1.Clinical and imaging characteristics of adrenomyeloneuropathy(report of one case)
Liang SONG ; Shengqi FU ; Baoyang SHI
Journal of Clinical Neurology 2025;38(5):327-331
Objective To analyze the clinical and imaging features of adrenomyeloneuropathy(AMN).Methods A retrospective analysis was conducted on the clinical data of one patient diagnosed with AMN through genetic testing.Results This patient was a young male with insidious onset,chronic course and progressive aggravation.The main clinical manifestations were speech,choking cough when drinking water,weakness of both lower limbs,unstable walking and urinary incontinence.Physical examination showed dysarthria,bilateral pyramidal tract,sphincter dysfunction,combined with systemic skin blackening,skin and mucosal pigmentation.MRI showed symmetrical abnormal high signal of bilateral corticospinals,significant atrophy of cerebellum,brainstem and cervical and thoracic spinal cord.Lumbar puncture CSF IL-6,IL-8 slightly high,serum myelin oligodendrocyte glycoprotein(MOG)antibody positive(1∶32).The localization diagnosis was adrenal cortex cerebellum bilateral corticospinal tracts peripheral nerve,and the qualitative diagnosis considered hereditary metabolic diseases,MOG antibody-related diseases could not be excluded.But the patient had a chronic course,atrophy of cerebellum,brainstem,cervical and thoracic spinal cord,and considered that the serum MOG antibody might be false positive,so the qualitative diagnosis hereditary metabolic diseases were more likely.Because of the limited technical conditions in our hospital,we could not detect plasma very long-chain fatty acids,while the completion of gene detection showed ABCD1 gene mutation:c.1628C>T(p.Pro543Leu)variant,so the diagnosis of AMN was clear.Conclusions AMN is an X-linked recessive genetic disorder.Its clinical manifestations primarily include impairment of neural conduction tracts(spastic paraplegia,sphincter dysfunction,and deep sensory disturbances),adrenal cortical insufficiency(skin and mucosal hyperpigmentation,sparse hair),and cerebral symptoms(dysgraphia,visual/hearing impairment,cognitive dysfunction,and personality changes).Imaging findings mainly show demyelination of the brain white matter in the bilateral corticospinal tract regions and degenerative spinal cord atrophy.Elevated levels of very long-chain fatty acids(VLCFAs)and mutations in the ABCD1 gene can confirm the diagnosis of AMN.
2.Clinical and imaging characteristics of adrenomyeloneuropathy(report of one case)
Liang SONG ; Shengqi FU ; Baoyang SHI
Journal of Clinical Neurology 2025;38(5):327-331
Objective To analyze the clinical and imaging features of adrenomyeloneuropathy(AMN).Methods A retrospective analysis was conducted on the clinical data of one patient diagnosed with AMN through genetic testing.Results This patient was a young male with insidious onset,chronic course and progressive aggravation.The main clinical manifestations were speech,choking cough when drinking water,weakness of both lower limbs,unstable walking and urinary incontinence.Physical examination showed dysarthria,bilateral pyramidal tract,sphincter dysfunction,combined with systemic skin blackening,skin and mucosal pigmentation.MRI showed symmetrical abnormal high signal of bilateral corticospinals,significant atrophy of cerebellum,brainstem and cervical and thoracic spinal cord.Lumbar puncture CSF IL-6,IL-8 slightly high,serum myelin oligodendrocyte glycoprotein(MOG)antibody positive(1∶32).The localization diagnosis was adrenal cortex cerebellum bilateral corticospinal tracts peripheral nerve,and the qualitative diagnosis considered hereditary metabolic diseases,MOG antibody-related diseases could not be excluded.But the patient had a chronic course,atrophy of cerebellum,brainstem,cervical and thoracic spinal cord,and considered that the serum MOG antibody might be false positive,so the qualitative diagnosis hereditary metabolic diseases were more likely.Because of the limited technical conditions in our hospital,we could not detect plasma very long-chain fatty acids,while the completion of gene detection showed ABCD1 gene mutation:c.1628C>T(p.Pro543Leu)variant,so the diagnosis of AMN was clear.Conclusions AMN is an X-linked recessive genetic disorder.Its clinical manifestations primarily include impairment of neural conduction tracts(spastic paraplegia,sphincter dysfunction,and deep sensory disturbances),adrenal cortical insufficiency(skin and mucosal hyperpigmentation,sparse hair),and cerebral symptoms(dysgraphia,visual/hearing impairment,cognitive dysfunction,and personality changes).Imaging findings mainly show demyelination of the brain white matter in the bilateral corticospinal tract regions and degenerative spinal cord atrophy.Elevated levels of very long-chain fatty acids(VLCFAs)and mutations in the ABCD1 gene can confirm the diagnosis of AMN.
