Shintaku Masayuki, Oyanagi Kiyomitsu, Kaneda Daita
Department of Pathology, Osaka Red Cross Hospital, Tennoji, Osaka 543-8555, Japan.
Neuropathology. 2007 Jun;27(3):295-9. doi: 10.1111/j.1440-1789.2007.00763.x.
An autopsy case of amyotrophic lateral sclerosis with dementia (ALS-D) showing clinically overt parkinsonism and severe degeneration of the substantia nigra is reported. The patient was a 78-year-old man who died after a 2-year clinical course characterized by parkinsonism that was responsive to Levodopa (L-DOPA) treatment. Motor neuron symptoms and dementia were not apparent ante-mortem. The autopsy demonstrated the severe degeneration of the substantia nigra without alpha-synucleinopathy-related changes. Finely granular mineralization of necrotic neurons was a unique finding in the substantia nigra. The mild loss of spinal anterior horn cells, the appearance of several Bunina bodies and the degeneration of the hippocampal subiculum and temporal cortex were also noted. A small number of ubiquitinated intra-cytoplasmic inclusions were found in neurons of the dentate fascia of the hippocampus and the temporal and frontal cortices. Although the degeneration of the substantia nigra is a common finding in ALS-D, patients seldom develop clinically overt parkinsonism. This case indicates that patients with ALS-D rarely present with predominantly parkinsonian clinical features and these symptoms and signs can be improved by L-DOPA treatment.
报告了一例肌萎缩侧索硬化症合并痴呆（ALS-D）的尸检病例，该病例表现出临床上明显的帕金森综合征以及黑质严重变性。患者为一名78岁男性，在经历了以帕金森综合征为特征、对左旋多巴（L-DOPA）治疗有反应的2年临床病程后死亡。运动神经元症状和痴呆在生前并不明显。尸检显示黑质严重变性，无与α-突触核蛋白病相关的改变。坏死神经元的细颗粒状矿化是黑质中的一个独特发现。还注意到脊髓前角细胞轻度丢失、出现一些布尼纳小体以及海马下托和颞叶皮质变性。在海马齿状回以及颞叶和额叶皮质的神经元中发现了少量泛素化的胞质内包涵体。虽然黑质变性在ALS-D中是常见发现，但患者很少出现临床上明显的帕金森综合征。该病例表明，ALS-D患者很少以帕金森为主的临床特征出现，且这些症状和体征可通过L-DOPA治疗得到改善。
