Abstract
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 frontal lobe cortices, degeneration and immuno-histochemical pathology were most obvious in the caudal area of the left middle frontal gyrus, or Exner’s area. Assuring this area plays a pivotal role in the kanji and kana formation used in writing the Japanese language, this case of ALS-D showed both agraphia and Exner's area stressed pathological lesions. It may thus be the first case to indicate an intimate relationship between the neuropsychological symptoms and an associated lesion for ALS-D.
Highlights
Amyotrophic lateral sclerosis with dementia (ALSD) is a nosological condition presenting motor neuron disease (MND) and dementia
The clinical features of dementia in ALS with dementia (ALS-D) are of the frontal lobe type, and ALS-D is located within a framework of frontotemporal lobar degeneration (FTLD) [1]
The presented case features a combination of progressive pseudobulbar palsy, frontal lobe type dementia, pyramidal tract signs and progressive amyotrophy
Summary
Amyotrophic lateral sclerosis with dementia (ALSD) is a nosological condition presenting motor neuron disease (MND) and dementia. The clinical features of dementia in ALS-D are of the frontal lobe type, and ALS-D is located within a framework of frontotemporal lobar degeneration (FTLD) [1]. Analysis of language function in ALS-D is considered difficult to carry out, due to severe bulbar palsy, we previously reported that writing disorder may exist in the early stage [2]. In Japanese, describes writing disorder in a patient with ALS-D [3]. We describe an autopsied case of ALS-D, present-. Ing progressive agraphia without aphasia, and discuss its clinicopathological relationship
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
