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世界核心医学期刊文摘（神经病学分册） 2005年第1期1卷 50-51页

作者： Blass D.M. hatanpaa k.j. Brandt j. 张玉龙 johns Hopkins Hospital Dept. of Psychiat. and Behav. Sci. 600 North Wolfe Street Baltimore MD 21287- 7279 United States Dr.

Objective: To characterize the clinical course of patholo gically diagnosed hippocampal sclerosis dementia (HSD). Background: Dementia associated with HSD is incompletely characterized. Previous studies suggest similarities to both Alzheimer disease (AD) and frontotemporal dementia (FTD). Methods: Case control analysis of the clinical course of patients with HSD, FTD, and AD from a neuropathology autopsy series conducted by a university hospital. Case histories were reviewed. Cumulative prevalence of behavioral, cognitive, psychiatric, and language symptoms were compared between groups, as was time of symptom onset. Clinical diagnostic criteria for FTD and AD were applied to case histories. Sensitivity and specificity of clinical FTD diagnostic criteria (Report of theWork Group on FTD and Pick’ s disease) were computed. Results: Cumulative prevalence of symptoms in HSD wasmost similar to that of FTD and differed from AD. Behavioral abnormalities such as decreased grooming and inappropriate behavior were more prevalent in HSD and FTD than AD. Hyperorality, inappropriate behavior, and decreased interest had earlier onset in HSD and FTD. Cognitive symptoms of disorientation, dyscalculia, apraxia, and agnosia were more prevalent in AD, as were psychiatric symptoms of hallucinations, delusions, and aggression. Most HSD patients met diagnostic criteria for FTD. Criteria sensitivity was 64.0% and specificity was 73.7% . Conclusions: FTD is a clinical syndrome associated with heterogeneous neuropathology. The clinical course of HSD is more similar to that of FTD than AD. These findings, together with the neuropathologic data presented in the accompanying article, support expanding the scope of FTD (Pick complex) to include HSD.

关键词：额颞性痴呆阿尔茨海默病硬化性认知障碍额颞痴呆累积发病率病理学确诊神经病理临床综合征病例对照分析

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大部分海马硬化性痴呆患者可表现为额颞性痴呆

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世界核心医学期刊文摘（神经病学分册） 2005年第1期1卷 56-56页

作者： hatanpaa k.j. Blass D.M. Pletnikova O. 高方 Department of Pathology UT Southwestern Medical School 5323 Harry Hines Blvd. Dallas TX 75390- 9073 United States Dr.

Hippocampal sclerosis dementia (HSD) is a disease of unknown etiology and pathogenesis. To determine whether HSD cases could be reclassified as variants of frontotemporal dementia (FTD), a heterogeneous group of disorders, 18 brain autopsy cases previously diagnosed as HSD were re evaluated. In 11 cases, ubiquitinated neuronal inclusions, similar to those of motor neuron disease inclusion dementia (MNDID), were found. Brain levels of soluble and insoluble tau were normal. Most patients with pathologic findings of HSD may actually have the MNDID variant of FTD.

关键词：额颞性痴呆硬化性运动神经元病变异型例生不可溶性包涵体尸检结果机能紊乱发病机制

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