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Amyotrophic lateral sclerosis with neuronal intranuclear protein inclusions.
- Source :
-
Acta neuropathologica [Acta Neuropathol] 2004 Jul; Vol. 108 (1), pp. 81-7. Date of Electronic Publication: 2004 Apr 27. - Publication Year :
- 2004
-
Abstract
- A 46-year-old patient developed amyotrophic lateral sclerosis (ALS) characterized by rapid progression. She needed respiratory assistance after a course of 9 months. She died 4.5 years after onset. Autopsy showed dramatic atrophy of the spinal cord, sparing only the posterior tracts, associated with neuronal loss and astrogliosis in various areas including the anterior horns, motor cortex, striatum, thalamus, and substantia nigra. Ubiquitin immunohistochemistry showed rare skein-like inclusions in the surviving spinal and medullary motor neurons. Eosinophilic inclusions were found in the nuclei of pyramidal neurons in the hippocampus. These inclusions were immunoreactive to antibodies against ubiquitin, promyelocytic leukemia gene product, proteasome, and ataxin-3. They were not immunoreactive to antibodies against tau, cystatin C, neurofilament, alpha-synuclein, SOD-1, and polyglutamine (1C2), and were not stained by ethidium bromide. Similar inclusions were found in the motor cortex. The immunoreactivity of the inclusions was similar to that encountered in diseases associated with CAG repeats, except for the negativity of the immunolabelling with 1C2. At the ultrastructural level, the nuclear inclusions were made of straight filaments (10-12 nm in diameter) arranged at random, reminiscent of the polyglutamine intranuclear hyaline inclusions.
- Subjects :
- Ataxin-3
Cysteine Endopeptidases metabolism
Female
Humans
Immunohistochemistry methods
Intranuclear Inclusion Bodies ultrastructure
Microscopy, Electron methods
Middle Aged
Multienzyme Complexes metabolism
Neoplasm Proteins metabolism
Nerve Tissue Proteins metabolism
Neurons ultrastructure
Nuclear Proteins metabolism
Postmortem Changes
Promyelocytic Leukemia Protein
Proteasome Endopeptidase Complex
Repressor Proteins
Staining and Labeling
Transcription Factors metabolism
Tumor Suppressor Proteins
Ubiquitin metabolism
Amyotrophic Lateral Sclerosis metabolism
Hippocampus pathology
Intranuclear Inclusion Bodies metabolism
Neurons metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0001-6322
- Volume :
- 108
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Acta neuropathologica
- Publication Type :
- Academic Journal
- Accession number :
- 15114487
- Full Text :
- https://doi.org/10.1007/s00401-004-0855-x