3.Efficacy and safety of tirofiban in treatment of branch atheromatous disease in elderly patients
Shengqi FU ; Lili ZHU ; Shengjie HU ; Jin ZHANG ; Haoran LI ; Sisen ZHANG
Chinese Journal of Geriatric Heart Brain and Vessel Diseases 2025;27(5):616-620
Objective To investigate the efficacy and safety of tirofiban in the treatment of branch atheromatous disease(BAD)in elderly patients.Methods A retrospective analysis was conducted on 215 elderly BAD patients admitted to our department from June 2021 to June 2023.According to their treatment,they were divided into tirofiban group 1(55 cases)and control group 1(160 ca-ses).Using propensity score matching in a ratio of 1∶1 algorithm for adjustment,the differences in baseline features between the two groups were eliminated,and there were finally 53 cases in the tirofiban group 2 and 53 cases in the control group 2 after matching.The NIHSS scores before treatment and at 24 h and 7 d after treatment were collected.All of them were followed up for 90 d,and modified Rankin scale(mRS)was applied to evaluate the prognosis.Results The tirofiban group 1 had significantly lower NIHSS score at 24 h and 7 d after treatment and shorter length of hospital stay than the control group 1(P<0.05,P<0.01),so were in the tirofiban group 2 than the control group[3(1,4)vs 3(2,6),1(0,3)vs 1(1,4),8(6,10)d vs 9(8,11)d,P<0.05].The proportion of mRS score ≤1 was obviously larger in the tirofiban group 1 than the control group 1(P<0.01).The tirofiban group 2 obtained notably larger proportions of mRS score≤1 and≤2(71.7%vs 43.4%,P=0.003;79.2%vs 60.4%,P=0.034),and smaller proportion of mRS≥4(5.7%vs 20.8%,P=0.045)when compared with the control group 2.Logistic regression analysis indicated that in the patients without diabetes and those non-smoking,tirofiban was associated with increased good outcomes(OR=0.266,95%CI:0.090-0.788,P=0.017;OR=0.341,95%CI:0.107-0.931,P=0.046).Conclusion Tirofiban may effectively improve the clinical outcomes in the elderly BAD patients.But further randomized controlled trials are needed for verification.
