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.
Highlights
Motor neuron disease (MND) and/or amyotrophic lateral sclerosis (ALS) has been documented to accompany varying degrees of cognitive impairment more often than previously considered [1,2,23,26,31, 33,40]
Full-blown dementia associated with MND and/or ALS (MND/ALS), in other word ALS/MND with dementia (ALS/MND-D), is understood to show typically the feature of frontotemporal dementia (FTD) [28,36], and aphasic syndrome associated with MND/ALS has been reported as rare cases [4,5,6,7,10,17,22,29,38]
We reviewed the medical records of Japanese patients with MND/ALS who were admitted to our department from May, 1990 to November, 2007, and found 87 patients who fulfilled the clinical and electrophysiological criteria for clinically definite ALS according to the Revised Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Motor Neuron Disease: El Escorial revised [41]
Summary
Motor neuron disease (MND) and/or amyotrophic lateral sclerosis (ALS) has been documented to accompany varying degrees of cognitive impairment more often than previously considered [1,2,23,26,31, 33,40]. Of the cognitive changes in MND and/or ALS (MND/ALS), the most consistent feature has been reported to be executive dysfunction represented by impaired verbal fluency, based on detailed neuropsychological assessments [1,2,23,26,31,33,40]. Dementia is usually recognized to precede motor symptoms, but the opposite observation has been documented [18, 31]. It is of note that full-blown dementia or aphasic syndrome has been reported most frequently in combination with bulbar-onset MND/ALS [4,5,6,7,10,17,22, 23,29,31,33,38], suggesting a strong linkage between such extra-motor and bulbar symptoms
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