Amyotrophic Lateral Sclerosis, A Neuropathological Study
Scientific Background: Amyotrophic lateral sclerosis (ALS) is a neurodejenerative disorder of unknown cause characterized by degeneration and death of motor neurons. The diagnosis requires clinical evidence of both upper and lower motor neuron dysfunction and diagnostic tests to exclude other di...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Galenos Yayinevi
2005-02-01
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| Series: | Türk Nöroloji Dergisi |
| Subjects: | |
| Online Access: | https://www.journalagent.com/tjn/pdfs/TJN_11_1_54_62.pdf |
| _version_ | 1797907310936326144 |
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| author | Geysu Karlıkaya Arthur P. Hays |
| author_facet | Geysu Karlıkaya Arthur P. Hays |
| author_sort | Geysu Karlıkaya |
| collection | DOAJ |
| description | Scientific Background: Amyotrophic lateral sclerosis (ALS) is a
neurodejenerative disorder of unknown cause characterized by degeneration
and death of motor neurons. The diagnosis requires clinical evidence of
both upper and lower motor neuron dysfunction and diagnostic tests to
exclude other disorders with similar symptomatology. Pathologically ALS
is characterized by loss of motor nerve cells in the spinal cord, brainstem
and primary motor cortex accompanied by degeneration of the corticospinal
tracts. A variety of inclusions have been described as major pathological
features in ALS and other neurodegenerative disorders, these inclusions
clarify the diagnosis and can provide insight into biochemical pathways
leading to cell death.
Objectives: This study is a review of the clinical and neuropathological
features of sporadic ALS with special attention on two of the most common
inclusions; Bunina bodies and ubiquitin immuonreactive (ub-ir) inclusions.
Material and Methods: We retrospectively analysed the clinical features
of 11 autopsy confirmed ALS patients and classified the patients
according to the revised El Escorial criteria. We also evaluated the
motor cortex, hypoglossal nucleus and spinal cord sections from
autopsies of these ALS patients and 7 controls, focusing on the specificity
of Bunina bodies and ub-ir inclusions.
Results: 4 patients qualified for clinically probable ALS, 1 patient for
clinically probable-laboratory supported ALS, 5 patients for progressive
spinal muscular atrophy and 1 patient for progressive bulbar palsy.
Both Bunina bodies and ub-ir inclusions were found in most of these
cases, %81.8 and %90.9 respectively. No Bunina bodies were seen
in the control cases while ub-ir inclusions were seen in 3 control cases.
Conclusions: This study confirms the specificity of Bunina bodies in
sporadic ALS. Since ub-ir inclusions were also seen in a few of the
controls, although they are characteristic of sporadic ALS they are not
entirely specific. |
| first_indexed | 2024-04-10T10:34:33Z |
| format | Article |
| id | doaj.art-7e2c6581429b41a5a5b00f8f8abbfe39 |
| institution | Directory Open Access Journal |
| issn | 1301-062X 1309-2545 |
| language | English |
| last_indexed | 2024-04-10T10:34:33Z |
| publishDate | 2005-02-01 |
| publisher | Galenos Yayinevi |
| record_format | Article |
| series | Türk Nöroloji Dergisi |
| spelling | doaj.art-7e2c6581429b41a5a5b00f8f8abbfe392023-02-15T16:20:58ZengGalenos YayineviTürk Nöroloji Dergisi1301-062X1309-25452005-02-011115462Amyotrophic Lateral Sclerosis, A Neuropathological StudyGeysu Karlıkaya0Arthur P. Hays1Haydarpafla Numune E¤itim ve Araflt›rma Hastanesi, 2. Nöroloji Klini¤i, ‹STANBULColumbia Üniversitesi, Patoloji Departman›, Nöropatoloji Divizyonu, NEWYORKScientific Background: Amyotrophic lateral sclerosis (ALS) is a neurodejenerative disorder of unknown cause characterized by degeneration and death of motor neurons. The diagnosis requires clinical evidence of both upper and lower motor neuron dysfunction and diagnostic tests to exclude other disorders with similar symptomatology. Pathologically ALS is characterized by loss of motor nerve cells in the spinal cord, brainstem and primary motor cortex accompanied by degeneration of the corticospinal tracts. A variety of inclusions have been described as major pathological features in ALS and other neurodegenerative disorders, these inclusions clarify the diagnosis and can provide insight into biochemical pathways leading to cell death. Objectives: This study is a review of the clinical and neuropathological features of sporadic ALS with special attention on two of the most common inclusions; Bunina bodies and ubiquitin immuonreactive (ub-ir) inclusions. Material and Methods: We retrospectively analysed the clinical features of 11 autopsy confirmed ALS patients and classified the patients according to the revised El Escorial criteria. We also evaluated the motor cortex, hypoglossal nucleus and spinal cord sections from autopsies of these ALS patients and 7 controls, focusing on the specificity of Bunina bodies and ub-ir inclusions. Results: 4 patients qualified for clinically probable ALS, 1 patient for clinically probable-laboratory supported ALS, 5 patients for progressive spinal muscular atrophy and 1 patient for progressive bulbar palsy. Both Bunina bodies and ub-ir inclusions were found in most of these cases, %81.8 and %90.9 respectively. No Bunina bodies were seen in the control cases while ub-ir inclusions were seen in 3 control cases. Conclusions: This study confirms the specificity of Bunina bodies in sporadic ALS. Since ub-ir inclusions were also seen in a few of the controls, although they are characteristic of sporadic ALS they are not entirely specific.https://www.journalagent.com/tjn/pdfs/TJN_11_1_54_62.pdfAmyotrophic lateral sclerosisBunina bodiesubiquitin |
| spellingShingle | Geysu Karlıkaya Arthur P. Hays Amyotrophic Lateral Sclerosis, A Neuropathological Study Türk Nöroloji Dergisi Amyotrophic lateral sclerosis Bunina bodies ubiquitin |
| title | Amyotrophic Lateral Sclerosis, A Neuropathological Study |
| title_full | Amyotrophic Lateral Sclerosis, A Neuropathological Study |
| title_fullStr | Amyotrophic Lateral Sclerosis, A Neuropathological Study |
| title_full_unstemmed | Amyotrophic Lateral Sclerosis, A Neuropathological Study |
| title_short | Amyotrophic Lateral Sclerosis, A Neuropathological Study |
| title_sort | amyotrophic lateral sclerosis a neuropathological study |
| topic | Amyotrophic lateral sclerosis Bunina bodies ubiquitin |
| url | https://www.journalagent.com/tjn/pdfs/TJN_11_1_54_62.pdf |
| work_keys_str_mv | AT geysukarlıkaya amyotrophiclateralsclerosisaneuropathologicalstudy AT arthurphays amyotrophiclateralsclerosisaneuropathologicalstudy |