4.Diagnostic value of arterial spin labeling perfusion weighted imaging in the Alzheimer's disease
Pinpin LI ; Wenhua GUAN ; Pei LI ; Yingying MIAO ; Xueqiang YUE ; Shengqi FU
Journal of Practical Radiology 2025;41(9):1441-1444,1476
Objective To investigate the application value of arterial spin labeling(ASL)perfusion weighted imaging in the early diagnosis of Alzheimer's disease(AD).Methods A total of 60 patients with different ages(control group)and 60 AD patients with different degrees(patient group)who underwent cerebral ASL perfusion weighted imaging examination were selected.At the work-station,the region of interest(ROI)of the hippocampus and limbic system brain structures were delineated and the cerebral blood flow(CBF)value of the ROI were measured.The CBF values of the brain structures of the control group at different ages and the corresponding ROI of the control group and the patient group were compared.Results There were statistically significant differences in the CBF values of the cingulate gyrus,parahippocampal gyrus,hippocampus,orbital part of frontal lobe,temporal pole of temporal lobe,insula lobe and amygdaloid body of the limbic system among the different ages in the control group(P<0.05),and the CBF value of brain structures gradually decreased with the increase of age.There were statistically significant differences in CBF values of ROI between the control group and mild,moderate,severe patient group(P<0.05),and the CBF value of brain structures gradually decreased with the severity of AD condition.Conclusion The CBF value of ASL perfusion weighted imaging can objectively reflect the blood flow changes and functional status of the hippocampus and limbic system brain structures in elderly people of different ages and AD patients of different degrees,and has important value in the early diagnosis of AD patients.
5.Correlation between serum cold-inducible RNA-binding protein, cystatin C and cognitive impairment in cerebral small vessel disease
Zhiming LI ; Lili ZHU ; Shengqi FU ; Baoyang SHI ; Jianhua ZHAO
Chinese Journal of Behavioral Medicine and Brain Science 2025;34(8):686-691
Objective:To explore the correlation between levels of serum cold-inducible RNA-binding protein(CIRBP), cystatin C(CysC) and cognitive impairment in patients with cerebral small vessel disease(CSVD), as well as the diagnostic value of CI in CSVD.Methods:A total of 90 CSVD patients admitted to the Neurology Department of Zhengzhou People's Hospital from January 2024 to December 2024 were consecutively selected. According to the mini-mental state examination(MMSE) and Montreal cognitive assessment(MoCA), they were divided into non cognitive impairment group(NCI group, n=47) and cognitive impairment group(CI group, n=43). The general clinical data of patients were collected.Fasting venous blood was collected in the morning on the second day of admission to measure serum CIRBP, CysC, homocysteine(Hcy) and high-sentivity C-reactive protein(hs-CRP) levels.A multiple-factor Logistic regression model was applied to identify independent risk factors for CI in CSVD patients, the predictive performance of the model was evaluated by receiver operating characteristic(ROC) curves, and the area under the curve(AUC) value was calculated to quantify the diagnostic accuracy of the model. Results:The levels of Hcy((13.01±4.22)μmol/L vs (11.44±3.00)μmol/L), hs-CRP((2.84±3.01)mmol/L vs (1.81±1.32)mmol/L), CIRBP((2 412.40±967.78)pg/mL vs (1 715.13±971.98)pg/mL), and CysC((1.93±1.08)mg/L vs (1.24±0.87)mg/L) in the CI group were significantly higher than those in the NCI group(all P<0.05). Multiple Logistic regression analysis showed that both CIRBP( OR=1.001, 95% CI=>1.000-1.002, P=0.011) and CysC( OR=1.833, 95% CI=1.056-3.181, P=0.031) were independently influencing factors of the occurrence of CI in CSVD patients(all P<0.05). ROC curve analysis showed that the optimal cutoff values for serum CIRBP and CysC levels to evaluate CI were 1 875.50 pg/mL and 1.42 mg/L, respectively. The AUC (95% CI) were 0.805(95% CI=0.713-0.897, P<0.001) and 0.716(95% CI=0.607-0.825, P<0.001), respectively.The AUC(95% CI) of combined detection of CIRBP+ CysC was 0.820(95% CI=0.733-0.907, P<0.001), with specificity and sensitivity of 89.4% and 67.4%. Conclusion:The serum CIRBP and CysC levels can serve as independent predictors of CI in CSVD patients. Combined testing can improve the accuracy of patient condition assessment and may assist in the diagnosis and prediction of cognitive impairment in CSVD.
