Poeck K, Luzzatti C
Department of Neurology, RWTH Aachen, FRG.
Brain. 1988 Feb;111 ( Pt 1):151-68. doi: 10.1093/brain/111.1.151.
Three patients are described presenting with a slowly progressive aphasic disorder associated with degenerative cortical disease. The symptoms began in the presenium and the length of illness was 4 to 5 years. The language disorder corresponded in all patients to a severe form of amnesic aphasia but a moderate to marked semantic breakdown was also found. Formal language examination was complemented by extensive neuropsychological testing. This revealed a severe deficit in language-dependent cognitive tasks. The patients were given a follow-up language and neuropsychological examination. In addition to the deterioration of language functions, a significant decline was observed in nonverbal intelligence tasks even though their level of performance still remained within the normal range. Follow-up with standardized intelligence tests might detect a trend towards generalized dementia in similar cases. This would mean that these patients should be considered as presenting with slowly progressive aphasia preceding generalized dementia.
本文描述了3例患有与退行性皮质疾病相关的缓慢进展性失语症的患者。症状始于中年前期，病程为4至5年。所有患者的语言障碍均符合严重形式的遗忘性失语，但也发现了中度至重度的语义障碍。通过广泛的神经心理学测试对形式语言检查进行了补充。这揭示了语言依赖性认知任务中的严重缺陷。对患者进行了随访语言和神经心理学检查。除了语言功能恶化外，非语言智力任务也出现了显著下降，尽管其表现水平仍在正常范围内。对类似病例进行标准化智力测试随访可能会发现向全身性痴呆发展的趋势。这意味着这些患者应被视为在全身性痴呆之前出现缓慢进展性失语症。
