of SLIs may provide a novel perspective on the degenerative processes of the anterior horn cells in MND.
AB - Skein-like inclusions (SLIs) in the anterior horn cells of patients with motor neuron diseases, including familial amyotrophic lateral sclerosis with posterior column degeneration, sporadic lower motor neuron disease and classical amyotrophic lateral sclerosis, were investigated morphologically with hematoxylin and eosin preparations, immunostaining for ubiquitin and immunoelectron microscopy. The SLIs were thready linear or tubular structures which immunostained with antiubiquitin antibodies. They were detected on hematoxylin and eosin preparations as eosinophilic thread-like structures often surrounded by pale areas. SLIs were occasionally present as networks of threads or tubules. Sometimes, they were aggregated and formed larger pale inclusions. Ultrastructurally, the SLIs were bundles of filaments which appeared thicker than neurofilaments. The SLIs tended to have central hollow spaces which were devoid of filaments. When the SLIs were clustered, fuzzy thick filaments were randomly and loosely arranged among the individual SLIs. The SLIs were histologically and ultrastructurally distinct from other inclusions such as Bunina bodies and hyaline inclusions. This unique morphology of SLIs may provide a novel perspective on the degenerative processes of the anterior horn cells in MND.
KW - Immunoelectron microscopy
KW - Motor neuron disease
KW - Skein-like inclusion
KW - Ubiquitin
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U2 - 10.1016/0022-510X(91)90112-K
DO - 10.1016/0022-510X(91)90112-K
M3 - Article
C2 - 1665504
AN - SCOPUS:0025770908
SN - 0022-510X
VL - 105
SP - 14
EP - 21
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
IS - 1
ER -