6.Correlation between serum cold-inducible RNA-binding protein, cystatin C and cognitive impairment in cerebral small vessel disease
Zhiming LI ; Lili ZHU ; Shengqi FU ; Baoyang SHI ; Jianhua ZHAO
Chinese Journal of Behavioral Medicine and Brain Science 2025;34(8):686-691
Objective:To explore the correlation between levels of serum cold-inducible RNA-binding protein(CIRBP), cystatin C(CysC) and cognitive impairment in patients with cerebral small vessel disease(CSVD), as well as the diagnostic value of CI in CSVD.Methods:A total of 90 CSVD patients admitted to the Neurology Department of Zhengzhou People's Hospital from January 2024 to December 2024 were consecutively selected. According to the mini-mental state examination(MMSE) and Montreal cognitive assessment(MoCA), they were divided into non cognitive impairment group(NCI group, n=47) and cognitive impairment group(CI group, n=43). The general clinical data of patients were collected.Fasting venous blood was collected in the morning on the second day of admission to measure serum CIRBP, CysC, homocysteine(Hcy) and high-sentivity C-reactive protein(hs-CRP) levels.A multiple-factor Logistic regression model was applied to identify independent risk factors for CI in CSVD patients, the predictive performance of the model was evaluated by receiver operating characteristic(ROC) curves, and the area under the curve(AUC) value was calculated to quantify the diagnostic accuracy of the model. Results:The levels of Hcy((13.01±4.22)μmol/L vs (11.44±3.00)μmol/L), hs-CRP((2.84±3.01)mmol/L vs (1.81±1.32)mmol/L), CIRBP((2 412.40±967.78)pg/mL vs (1 715.13±971.98)pg/mL), and CysC((1.93±1.08)mg/L vs (1.24±0.87)mg/L) in the CI group were significantly higher than those in the NCI group(all P<0.05). Multiple Logistic regression analysis showed that both CIRBP( OR=1.001, 95% CI=>1.000-1.002, P=0.011) and CysC( OR=1.833, 95% CI=1.056-3.181, P=0.031) were independently influencing factors of the occurrence of CI in CSVD patients(all P<0.05). ROC curve analysis showed that the optimal cutoff values for serum CIRBP and CysC levels to evaluate CI were 1 875.50 pg/mL and 1.42 mg/L, respectively. The AUC (95% CI) were 0.805(95% CI=0.713-0.897, P<0.001) and 0.716(95% CI=0.607-0.825, P<0.001), respectively.The AUC(95% CI) of combined detection of CIRBP+ CysC was 0.820(95% CI=0.733-0.907, P<0.001), with specificity and sensitivity of 89.4% and 67.4%. Conclusion:The serum CIRBP and CysC levels can serve as independent predictors of CI in CSVD patients. Combined testing can improve the accuracy of patient condition assessment and may assist in the diagnosis and prediction of cognitive impairment in CSVD.
7.Efficacy and safety of intravenous thrombolysis with tenecteplase in treatment of capsular warning syndrome
Tengfei ZHANG ; Shengjie HU ; Shengqi FU ; Ying ZHANG ; Lili ZHU ; Liang SONG ; Sisen ZHANG
Chinese Journal of Geriatric Heart Brain and Vessel Diseases 2025;27(8):1056-1060
Objective To investigate the clinical efficacy and safety of intravenous thrombolysis with tenecteplase in the treatment of CWS.Methods A prospective study was conducted on 136 CWS patients consecutively admitted in Department of Neurology of Zhengzhou People's Hospital from March 2019 to March 2024.They were randomly divided into a tenecteplase group(67 cases)and a control group(69 cases).NIHSS was used to evaluate the recovery of neurological function after treatment.mRS was employed to assess long-term prognosis.Results Significantly larger proportion of white matter lesions and higher baseline SBP level were observed in the tenecteplase group than the control group(P<0.05).The tenecteplase group obtained obviously greater ratio of overall recovery than the control group,with notably lower NIHSS score,incidence of new CWS attacks and proportion of new acute cerebral infarction at 24 and 72 h and 7 d after treat-ment(P<0.05,P<0.01).Moreover,the proportion of mRS score of 0-2 was notably greater,while that of the score of 3-6 was lower in the tenecteplase group than the Control group(P<0.05).Intravenous thrombolysis with tenecteplase was an influencing factors for 90-day mRS score of 0-2 and of 3-6 in the CWS patients(OR=0.264,95%CI:0.089-0.813;OR=4.144,95%CI:1.184-14.506,P<0.05).Conclusion Intravenous thrombolysis with tenecteplase for CWS significantly improves the proportion of patients with good prognosis.
