title: Is lesion of Exner's area linked to progressive agraphia in amyotrophic lateral sclerosis with dementia? An autopsy case report.
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.
authors.affiliation: Department of Neurology, Showa University School of Medicine, Shinagawaku, Tokyo, Japan. kishihara@mvj.biglobe.ne.jp
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishihara
authors.forename: Hiroo
authors.initials: H
authors.lastname: Ichikawa
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Jun'ichi
authors.initials: J
authors.lastname: Shiota
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawamura
mesh_terms: D000368:Aged; D000381:Agraphia / Q000209:etiology* / Q000473:pathology*; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000473:pathology*; D001344:Autopsy; D001921:Brain / Q000473:pathology; D001943:Breast Neoplasms / Q000150:complications; D004268:DNA-Binding Proteins / Q000378:metabolism; D003704:Dementia / Q000150:complications* / Q000523:psychology*; D004401:Dysarthria / Q000209:etiology; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D006236:Handwriting; D006801:Humans; D007150:Immunohistochemistry; D007807:Language Tests; D011712:Pyramidal Tracts / Q000473:pathology; D013060:Speech; D013116:Spinal Cord / Q000473:pathology
chemical_list: D004268:DNA-Binding Proteins