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半球卒中患者采用脑对称指数进行持续定量EEG监测

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世界核心医学期刊文摘（神经病学分册） 2005年第4期1卷 56-56页

作者： Van PuttenM.J.A.M. Tavy D.L.J 张玉龙 dept. neurol. Clin. Neurophysiol. Ziekenhuis LeyenburgLeyweg 275 2545 CH The Hague Netherlands Dr

Background and Purpose - There is increased awareness that continuous brain monitoring might benefit neurological patients, because it may allow detection of derangement of brain function in a possible reversible state, allowing early intervention. Here, we explore if quantitative continuous electroencephalography (cEEG) monitoring is technically feasible and possibly clinically relevant in patients with acute ischemic hemispheric stroke. Materials - Twenty- one consecutive patients with an acute hemispheric stroke were monitored in our stroke unit, using cEEG for 12 to 24 hours on the day of admission. EEGs were quantified using a particular measure for symmetry, the brain symmetry index (BSI). This measure was subsequently correlated with the clinical condition of the patient using the National Institute of Health Stroke Scale (NIHSS)- . Results - cEEG was technically feasible. We found a most satisfying positive correlation between the BSI and the NIHSS, with p≈ 0.86 (P neurological condition of our stroke patients. This suggests that the BSI can be used as a measure to monitor possible changes of brain function in this patient category.

关键词： EEG监测卒中单元缺血性脑卒中神经系统疾病早期干预治疗脑功能临床状态临床相关研究测量方法正相关性

MECP2基因突变患儿出现脑病和皮质性肌阵挛的早期发病

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世界核心医学期刊文摘（神经病学分册） 2005年第4期1卷 48-48页

作者： Leuzzi V. Di Sabato M.L. Zollino M. 张玉龙 dept. of Child neurol. and Psychiat. University of Rome La Sapienza Via dei Sabelli 108 00185 Rome Italy Dr

The authors report the unusual clinical and neurophysiologic features of a sporadic case of a boy carrying an 806delG mutation on the MECP2 gene. A 28- month- old boy was examined for severe developmental delay, seizures, microcephaly, breathing dysfunction, and spontaneous and evoked myoclonic jerks of upper limbs. Neurophysiologic study proved the corticalorigin of myoclonus; however, it was not associated with signs of cortical hyperexcitability. 3- Methoxy- 4- hydroxy- phenyleth- ylene glycol and valine concentrations were low in CSF.

关键词：皮质性肌阵挛 MECP2基因脑病基因突变小头畸形神经生理学上肢肌呼吸功能亚乙基

原发性成年起病型肌张力障碍家族史资料的有效性

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世界核心医学期刊文摘(神经病学分册) 2005年第2期 62-62页

作者： Martino D. ,Aniello M.S. ,Masi G. ,G. Defazio,郭俊 dept. of neurol. and Psychiat. Sci. University of Bari Piazza Giulio Cesare 11 1-70124 Bari Italy Dr.

Background: To our knowledge, no study has assessed thevalidity of family hist ory data provided by probandswith adultonset dystonia. Objective: To measure the sensitivity and specificity of interviewing patients with primary adult onset dystonia as a method for obtaining information on dystonia in first degree rela tives. Participants: Seventy probands with primary adult onset dystonia were as ked to identify first degree relatives who had dystonia. Available relatives we re then directly examined by a trained neurologist. The validity of the probands reports was tested against the neurologistsdiagnoses. Results: Among 300 fir st degree relatives who were examined, 26 received a diagnosis of dystonia. Onl y 7 of the 26 were identified by the probandsreports. Among the 274 relatives free of dystonia, the probands reported 5 as having dystonia. The probandsrepo rts therefore yielded a sensitivity of 27.0%and a specificity of 98.2%. Conclu sions: Because the family history method yields low sensitivity and incurs a ris k of misclassification, it is of limited use in family studies of adult onset d ystonia. The only valid means of ascertaining dystonia among relatives remains n eurological examination of at risk subjects.

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Ia型遗传性运动感觉神经病的临床严重程度和轴突功能障碍

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世界核心医学期刊文摘（神经病学分册） 2005年第6期1卷 30-30页

作者： Verhamme C. Van Schaik I.N. Koelman J.H.T.M. 袁海峰 dept. neurol. Clin. Neurophysiol. Academic Medical Centre University of Amsterdam PO Box 22700 1100 DE Amsterdam Netherlands