8.Correlation of CT perfusion imaging parameters combined with serum biomarkers and prognosis in patients with BAD
Shengjie HU ; Shengqi FU ; Haoran LI ; Lili ZHU ; Meng YU ; Haiqiang QIN
Chinese Journal of Geriatric Heart Brain and Vessel Diseases 2025;27(3):317-321
Objective To investigate the correlation of imaging parameters of CT perfusion scan-ning combined with serum suPAR and Mac-2BP with END and poor outcomes in patients with BAD.Methods A total of 176 BAD patients admitted to Department of Neurology of Zhengzhou People's Hospital from June 2019 to March 2024 were prospectively enrolled,and according to whether END occurred within 72 h after onset,they were divided into END group(42 cases)and non-END group(134 cases).Based on their outcomes at 90 d of follow-up,they were also assigned into good outcome group(129 cases)and poor outcome group(47 cases).The serum suPAR and Mac-2BP levels were compared between the END and non-END groups,as well as the good and poor outcome groups.ROC curves were plotted to analyze the accuracies of serum suPAR and Mac-2BP in predicting END and poor outcomes.Results The END group had significantly higher suPAR and Mac-2BP levels,longer MTT and TTP,and larger proportions of DWMH,basal gan-glia EPVS and cerebral perfusion impairment,but lower CBV and CBF when compared with the non-END group(P<0.05,P<0.01).Obviously higher suPAR and Mac-2BP levels,NIHSS score at END,longer MTT and TTP,and larger proportions of EDN,basal ganglia EPVS and cerebral perfusion impairment,but lower CBV and CBF were observed in the poor outcome group than the good outcome group(P<0.05,P<0.01).suPAR and Mac-2BP levels,cerebral perfusion impair-ment and DWMH were independent risk factors for END(P<0.05,P<0.01).suPAR,Mac-2BP,NIHSS score at END,END,cerebral perfusion impairment,and basal ganglia EPVS were inde-pendent risk factors for poor outcomes(P<0.05,P<0.01).The AUC value of combined suPAR and Mac-2BP in predicting END was 0.8844(95%CI:0.822-0.947),and the value in predicting poor outcomes was 0.8742(95%CI:0.810-0.938)in BAD patients.Conclusion Cerebral perfu-sion impairment,suPAR and Mac-2BP are independent risk factors for END and poor outcome at 90 d in BAD patients.Combined detection can is helpful in evaluating the patient's condition and predicting the prognosis.
