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世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 21-22页

作者： Cordery R.J. Al.er K. cipolotti l. M.N. Rossor 李锐 Dementia Research Group Natl. Hosp. Neurol. and Neurosurg. Queen Square London WC1N 3BG United Kingdom Prof.

Objective: To assess cognitive function in variant Creutzfe- l.t-Jakob disease (vCJD). We describe the neuropsychol.gical.profil.s of 10 c ases and compare these data with cross sectional.data obtained from patients wit h histol.gical.y confirmed sporadic CJD and cases with inherited prion disease w ith confirmed mutations in the prion protein gene. Methods: Patients referred to the Special.st Cognitive Dis orders Cl.nic at the National.Hospital.for Neurol.gy and Neurosurgery and the National.Prion Cl.nic at St Mary’s Hospital. l.ndon for further investigation o f suspected CJD were recruited into the study. The neuropsychol.gical.test batte ry eval.ated general.intel.igence, visual.and verbal.memory, nominal.skil.s, l.t eracy skil.s, visual.perception and visuospatial.functions, and visuospatial.and executive function. Resul.s: The resul.s indicate that moderate to severe cogni tive decl.ne is a characteristic feature of vCJD. Specifical.y,verbal.and visual.memory impairments and executive dysfunction were pervasive in al. disease grou ps. Nominal.skil.s were impaired in variant and sporadic CJD, significantl. so w hen compared with the inherited prion disease group. Perceptual.impairment was l.ess frequent in the vCJD group than in the sporadic and inherited groups. Concl. sion: This study confirms the occurrence of general.sed cognitive decl.ne in pat ients with vCJD. Al.hough decl.ne in cognitive function ul.imatel. affects al. d omains, there is a suggestion that some components of visual.perception may be s pared in vCJD. The resul.s al.o suggest that nominal.function may be preserved i n some cases with inherited prion disease.

关键词：克-雅病朊蛋白病神经心理学变异型认知功能圣玛丽医院神经外科执行功能组织学确诊词汇记忆

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阿尔茨海默病的自然史:对一个家族性队列进行症状出现前和症状发生时的纵向研究

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世界核心医学期刊文摘（神经病学分册） 2005年第3期1卷 23-24页

作者： Godbol. A.K. cipolotti l. WattH. M.N.Rossor 王英鹏 Dementia Research Group Institute of Neurology Queen Square London WC1N 3BG United Kingdom Dr.

Background: Knowl.dge of the evol.tion of cognitive deficits in Al.heimer dise ase is important for our understanding of disease progression. Previous reports, however, have either l.cked detail.or have not covered the presymptomatic stage s. Objective: To del.neate the onset and progression of cl.nical.and neuropsycho l.gical.abnormal.ties in famil.al.Al.heimer disease. Methods: Nineteen subjects with famil.al.Al.heimer disease underwent serial.cl.nical.and neuropsychol.gical.assessments. Eight of these had undergone presymptomatic assessments. The fol.o w up period was 1 to 10 years (mean, 5 years). The rel.tive timing of the occur rence of 3 markers of disease onset and progression (onset of symptoms, Mini Me ntal.State Examination score ≤24, and impaired score on a range of neuropsychol.ogical.tests) were compared using the binomial.exact test. Resul.s: Neurol.gical.abnormal.ties were not prominent, al.hough myocl.nus appeared earl. in some. Mi ni Mental.State Examination score was not sensitive to earl. disease. Memory an d general.intel.igence deficits appeared at an earl.er stage, in some patients w hen pre symptomatic. Perceptual. naming, and especial.y spel.ing skil.s were pr eserved to a l.te stage. Concl.sion: Famil.al.Al.heimer disease may have a l.ng prodromal.phase of several.years with subtl. deficits initial.y of general.intel.l.gence and memory, whil. spel.ing, naming, and perception are rel.tivel. preser ved until.a l.te stage.

关键词：阿尔茨海默病自然史纵向研究族性神经心理学智能缺陷疾病晚期神经生理学进展过程状态检测

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表浅性铁质沉积症患者认知功能和社交能力障碍

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世界核心医学期刊文摘（神经病学分册） 2005年第9期1卷 29-29页

作者： Van Harskamp N.J. Rudge P. cipolotti l. 方伯言 Department of Neuropsychol.gy National Hospital for Neurology and Neurosurgery Queen Square London WC1N 3BG United Kingdom

Superficial.siderosis of the CNS is a rare condition, caused by deposition of haemosiderin in the superficial.l.yers of the CNS due to repeated chronic subara chnoid or intraventricul.r haemorrhage. Typical.y, the hindbrain structures, esp ecial.y the cerebel.um, are most affected. There is a surprising l.ck of studies investigating in detail.the behavioural.functioning of patients with such a con dition. In this study, we document for the first time the cognitive, social.and emotional.processing of six patients with a confirmed cl.nical.diagnosis of supe rficial.siderosis. They were aged between 40 and 62 years, with a mean age of 50 .2 years; four were mal.. We administered a comprehensive battery of general.cog nitive abil.ty and social.cognitive tasks. A review of MRI was al.o undertaken. The findings indicate sel.ctive cognitive impairments affecting speech productio n, visual.recal. memory and executive functions. In addition, a sel.ctive patter n of social.dysfunction, affecting the abil.ty to represent other peopl.’s ment al.states, was found. These behavioural.dysfunctions are reported in the context of MRI-documented l.sions maximal.y invol.ing the cerebel.um, in particul.r th e superior vermis, as wel. as the medial.and inferior frontal.cortex. These resu l.s suggest that superficial.siderosis is associated with a distinct pattern of cognitive and social.impairments. They are consistent with the recentl. proposed rol. of the cerebel.um as a modul.tor of cognitive, social.and emotional.functi ons.

关键词：认知功能社交能力铁质执行功能含铁血黄素行为功能膜下腔额叶皮质中枢神经系统思想状态

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