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Objective:To study peripheral nerve morphology in patients with transthyretin familial amyloid polyneuropathy (TTR-FAP) using high-frequency ultrasonography (HFUS), and to evaluate the value of HFUS in diagnosis of TTR-FAP.Methods:Thirty-eight patients with TTR-FAP diagnosed by gene detection and 23 normal controls from June 2015 to June 2021 in Peking University First Hospital were enrolled in this study. Consecutive ultrasonography scanning was performed in 6 pairs of nerves of bilateral limbs with 30 sites. The cross sectional area (CSA), CSA variability and inter-nerve CSA variability data of the two groups were retrospectively calculated and compared.Results:Compared with the normal controls, TTR-FAP patients showed larger CSA values at most nerve sites of both upper and lower limbs, and there were statistically significant differences at M1(median nerve) [8.55 (6.90, 9.40) mm 2vs 10.05 (9.10, 14.10) mm 2, Z=5.58, P<0.001], M3 (median nerve) [(6.98±1.66) mm 2vs (9.29±2.30) mm 2, t=6.28, P<0.001], M5 (median nerve) [(8.91±1.81) mm 2vs (14.33±4.20) mm 2, t=9.84, P<0.001], U5 (ulnar nerve) [(6.20±1.93) mm 2vs (9.34±2.85) mm 2, t=7.31, P<0.001], Sci1 (sciatic nerve) [(53.50±17.24) mm 2vs (79.74±20.75) mm 2, t=7.57, P<0.001], Sci2 (sciatic nerve) [(53.66±14.21) mm 2vs (73.98±19.21) mm 2, t=6.82, P<0.001] and Tib (tibial nerve) [(31.05±8.43) mm 2vs (46.29±13.14) mm 2, t=7.84, P<0.001] sites. There was no statistically significant difference in CSA at each site among the different subtypes and disease severity of TTR-FAP patients ( P>0.05). There was no statistically significant difference in CSA-variability of the median and ulnar nerves between the patients with TTR-FAP and the normal controls ( P>0.05). The side-to-side difference ratio of intra-nerve CSA variability of the ulnar nerve in the patients with TTR-FAP was smaller than that of the normal controls (1.15±0.10 vs 1.46±0.43, t=3.43, P=0.002), whereas no statistically significant difference of that in the median nerve was found between the two groups ( P>0.05). Conclusions:The most pronounced peripheral nerve thickening in the proximal limb segments with no signs of asymmetric distribution or lateralization is confirmed by HFUS in TTR-FAP patients and should be regarded as a marker of TTR-FAP. HFUS has clinical value in diagnosis of peripheral neuropathy in TTR-FAP patients.
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Objective To investigate the effect of cardiac function and renal function on early neurological function recovery of acute stroke patients.Methods Hospitalized acute stroke patients from January 2010 to May 2018 were recruited in the Department of Neurology,Peking University First Hospital,who were divided into two groups according to the modified Rankin Scale (mRS) score at discharge,good recovery (mRS score ≤2) and poor recovery (mRS score ＞2).The clinical characteristics,laboratory and echocardiography data were collected respectively.All cases were classified according to Trial of Org 10 172 in Acute Stroke Treatment (TOAST) criteria.Estimated glomerular filtration rate (eGFR) was calculated by chronic kidney disease epidemiology collaboration equation in renal function assessment,and cardiac function was calculated by left ventricular ejection fraction (LVFF) and E-wave/A-wave ratio (E/A).Multivariate Logistic regression analysis and stratified analysis were performed to explore the effects of cardiac and renal function and interactions on stroke patients.Results A total of 517 patients with acute stroke were enrolled in this study,23.4％ (121/517) of which presented with chronic kidney disease.Poor recovery was aggravated with the progression of eGFR stage in patients with acute stroke (x2=14.627,P=0.001).Compared with the good recovery group,LVEF and E/A were significantly lower in the poor recovery group,while left atrium diameter ((3.87±0.52) cm vs (3.77±0.49) cm,t=-2.139,P=0.033),interventricular septum thickness ((1.10±0.19) cm vs (1.04±0.16) cm,t=-4.056,P=0.000),left ventricular posterior wall thickness ((1.00±0.13) cm vs (0.98±0.13) cm,t=-2.190,P=0.029) and left ventricular mass index ((102.03±25.73) g/m2 vs (94.94±23.63) g/m2,t=-3.145,P=0.002) were significantly higher in the poor recovery group.Stratified analysis showed that the rate of poor recovery increased with the decrease of eGFR at different levels of LVEF and E/A.Compared with patients of normal renal function and mild impairment of renal function or with patients of high third tertile of LVEF,chronic kidney disease significantly increased the rate of poor recovery in patients with low third tertile of LVEF,while the rate of poor recovery was not significantly different in patients with chronic kidney disease among third tertile of E/A.The levels of eGFR and LVEF in different stroke subtypes were significantly different (F=7.433,P=0.000;F=2.617,P=0.034).The eGFR and LVEF levels of the cardioembolism (CE) group were the lowest compared with other subtypes of stroke.The eGFR levels in the CE group were significantly lower than that in other subtypes except the large artery atherosclerosis group,and the LVEF level was significantly lower in the CE group compared to the small artery occlusion group.Multivariate Logistic regression analysis revealed that eGFR (OR=0.866,95％CI 0.760-0.987,P=0.031),LVEF (OR=0.798,95％CI 0.688-0.925,P=0.003),E/A (OR=0.136,95％CI 0.034-0.548,P=0.005) and eGFR by LVEF (OR=1.002,95％CI 1.000-1.004,P=0.022) were significantly associated with early functional outcome in patients with acute stroke after adjusting for relevant clinical confounders (all P＜0.05).Conclusions Renal function,cardiac systolic and diastolic function were associated with the early functional recovery in patients with acute stroke.Moreover,cardiac systolic dysfunction and renal dysfunction interacted significantly with the early neurological function recovery in patients with acute stroke.
