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.

中文翻译：

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Exner 区的病变是否与伴有痴呆的肌萎缩侧索硬化症的进行性失写有关？尸检病例报告。

失字症作为ALS，尤其是ALS伴痴呆（ALS-D）的一种神经心理症状，最近引起了更多的关注。然而，负责的脑损伤尚未确定。在这里，我们展示了一个 ALS-D 患者的尸检案例，该患者有明显的失语症，没有失语症，该患者也出现了与 ALS-D 相容的 TDP-43 病理学的脑脊髓变性。在运动前额叶皮质中，左中额回的尾部区域或 Exner 区域的变性和免疫组织化学病理学最明显。确保该区域在用于书写日语的汉字和假名形成中发挥关键作用，此 ALS-D 病例显示失字症和 Exner 区域强调病理性病变。