9.Bleeding risk and efficacy of rivaroxaban anticoagulation therapy in atrial fibrillation patients within 48 h after stroke onset
Tengfei ZHANG ; Lili ZHU ; Shengqi FU ; Shengjie HU ; Liang SONG ; Sisen ZHANG
Chinese Journal of Geriatric Heart Brain and Vessel Diseases 2025;27(1):22-26
Objective To investigate the effects of different timing of initiation of rivaroxaban anti-coagulation therapy on the efficacy and bleeding risk of atrial fibrillation patients after stroke.Methods A total of 336 patients with atrial fibrillation and stroke admitted in our hospital be-tween January 2021 and December 2023 were consecutively enrolled,and randomly divided into an experimental group(165 cases)and a control group(171 cases).The experimental group received rivaroxaban treatment within 48 h of symptom onset,whereas the control group initiated oral ri-varoxaban treatment on the 3rd,6th,and 12th day post-stroke onset,respectively,depending on stroke severity(mild,moderate,and severe).Their baseline clinical data were collected,and all of them were followed up till 90 d after stroke.The incidences of recurrent ischemic stroke,symp-tomatic intracranial hemorrhage and extracranial hemorrhage,mortality,and proportion of pa-tients with mRS score≤ 2 and distribution of the score were observed and analyzed in the two groups.Results During a 90-day follow-up period,the patients with moderate stroke from the ex-perimental group of patients exhibited a significantly lower rate of recurrent ischemic stroke than those in the control group(5.4%vs 15.9%,P=0.037).However,for patients with mild and se-vere stroke,no obvious difference was observed in the primary endpoint of recurrent ischemic stroke between the experimental and control groups(3.0%vs 3.2%,12.5%vs 14.8%,P>0.05).Though no statistical differences were observed,lower rates of symptomatic intracranial hemor-rhage(1.5%vs 3.2%,5.4%vs 9.8%),reduced incidence of extracranial hemorrhage(9.0%vs 14.5%,12.2%vs 15.9%),and lower mRS score[1(0,2)vs 1(1,2),3(1,4)vs 3(2,4)]were seen in the patients with mild and moderate stroke from the experimental group when compared with the control group(P>0.05).Similarly,there were no statistically differences for the severe stroke patients between the experimental and control groups(P>0.05)in the incidence of extracranial hemorrhage(20.8%vs 22.2%),rate of symptomatic intracranial hemorrhage(20.8%vs 7.4%),mortality(8.3%vs 3.7%),and mRS score[4(3,4)vs 3(3,4)].Two patients from the experimen-tal group died,with one case due to secondary pulmonary infection and the other due to brainstem hemorrhage.In the control group,only one death occurred due to brainstem hemorrhage.Conclu-sion For atrial fibrillation patients,anticoagulation with rivaroxaban within 48 h after stroke has no significant increase in the risk of bleeding,reduces the proportion of recurrent ischemic stroke in patients with moderate stroke,and may improve the prognosis of patients.
10.Efficacy and safety of intravenous thrombolysis with tenecteplase in treatment of capsular warning syndrome
Tengfei ZHANG ; Shengjie HU ; Shengqi FU ; Ying ZHANG ; Lili ZHU ; Liang SONG ; Sisen ZHANG
Chinese Journal of Geriatric Heart Brain and Vessel Diseases 2025;27(8):1056-1060
Objective To investigate the clinical efficacy and safety of intravenous thrombolysis with tenecteplase in the treatment of CWS.Methods A prospective study was conducted on 136 CWS patients consecutively admitted in Department of Neurology of Zhengzhou People's Hospital from March 2019 to March 2024.They were randomly divided into a tenecteplase group(67 cases)and a control group(69 cases).NIHSS was used to evaluate the recovery of neurological function after treatment.mRS was employed to assess long-term prognosis.Results Significantly larger proportion of white matter lesions and higher baseline SBP level were observed in the tenecteplase group than the control group(P<0.05).The tenecteplase group obtained obviously greater ratio of overall recovery than the control group,with notably lower NIHSS score,incidence of new CWS attacks and proportion of new acute cerebral infarction at 24 and 72 h and 7 d after treat-ment(P<0.05,P<0.01).Moreover,the proportion of mRS score of 0-2 was notably greater,while that of the score of 3-6 was lower in the tenecteplase group than the Control group(P<0.05).Intravenous thrombolysis with tenecteplase was an influencing factors for 90-day mRS score of 0-2 and of 3-6 in the CWS patients(OR=0.264,95%CI:0.089-0.813;OR=4.144,95%CI:1.184-14.506,P<0.05).Conclusion Intravenous thrombolysis with tenecteplase for CWS significantly improves the proportion of patients with good prognosis.

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