Background: Hereditary motor and sensory neuropathy type Ia (HMSN Ia) is known as a primarily demyelinating peripheral nerve disease. Evidence is accumulating that axonal involvement determines the course of the disease process. Methods: Fifty- one patients were investigated. Physical disability and impairments were scored. Nerve conduction velocities (NCVs) were used as indirect measures for myelination status and compound muscle/sensory nerve action potential (CMAP/SNAP) amplitudes served as indirect measures for axonal function. Results: Median age was 39 years (range 6- 69). Muscle weakness and sensory dysfunction was more severe in the legs than in the arms and distally more than proximally. However, more than 40 % of the patients had proximal muscle weakness in the legs. Three point grip was used as representative of combined distal arm muscle groups. CMAP amplitude was the most important independent variable in a multiple linear regression model (forward selection) to explain the relation between three point grip strength and four different features, i. e., CMAP amplitude of the abductor pollicis brevis, median nerve MNCV, gender, and duration of signs and symptoms. The severity of axonal dysfunction was nerve length- dependent and was related to the myelination status. The mild physical disability due to both muscle weakness and sensory dysfunction was also related to axonal dysfunction. Conclusions: In HMSN Ia, clinical disease severity at the impairment and disability levels is related to the severity of axonal dysfunction. Our data support the hypothesis that the myelination status is one of the factors that determine the extent of axonal dysfunction later in life. Proximal weakness of the legs is encountered in a considerable proportion of our patients.

关键词：运动感觉 Ia 临床严重程度功能障碍下肢近端肌神经传导速度正中神经肌无力髓鞘复合动作电位

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早期发病的多发性硬化症患者CSF的特征

世界核心医学期刊文摘（神经病学分册） 2005年第4期1卷 48-48页

作者： PohlD. Rostasy K. Reiber H. Hanefeld F. 张玉龙 dept. of Pediat. and Pediat. neurol. Georg- August- University Goettingen Robert- Koch- Stras- se 40 37075 Goettingen Germany Dr

The authors studied CSF characteristics in 136 patients with multiple sclerosis (MS) with a disease onset before age 16. In the initial diagnostic lumbar puncture, CSF- pleocytosis was observed in 66% , blood- CSF barrier dysfunction in 13% , and oligoclonal IgG in 92% of the early- onset MS (EOMS) patients. CSF oligoclonal IgG supports the early diagnosis of MS in childhood with a sensitivity similar to adult- onset MS.

关键词：多发性硬化症 CSF 血-脑脊液屏障 IgG 早期诊断

患家族性腺瘤性息肉病的同胞10岁前和13岁前的结肠直肠癌2例

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世界核心医学期刊文摘（儿科学分册） 2005年第10期1卷 50-50页

作者： Jerkic S. Rosewich H. Scharf J.-G. J. Grtner 虎小毅 dept. Paediatr. Paediatr. neurol. Georg August University Faculty of Medicine Robert-Koch-Strasse 40 37075 Gttingen Germany

Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that ch aracteristically presents with colon cancer in early adult life. We describe a P akistani FAP family in which two sons had an unusually early manifestation of co lorectal cancer. The index patient presented at 11 years of age with abdominal p ain, rectal bleeding and iron deficiency anaemia. Colonoscopy showed that the co lon was carpeted with a myriad of polyps. Oesophago-gastric and duodenal endosc opy revealed that polyps had also developed in the duodenum. Multiple biopsies i ndicated neoplastic lesions. The patient underwent a proctocolectomy and endosco pic duodenal mucosectomy. The diagnosis of an adenocarcinoma of the colon and fu rther adenomatous polyps with low-grade and high-grade dysplasia was confirmed by histology. Family screening including a blood test for anaemia and bowel exa mination revealed that his 12-year-old brother was also affected. Conclusion: Children with familial adenomatous polyposis are at risk for colon cancer and em phasise the need for early tumour recognition. Gastrointestinal symptoms in chil dren should be thoroughly evaluated and standard screening for colonic polyposis should be performed in all individuals with a positive family history and/or kn own mutations in cancer-associated genes, particularly in children who are unde r 10 years of age.

关键词：结肠直肠癌结肠切除术结肠腺癌腺瘤性息肉结肠癌内窥镜检成年早期贫血状况肠道症状早期肿瘤

帕金森病、绝经期提前与线粒体DNA聚合酶γ突变:临床及分子遗传学研究

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世界核心医学期刊文摘（神经病学分册） 2005年第2期1卷 6-6页

作者： Luoma P. Melberg A. Rinne J.O. A. Suomalainen 黄卫东 dept. neurol. and Prog. of Neurosci. Biomedicum Helsink i Helsinki Univ. Helsinki Univ. C. Helsinki Finland Dr.

