初期に失行が目立った大脳皮質基底核変性症の1剖検例

An Autopsy Case of Corticobasal Degeneration with Notable Early Onset Apraxia: A Case Report and Literature Review Focused on Apraxia

はじめに

　失行を初期症状とし，それが緩徐に進行する病態は進行性失行，原発性進行性失行と呼ばれるが，詳細な失行の記載がある剖検報告は少ない^1-4)。本報告では両側性失行を伴う大脳皮質基底核変性症（corticobasal degeneration：CBD）症例の臨床・病理学的所見を提示し，その関係を考察する。なお，本報告での「失行」は観念性失行および観念運動性失行を指し，肢節運動失行を含まない。

Abstract

　We report the autopsy case of a 74-year-old woman. Onset of gait disturbance and left-side dominant bilateral motor disturbance in the patient led to bilateral progressive apraxia. This was associated with a decline in motor imagery, right-side dominant atrophy of the central sulcus region, and a decrease in cerebral blood flow during illness. She died of respiratory failure that had progressively worsened over a 9-year period. Pathologically, she exhibited right-side dominant cerebral atrophy; neuronal loss, gliosis, and astrocytic plaques were mainly present in the frontal lobe. She was subsequently diagnosed with corticobasal degeneration (CBD). The premotor and primary motor areas revealed marked degeneration; in addition, severe myelin pallor was observed in these regions, and it was suggested that such pathological features were responsible for the apraxia. We believe the present case is valuable since very few reports have provided a detailed description of clinicopathological apraxia in association with CBD. (Received: October 9, 2012, Accepted: February 20, 2013)