Pure hippocampal sclerosis - A rare cause of dementia mimicking Alzheimer's disease
Pure hippocampal sclerosis - A rare cause of dementia mimicking Alzheimer's disease
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DOI：
10.1212/wnl.54.4.843
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发表时间：
2000-02-22
期刊：
影响因子：
9.9
通讯作者：
Frey, WH
中科院分区：
文献类型：
--
作者：
Ala, TA;Beh, GO;Frey, WH
目的：识别因单纯海马硬化（HS）导致痴呆的患者，确定他们是否有低血压或缺氧病史，并将单纯HS患者的临床特征与无HS的阿尔茨海默病（AD）对照组进行比较。
方法：在一项回顾性研究中，作者回顾了1978年至1996年其痴呆脑库接收的所有1771例病例，以识别那些单纯HS患者，单纯HS定义为在无其他明显痴呆疾病（如阿尔茨海默病改变）的情况下，海马结构的CA1区和下托出现严重变性和神经胶质增生。对照组包括同期接收的所有无HS、无梗死或其他痴呆疾病的重度AD患者。
结果：确定了7例单纯HS病例（0.4%）。在痴呆发病时，均无晕厥、低血压或缺氧发作的相关报告。6例以记忆力减退为主要首发症状，且均逐渐发展为痴呆。确定了45例无HS的AD患者作为对照组。两组在性别、发病年龄、血管疾病危险因素、脑血管疾病症状、镇静药物治疗、抑郁症治疗或养老院安置方面无明显临床差异。单纯HS患者有心脏病更普遍且病程更短的趋势。
结论：在我们的痴呆患者中，单纯海马硬化（HS）仅占0.4%。临床上，7例单纯HS患者与我们的AD对照组相似。需要进一步研究以确定HS的病因以及为什么HS似乎类似于AD。
Objectives: To identify patients with pure hippocampal sclerosis (HS) as a cause of dementia, to determine whether they have had histories of hypotension or hypoxia, and to compare the clinical features of patients with pure HS with a control group of AD patients without HS. Methods: In a retrospective study, the authors reviewed all 1771 cases received in their dementia brain bank from 1978 through 1996 to identify those patients with pure HS, defined as severe degeneration and gliosis of the CA1 sector and subiculum of the hippocampal formation in the absence of other significant dementing disease such as Alzheimer's changes. The control group included all patients received during the same period with severe AD without HS, infarcts, or other dementing disease. Results: Seven pure HS cases (0.4%) were identified. None had any episodes of syncope, hypotension, or hypoxia reported in association with dementia onset. Six had memory loss as the primary presenting symptom, and all became progressively demented. Forty-five AD patients without HS were identified for the control group. There were no clear clinical differences between the two groups with regard to sex, age at onset, risk factors for vascular disease, symptoms of cerebrovascular disease; treatment with tranquilizing medications, treatment for depression, or nursing home placement. There was a tendency for heart disease to be more prevalent and the duration of illness to be shorter in the patients with pure HS. Conclusions: Pure hippocampal sclerosis (HS) occurred in only 0.4% of our dementia patients. Clinically, the seven patients with pure HS were similar to our AD control group. Further research is needed to determine the causes of HS and why HS appears to mimic AD.