Agraphia in Amyotrophic Lateral Sclerosis with Frontotemporal Lobe Degeneration
نویسندگان
چکیده
Frontotemporal lobe degeneration (FTLD) refers to a neurodegenerative dementia syndrome, which could be clinically classified into behavioral and language variant. Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder involving both upper motor neuron (UMN) and lower motor neuron (LMN), eventually leading to muscle atrophy and weakness, bulbar palsy, and respiratory failure. Once regarded as two independent entities, they now have been embraced into one continuum because of their clinical and pathological overlaps, namely ALS‐FTLD and TAR DNA‐binding protein 43. Deterioration in personality and behaviors serves as core feature in diagnosis while it seems difficult to identify language deficit due to dysarthria. Agraphia has been reported to appear before frank dementia/aphasia,[1] making it a potential clue to detect FTLD in the context of ALS. However, researches about writing ability of Chinese patients with ALS/FTLD spectrum disease turned to be limited. Hence, we described writing errors in a Chinese‐speaking patient with ALS‐FTLD.
منابع مشابه
Is Lesion of Exner’s Area Linked to Progressive Agraphia in Amyotrophic Lateral Sclerosis with Dementia? An Autopsy Case Report
Agraphia, as a neuropsychological symptom of ALS, especially ALS with dementia (ALS-D), has recently attracted more attention. However, the brain lesion responsible has not been identified. Here we present an autopsy case of ALS-D of a patient with obvious agraphia, without aphasia, that also presented cerebrospinal degeneration with TDP-43-pathology compatible with ALS-D. Of the pre-motor fron...
متن کاملAgraphia 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 was also explored and single photon emission computed tomography (SPECT) images of the brain were r...
متن کاملEstablishing subtypes of the continuum of frontal lobe impairment in amyotrophic lateral sclerosis.
This review summarizes recent advances in our understanding of the cognitive changes seen in patients with amyotrophic lateral sclerosis (ALS). Emphasis is placed on identifying and diagnosing subtypes of ALS patients with a continuum of frontotemporal impairment. The reviewed literature focuses on progress made in the past 20 years, with an emphasis on studies measuring abnormalities in ALS pa...
متن کامل