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 alphabe...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.展开更多
Background:In the clinic,practitioners encounter many patients with an abnormal pattern of dense punctate magnetic resonance imaging(MRI)signal in the basal ganglia,a phenomenon known as"cheese sign".This si...Background:In the clinic,practitioners encounter many patients with an abnormal pattern of dense punctate magnetic resonance imaging(MRI)signal in the basal ganglia,a phenomenon known as"cheese sign".This sign is reported as common in cerebrovascular diseases,dementia,and old age.Recently,cheese sign has been speculated to consist of dense perivascular space(PVS).This study aimed to assess the lesion types of cheese sign and analyze the correlation between this sign and vascular disease risk factors.Methods:A total of 812 patients from Peking Union Medical College Hospital(PUMCH)dementia cohort were enrolled.We analyzed the relationship between cheese sign and vascular risk.For assessing cheese sign and defining its degree,the abnormal punctate signals were classified into basal ganglia hyperintensity(BGH),PVS,lacunae/infarctions and microbleeds,and counted separately.Each type of lesion was rated on a four-level scale,and then the sum was calculated;this total was defined as the cheese sign score.Fazekas and Age-Related White Matter Changes(ARWMC)scores were used to evaluate the paraventricular,deep,and subcortical gray/white matter hyperintensities.Results:A total of 118 patients(14.5%)in this dementia cohort were found to have cheese sign.Age(odds ratio[OR]:1.090,95%confidence interval[CI]:1.064-1.120,P<0.001),hypertension(OR:1.828,95%CI:1.123-2.983,P=0.014),and stroke(OR:1.901,95%CI:1.092-3.259,P=0.025)were risk factors for cheese sign.There was no significant relationship between diabetes,hyperlipidemia,and cheese sign.The main components of cheese sign were BGH,PVS,and lacunae/infarction.The proportion of PVS increased with cheese sign severity.Conclusions:The risk factors for cheese sign were hypertension,age,and stroke.Cheese sign consists of BGH,PVS,and lacunae/infarction.展开更多
To the Editor:Idiopathic normal pressure hydrocephalus(iNPH)patients present disturbances in gait,cognition,and/or control of urination with neuroimaging characterized by enlargement of the cerebral ventricles.Typical...To the Editor:Idiopathic normal pressure hydrocephalus(iNPH)patients present disturbances in gait,cognition,and/or control of urination with neuroimaging characterized by enlargement of the cerebral ventricles.Typically,gait disturbance in iNPH patients is the initial and most prominent symptom,but motor impairments can also extend to upper extremity.The cerebrospinaluid(CSF)tap test(TT),in which the symptoms of iNPH patients are assessed before and after drainage of 30-50mL CSF by lumbar puncture,is a commonly used auxiliary test for predicting shunt responsiveness.Standard methods for determining the CSF TT response are based on the clinical impression of changes in gait after lumber puncture.iNPH patients who are unable to ambulate(e.g.,the patient is wheelchair bound)may not be able to comply with the gait evaluation but still benet from shunt placement.The grooved pegboard test(GPT)and symbol-digit modalities test(SDMT)are psychometric measures of upper extremity motor and psychomotor speed.Herein,we sought to identify whether these tests could be used to objectively assess responsiveness to the CSF TT.展开更多
基金supported by grants from CAMS Innovation Fund for Medical Sciences(No.2016-I2M-1-004)National Natural Science Foundation of China(No.81550021)+1 种基金13th Five-year National Key Research and Development Program of China(No.2016YFC1306300)the strategic priority research program(pilot study)“Biological basis of aging and therapeutic strategies”of the Chinese Academy of Sciences(XDPB10)。
文摘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.
基金National Key Research and Development Program of China(Nos.2020YFA0804500 and 2020YFA0804501)CAMS Innovation Fund for Medical Sciences(CIFMS)(Nos.2021-I2M-1-020 and 2020-I2M-C&T-B-010)+1 种基金National Natural Science Foundation of China(Nos.81550021 and 30470618)Science Innovation 2030-Brain Science and Brain-Inspired Intelligence Technology Major Project(No.2021ZD0201106)
文摘Background:In the clinic,practitioners encounter many patients with an abnormal pattern of dense punctate magnetic resonance imaging(MRI)signal in the basal ganglia,a phenomenon known as"cheese sign".This sign is reported as common in cerebrovascular diseases,dementia,and old age.Recently,cheese sign has been speculated to consist of dense perivascular space(PVS).This study aimed to assess the lesion types of cheese sign and analyze the correlation between this sign and vascular disease risk factors.Methods:A total of 812 patients from Peking Union Medical College Hospital(PUMCH)dementia cohort were enrolled.We analyzed the relationship between cheese sign and vascular risk.For assessing cheese sign and defining its degree,the abnormal punctate signals were classified into basal ganglia hyperintensity(BGH),PVS,lacunae/infarctions and microbleeds,and counted separately.Each type of lesion was rated on a four-level scale,and then the sum was calculated;this total was defined as the cheese sign score.Fazekas and Age-Related White Matter Changes(ARWMC)scores were used to evaluate the paraventricular,deep,and subcortical gray/white matter hyperintensities.Results:A total of 118 patients(14.5%)in this dementia cohort were found to have cheese sign.Age(odds ratio[OR]:1.090,95%confidence interval[CI]:1.064-1.120,P<0.001),hypertension(OR:1.828,95%CI:1.123-2.983,P=0.014),and stroke(OR:1.901,95%CI:1.092-3.259,P=0.025)were risk factors for cheese sign.There was no significant relationship between diabetes,hyperlipidemia,and cheese sign.The main components of cheese sign were BGH,PVS,and lacunae/infarction.The proportion of PVS increased with cheese sign severity.Conclusions:The risk factors for cheese sign were hypertension,age,and stroke.Cheese sign consists of BGH,PVS,and lacunae/infarction.
基金National Key Research and Development Program of China(No.2020YFA0804500)CAMS Innovation fund for Medical Sciences(No.2016-12 M-1-004)National Natural Science Foundation of China(No.81550021)
文摘To the Editor:Idiopathic normal pressure hydrocephalus(iNPH)patients present disturbances in gait,cognition,and/or control of urination with neuroimaging characterized by enlargement of the cerebral ventricles.Typically,gait disturbance in iNPH patients is the initial and most prominent symptom,but motor impairments can also extend to upper extremity.The cerebrospinaluid(CSF)tap test(TT),in which the symptoms of iNPH patients are assessed before and after drainage of 30-50mL CSF by lumbar puncture,is a commonly used auxiliary test for predicting shunt responsiveness.Standard methods for determining the CSF TT response are based on the clinical impression of changes in gait after lumber puncture.iNPH patients who are unable to ambulate(e.g.,the patient is wheelchair bound)may not be able to comply with the gait evaluation but still benet from shunt placement.The grooved pegboard test(GPT)and symbol-digit modalities test(SDMT)are psychometric measures of upper extremity motor and psychomotor speed.Herein,we sought to identify whether these tests could be used to objectively assess responsiveness to the CSF TT.