Background Mutations in the gene encoding mitochondrial DNA polymerase γ.(POL G), the enzyme that synthesises mitochondrial DNA (mtDNA), have been associated with a mitochondrial disease autosomal dominant or recessive progressive extern al ophthalmoplegia and multiple deletions of mtDNA. Mitochondrial dysfunction is also suspected to participate in the pathogenesis of Parkinsons disease. Howe ver, no primary gene defects affecting mitochondrial proteins causing mendelian transmission of parkinsonism have been characterised. We aimed to analyse the ge ne sequence of POLG in patients with progressive external ophthalmoplegia and th eir healthy relatives. Methods In seven families of various ethnic origins we as sessed patients with progressive external ophthalmoplegia and unaffected individ uals by clinical, biochemical, morphological, and molecular genetic characterisa tion and positron emission tomography (PET). Findings We recorded mutations in P OLG in members of all seven families. Clinical assessment showed significant cos egregation of parkinsonism with POLG mutations (p < 0 0001), and PET findings w ere consistent with dopaminergic neuron loss. Post mortem examination in two in dividuals showed loss of pigmented neurons and pigment phagocytosis in substanti a nigra without Lewy bodies. Furthermore, most women with progressive external o phthalmoplegia had early menopause before age 35 years. The POLG gene defect r esulted in secondary accumulation of mtDNA deletions in patientstissues. Inter pretation Dysfunction of mitochondrial POLG causes a severe progressive multisys tem disorder including parkinsonism and premature menopause, which are not typic

关键词：帕金森病聚合酶 DNA 分子遗传学正电子发射扫描线粒体蛋白多巴胺能线粒体功能运动障碍性疾病眼外肌麻痹

硬脑膜移植相关克-雅病等朊蛋白病的周围神经受累

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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 21-21页

作者： IshidaC. Okino S. Kitamoto T. Yamada M. 李锐 dept. neurol. and Neurbio. of Aging Kanazawa Univ.Grad. Sch. Med. Sci. Takara-machi 13-1 KanazawaIshikawa 920-8640 Japan Dr.

Objective: To investigate abnormal prion protein (PrP) deposition in the perip heral nervous system (PNS) in human prion diseases. Methods: Eight patients with prion diseases were examined:three with sporadic Creutzfeld-Ja- kob disease (sCJD),two with dural graft associated CJD (dCJD), one with Gerstm ann-Strussler-Scheinker disease (GSS) with a PrP P102L mutation (GSS102), an d two with a P105L mutation (GSS105).An atypical case of sCJD with PrP plaques i n the brain presented clinically with peripheral neuropathy, and showed demyelin ation in 12%of the teased fibres of the sural nerve. The PNS was investigated b y immunohistochemical and western blotting analyses of PrP. Results: In immunohi stochemical studies,granular PrP deposits were detected in some neurones of dors al root ganglia and a few fibres of peripheral nerves and spinal posterior roots in one sCJD and two dCJD patients, but not in GSS102 or GSS105 patients. The at ypical case of sCJD with peripheral neuropathy showed no obvious PrP deposition in the *** blotting analysis of the PNS from the dCJD patients reveal ed a small amount of protease K resistant PrPin the dorsal root ganglia and peri pheral nerves. Conclusions:Abnormal PrP deposition occurs in the dorsal root gan glia and peripheral nerves in sCJD and dCJD. The PrP deposits in the PNS are not correlated with clinical manifestation of peripheral neuropathy in CJD.

关键词：克-雅病朊蛋白病硬脑膜腓神经免疫印记颗粒样脱髓鞘背根神经节位点突变神经根

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慢性中风的皮质间耦联与康复的相关性研究

世界核心医学期刊文摘（神经病学分册） 2005年第1期1卷 49-49页

作者： Strens L.H.A. Asselman P. PogosyanA. P. Brown 张玉龙 Sobell dept. Motor Neurosci./M. D. Institute of Neurology Natl. Hosp. for Neurol./Neurosurg. 8- 11 Queen Square London WCIN 3BG United Kingdom Dr.

Background: The mechanisms behind motor recovery from stroke are not clearly understood. Functional imaging studies have demonstrated task related brain activation in several motor areas, but few studies have attempted to correlate this with stroke outcome. Moreover, these studies have focused on how motor areas may individually contribute to compensation. Here, the authors investigate whether different cortical areas interact to form dynamic assemblies that may then compensate for disability. Metho ds: The authors investigated corticocortical coherence in 16 healthy subjects and 25 patients with chronic stroke involving one cerebral hemisphere and having varying degrees of motor recovery. Scalp EEG was recorded at rest and while right handed subjects performed a unimanual grip task. The degree of functional recovery after stroke was assessed using a range of outcome measures. Results: Compared with healthy subjects, hand related asymmetries in task related EEG EEG coherence were increased between mesial and lateral frontal regions of the affected hemisphere, over mesial frontal regions, and over lateral frontal areas of the unaffected hemisphere when patients with stroke gripped with their affected hand. Mesial hand related asymmetries in task related power and coherence were negatively correlated with recovery. Conclusion: Increases in task related coupling between cortical areas may dynamically compensate for brain damage after stroke. Some of this increased coupling, particularly that over mesial frontal areas, decreases as patients make a functional recovery.

关键词：耦联额区功能影像学功能恢复健康受试者大脑半球不对称性右利手动态联系运动区