Agraphia in bulbar-onset amyotrophic lateral sclerosis: not merely a consequence of dementia or aphasia.
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| Abstract |
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.
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| Authors | Hiroo Ichikawa, Nobuyoshi Takahashi, Soutaro Hieda, Hideki Ohno, Mitsuru Kawamura |
| Journal | Behavioural neurology (Behav Neurol) Vol. 20 Issue 3 Pg. 91-9 ( 2008) ISSN: 1875-8584 [Electronic] Netherlands |
| PMID | 19641246 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't) |
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