Affiliations: Department of Neurology, Showa University School of
Medicine, Tokyo, Japan | Department of Neurology, Ushioda General Hospital,
Kanagawa, Japan | Department of Neurology, Jichi Medical School,
Tochigi, Japan
Note: [] Corresponding author: Kenji Ishihara, Department of Neurology,
Showa University School of Medicine, Hatanodai 1-5-8, Shinagawa-ku, Tokyo
142-8666, Japan. Tel.: +81 3 3784 8781; Fax: +81 3 3784 1936; E-mail:
k-ishihara@mvj.biglobe.ne.jp
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.
Keywords: Amyotrophic lateral sclerosis with dementia (ALS-D), fronto-temporal lobar degeneration with TDP-43 proteinopathy
(FTLD-TDP), progressive agraphia, Exner's area
