Bertolucci Paulo H F, Siviero Marilena O, Bueno Orlando F A, Okamoto Ivan H, Camargo Candida H P, Santos Ruth F
Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, Brazil.
Clin Invest Med. 2004 Apr;27(2):101-6.
To present a case of permanent global amnesia related to hippocampal damage. Permanent global amnesia is a very rare condition of unknown etiology; lesions restricted to the hippocampus are uncommon, which hinders investigations of this pattern of memory loss. This case is unusually well documented, as the patient underwent extensive neuropsychological evaluations.
A cheerful right-handed, 83-year-old retired electrician was first evaluated in 1990 for progressive difficulty in learning new information and in recalling events over the preceding 3-4 years. Tests over the next 5 years showed that the impairment was confined to episodic declarative memory. New verbal information could be stored only in episodic memory in a restricted manner, limited by short-term memory capacity. A relatively mild retrograde amnesia was detected. Semantic and implicit memory was spared, as were other functions evaluated. The patient's language and executive function were strikingly efficient. Magnetic resonance imaging of the brain showed bilateral atrophy of the hippocampi and amygdalae, ruling out conditions such as tumour growth. A diagnosis of permanent global amnesia was made. In the ensuing years, the retrograde amnesia worsened, but no new deficits were identified.
This case, the first with a detailed cognitive examination, is evidence of a relatively pure hippocampal pattern for memory loss in permanent global amnesia.
报告一例与海马体损伤相关的永久性全面性遗忘症病例。永久性全面性遗忘症是一种病因不明的极为罕见的病症；局限于海马体的病变并不常见，这妨碍了对这种记忆丧失模式的研究。该病例记录极为详尽，因为患者接受了广泛的神经心理学评估。
一名开朗的右利手83岁退休电工于1990年首次接受评估，原因是在学习新信息以及回忆此前3 - 4年发生的事件方面存在渐进性困难。在接下来的5年里进行的测试表明，损伤仅限于情景陈述性记忆。新的言语信息只能以受限的方式存储在情景记忆中，受短期记忆容量的限制。检测到相对较轻的逆行性遗忘。语义和内隐记忆未受影响，其他评估的功能也未受影响。患者的语言和执行功能非常高效。脑部磁共振成像显示双侧海马体和杏仁核萎缩，排除了肿瘤生长等情况。诊断为永久性全面性遗忘症。在随后的几年里，逆行性遗忘症加重，但未发现新缺陷。
该病例是首例进行详细认知检查的病例，证明了永久性全面性遗忘症中存在相对纯粹的海马体记忆丧失模式。
