Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia
The clinical significance and characteristics of writing errors in bulbar-onset amyotrophic lateral sclerosis (ALS) are not clear. We retrospectively investigated writing samples in 19 patients with bulbar-onset ALS without preceding extra-motor symptoms. Co-development of dementia and/or aphasia wa...
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| Format: | Article |
| Language: | English |
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Wiley
2008-01-01
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| Series: | Behavioural Neurology |
| Online Access: | http://dx.doi.org/10.3233/BEN-2008-0219 |
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| author | Hiroo Ichikawa Nobuyoshi Takahashi Soutaro Hieda Hideki Ohno Mitsuru Kawamura |
| author_facet | Hiroo Ichikawa Nobuyoshi Takahashi Soutaro Hieda Hideki Ohno Mitsuru Kawamura |
| author_sort | Hiroo Ichikawa |
| collection | DOAJ |
| description | The clinical significance and characteristics of writing errors in bulbar-onset amyotrophic lateral sclerosis (ALS) are not clear. We retrospectively investigated writing samples in 19 patients with bulbar-onset ALS without preceding extra-motor symptoms. Co-development of dementia and/or aphasia was also explored and single photon emission computed tomography (SPECT) images of the brain were reviewed. As a result, a high prevalence of writing errors (15 of the 19 patients) was found. Of note were isolated writing errors with neither dementia nor aphasia verified in 2 patients whose dysarthria was mild enough to evaluate spoken language. The remaining 13 patients also showed agraphia, but either dysarthria was too severe to evaluate aphasia or frontotemporal dementia (FTD)-like features co-existed. Of these patients, one who initially lacked dementia subsequently developed FTD-like features. The frequent writing errors were omission or substitution of kana letters and syntactic errors. SPECT images showed bilateral or left-side dominant hypoperfusion in the frontotemporal lobes as a consistent feature. These results show that patients with bulbar-onset ALS frequently exhibit agraphic writing errors and that these are not merely consequences of dementia or aphasia. However, these writing errors may indicate the involvement of frontotemporal language-related areas beyond the primary motor cortex. |
| format | Article |
| id | doaj-art-ac3a1d16ce854dbb92f78aa2de021272 |
| institution | Kabale University |
| issn | 0953-4180 1875-8584 |
| language | English |
| publishDate | 2008-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Behavioural Neurology |
| spelling | doaj-art-ac3a1d16ce854dbb92f78aa2de0212722025-08-20T03:39:14ZengWileyBehavioural Neurology0953-41801875-85842008-01-01203-4919910.3233/BEN-2008-0219Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or AphasiaHiroo Ichikawa0Nobuyoshi Takahashi1Soutaro Hieda2Hideki Ohno3Mitsuru Kawamura4Department of Neurology, Showa University School of Medicine, Shinagawa-ku, Tokyo, JapanDepartment of Neurology, Showa University School of Medicine, Shinagawa-ku, Tokyo, JapanDepartment of Neurology, Showa University School of Medicine, Shinagawa-ku, Tokyo, JapanDepartment of Neurology, Showa University School of Medicine, Shinagawa-ku, Tokyo, JapanDepartment of Neurology, Showa University School of Medicine, Shinagawa-ku, Tokyo, JapanThe clinical significance and characteristics of writing errors in bulbar-onset amyotrophic lateral sclerosis (ALS) are not clear. We retrospectively investigated writing samples in 19 patients with bulbar-onset ALS without preceding extra-motor symptoms. Co-development of dementia and/or aphasia was also explored and single photon emission computed tomography (SPECT) images of the brain were reviewed. As a result, a high prevalence of writing errors (15 of the 19 patients) was found. Of note were isolated writing errors with neither dementia nor aphasia verified in 2 patients whose dysarthria was mild enough to evaluate spoken language. The remaining 13 patients also showed agraphia, but either dysarthria was too severe to evaluate aphasia or frontotemporal dementia (FTD)-like features co-existed. Of these patients, one who initially lacked dementia subsequently developed FTD-like features. The frequent writing errors were omission or substitution of kana letters and syntactic errors. SPECT images showed bilateral or left-side dominant hypoperfusion in the frontotemporal lobes as a consistent feature. These results show that patients with bulbar-onset ALS frequently exhibit agraphic writing errors and that these are not merely consequences of dementia or aphasia. However, these writing errors may indicate the involvement of frontotemporal language-related areas beyond the primary motor cortex.http://dx.doi.org/10.3233/BEN-2008-0219 |
| spellingShingle | Hiroo Ichikawa Nobuyoshi Takahashi Soutaro Hieda Hideki Ohno Mitsuru Kawamura Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia Behavioural Neurology |
| title | Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia |
| title_full | Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia |
| title_fullStr | Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia |
| title_full_unstemmed | Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia |
| title_short | Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia |
| title_sort | agraphia in bulbar onset amyotrophic lateral sclerosis not merely a consequence of dementia or aphasia |
| url | http://dx.doi.org/10.3233/BEN-2008-0219 |
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