Numerous conglomerate inclusions in slowly progressive familial amyotrophic lateral sclerosis with posterior column involvement
| Autor: | Yasuhiro Yamamura, Hitoshi Ohishi, Shigenobu Nakamura, Kouichi Noda, Kohtaro Asayama, Chigusa Watanabe, Sadao Katayama, Shigeo Murayama, Kouki Inai, Takashi Nishisaka |
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| Rok vydání: | 1999 |
| Předmět: |
Hypoglossal nucleus
Accessory cuneate nucleus Thalamus SOD1 Fatal Outcome Superoxide Dismutase-1 Anterior Horn Cells Neurofilament Proteins medicine Humans Genetic Predisposition to Disease Ubiquitins Trigeminal nerve Inclusion Bodies Chemistry Superoxide Dismutase Amyotrophic Lateral Sclerosis Brain Anatomy Middle Aged Immunohistochemistry Pedigree medicine.anatomical_structure Dentate nucleus Neurology Spinal Cord Female Neurology (clinical) Nucleus Motor cortex |
| Zdroj: | Journal of the neurological sciences. 171(1) |
| ISSN: | 0022-510X |
| Popis: | A 59-year-old woman with slow progression of the loss of motor function and predominant lower motor manifestation during a 14-year period showed familial amyotrophic lateral sclerosis (fALS) with posterior column involvement, neuropathologically. Conglomerate inclusions (CIs) were observed in the remaining neurons in various areas, including the spinal anterior horn, posterior horn, Clark's column, accessory cuneate nucleus, tegmental reticular formation, motor nucleus of the trigeminal nerve, nucleus of the facial nerve, hypoglossal nucleus, medial nucleus of the thalamus, dentate nucleus, and motor cortex (Betz cells). Immunohistochemically, it was newly identified that the CIs showed marked immunoreactions with antibodies to phosphorylated and non-phosphorylated neurofilaments and to 64, 120, and 200 kD neurofilaments. The CIs were partially immunoreactive with the anti-ubiquitin antibody, although they reacted only weakly (or not at all) with anti-Cu/Zn superoxide dismutase (SOD1) antibody. Ultrastructurally, the CIs were comprised of neurofilaments. These data suggest that this case might have been different from an example of fALS with Ile 113 Thr mutation in the SOD1 gene. |
| Databáze: | OpenAIRE |
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