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Objective Linguistic problem is common in Huntington's disease (HD) patients. It has been studied before in native speakers of alphabetic languages, such as English. As a hieroglyphic language, Chinese differs from alphabetic languages in terms of phonology, morphology, semantics and syntax. We aimed to investigate the linguistic characteristics of manifest HD in native speakers of Mandarin. Meanwhile, we expected to explore the linguistic differences associated with cortical or subcortical pathology.Methods Five HD patients and five Alzheimer's disease (AD) patients matched in age, gender, disease course and educational level were enrolled. All the participants were Mandarin native speakers. All finished history inquiry, physical examination, basic test, genetic test and neuropsychological assessment. Language evaluation was performed by Aphasia Battery of Chinese.Results HD patients had a mean disease course of 5.4±2.97 (range, 2-10) years. They showed a linguistic disorder close to transcortical motor aphasia. They exhibited prominent phonological impairment, as well as slight semantic and syntactic abnormality. Tonic errors were found in speech. Character structural errors and substitutions were detected in writing. In comparison, AD patients showed a more severe linguistic impairment, characterized by global aphasia with more semantic errors. Conclusion Mandarin-speaking HD patients have a transcortical motor aphasia-like disturbance with prominent phonological impairment, whereas AD patients have a more severe global aphasia with salient semantic impairment.
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<p><b>OBJECTIVE</b>To explore the correlations between serum uric acid (UA) levels and the clinical and cerebrospinal fluid (CSF) parameters of multiple sclerosis (MS).</p><p><b>METHODS</b>The medical reports of 47 MS patients admitted to Peking Union Medical College Hospital during 2008 and 2010 were reviewed. And 49 age- and gender-matched cerebral infarction patients were enrolled as control. The mean serum UA level of the MS patients was compared with that of the control group. The correlations between the UA levels and the clinical parameters including gender, disease duration, relapse rate, and disease disabilities as assessed by the Expanded Disability Status Scale score, were explored. Forty-one patients had CSF examinations. The correlations between the UA levels and the CSF parameters reflecting inflammation and tissue damage, including CSF protein, white blood cell count, oligoclonal band, 24-hour IgG index, and myelin basic protein, were also investigated.</p><p><b>RESULTS</b>The mean serum UA level in the MS patients was lower than that in the control group (247.75±52.59 µmol/L vs. 277.94±74.33 µmol/L, P=0.025) and inversely correlated with the relapse rate (P=0.049). MS patients with lower serum UA levels tended to have higher white blood cell counts and myelin basic protein level. But there was no correlation between CSF protein levels (r=0.165, P=0.273), white blood cell counts (r=-0.051, P=0.732), IgG index (r=0. 045, P=0.802), or myelin basic protein level (r=-0.248, P=0.145) and the serum UA level, respectively.</p><p><b>CONCLUSION</b>In MS patients, UA levels might partly reflect the extent of disability and inflammation.</p>
Тема - темы
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Case-Control Studies , Disease Progression , Inflammation , Blood , Cerebrospinal Fluid , Inflammation Mediators , Cerebrospinal Fluid , Metabolism , Multiple Sclerosis , Blood , Cerebrospinal Fluid , Allergy and Immunology , Metabolism , Spine , Uric Acid , BloodРеферат
<p><b>BACKGROUND</b>Whether antibody to myelin oligodendrocyte glycoprotein (MOG) can be a diagnostic marker for multiple sclerosis (MS) is still controversial. Recent studies suggested that serum specific anti-MOG epitope antibody might be an MS specific marker. However, these studies did not include neuromyelitis optica (NMO) which might be proven to also have anti-MOG antibody. Hence, the present study was undertaken to investigate the clinical value of serum antibodies to 25 MOG epitopes in conventional MS (CMS) and NMO.</p><p><b>METHODS</b>Serum anti-MOG epitope IgG was detected in 61 CMS patients, 54 NMO patients, and 77 healthy controls, using enzyme-linked immunosorbent assay (ELISA).</p><p><b>RESULTS</b>Anti-MOG(27-38) IgG levels in both CMS and NMO patients were significantly higher than that in healthy controls (optical density (OD): 0.64 ± 0.38, 0.48 ± 0.23 vs. 0.19 ± 0.09; P = 0.000). CMS and NMO patients in relapse stage had significantly higher anti-MOG(27-38) IgG level than patients in remission stage (OD: 0.55 ± 0.14 vs. 0.24 ± 0.09, P = 0.027).</p><p><b>CONCLUSION</b>Although serum anti-MOG epitope IgG could not differentiate MS from NMO, it may be a useful marker for monitoring disease activity.</p>
Тема - темы
Adult , Female , Humans , Male , Middle Aged , Antibodies , Blood , Allergy and Immunology , Epitopes , Allergy and Immunology , Multiple Sclerosis , Blood , Allergy and Immunology , Myelin-Oligodendrocyte Glycoprotein , Allergy and Immunology , Neuromyelitis Optica , Blood , Allergy and ImmunologyРеферат
<p><b>OBJECTIVE</b>To investigate the characteristics of cerebral artery lesions in patients with limb-shaking transient ischemic attacks (LS-TIA) and its treatment.</p><p><b>METHODS</b>We retrospectively analyzed the clinical data of 20 patients with LS-TIA who received treatment in Peking Union Medical College Hospital from 2005 to 2008.</p><p><b>RESULTS</b>Critical stenosis or occlusion of contralateral arteries were found in the siphonic part of internal carotid artery (ICA) in 6 patients, terminal ICA or proximal middle cerebral artery (MCA) in 6 patients, and distal MCA in 1 patient. Seven patients had proximal ICA occlusion. The brain MRI showed typical watershed cerebral infarctions in 8 patients. EEG studies failed to show epileptiform activity associated with LS-TIA, but found focal frontotemporal lobe slow activity in 6 patients, which was consistent with hypoperfusion area in CT perfusion. Six patients received surgical revascularization and no one recurred.</p><p><b>CONCLUSION</b>Intracranial artery including the siphonic part of ICA, terminal ICA and proximal MCA stenosis is the main underlying cause of LS-TIA in Chinese, and surgical revascularization may be effective in abolishing the attacks.</p>