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Objective The aim of this study was to establish a reasonable protocol for a rat model of ferric chloride-induced carotid arterial thrombosis by ultrasonic continuous evaluation.Methods Twenty SD rats were randomly divided into four groups and then treated with 20％,30％,40％ or 50％ concentrations of FeCl3,respectively.The vascular conditions were evaluated by 14L ultrasonic probe,at 10 mins,15 mins,and 20 mins.We then selected the best group through the examination of the rate of spontaneous reperfusion of blood vessels and the rate of reperfusion after intravenous injection of urokinase.At the end of the experiment,vessels were fixed in 10％ formalin solution and stained with HE.Results After external application of FeCl3 on rat common carotid artery for 20 mins,the artery occlusion rate was 100％,20％,0％ and 0％ in animals receiving 50％,40％,30％ and 20％ FeCl3,respectively.After external application of FeCl3 on rat common carotid artery for 120 mins,the spontaneous revascularization rate was 0％ in 50％ concentration group whereas were 100％ in rest other groups (P＜ 0.001).In 50％ concentration group,the partial recanalization rate was 40％ after intravenous injection of urokinase.HE staining revealed that the thrombus was dense and the lumen was partially recanalized after the urokinase intervention in 50％ concentration group.Conclusion By use of uultrasonic continuous evaluation of ferric chloride-induced thrombosis of rat common carotid artery,we have demonstrated that external application of 50％ ferric chloride solution for 20 mins is effective for the formation of thrombus model,which may be suitable for the studv of thrombolysis.
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Objective To investigate the clinical features and TCD-detected microembolic signals in patients with hypercoagulability related multiple acute cerebral infarcts within non-single arterial territories, and to explore the possi?ble underlying mechanisms. Methods A retrospective review was conducted on all clinical, laboratory, radiological and TCD monitoring records from patients with hypercoagulability related multiple acute cerebral infarcts within non-single arterial territories, who admitted to the neurology department in our hospital. Results The data from twenty-two cases were finally included in this study. All patients presented with acute-onset localized neurological dysfunction, e.g. hemi?paresis, aphasia, hemiparesthesia, dysarthria, hemianopsia and cortical blindness. Their hypercoagulability related diseas?es included 10 cases of systemic malignancy, 5 moderate to severe hyperhomocystynemia (HCY>50μmol/L), 2 nephrot?ic syndrome, 2 antiphospholipid syndrome, 1 ulcerative colitis, 1 polycythemia vera,1 paroxysmal nocturnal hemoglobin?uria. In 18 cases, the hypercoagulability related diseases were diagnosed after their initial stroke onset. DWI showed mul?tiple disseminated acute cerebral infarcts in non-single arterial territories involving bilateral anterior or anterior plus pos?terior cerebral circulation simultaneously. Foci involved lobar cortex/subcortex of cerebral hemisphere in 22 cases, deep cerebral hemisphere in 12 cases, cerebellum foci in 10 cases,brainstem foci in 2 cases. TCD revealed microembolic sig? nals in ten of 22 patients monitored. Conclusions Patients with multiple acute cerebral infarcts involving non-single arte?rial territories, should be screened for hypercoagulability as in that hypercoagulability and microembolism might be in?volved in the etiology of cerebral infarction.
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Objective To characterize the clinical manifestations, laboratory findings of patients with occult sys?temic malignant neoplasms, whose initial manifestation presented as multiple acute cerebral infarcts including coagula?tion function,radiological imaging and microembolic signals (MES) detection by transcranial Doppler sonography (TCD) and to explore the possible underlying mechanisms. Methods All clinical records, laboratory hematological tests includ?ing hypercoagulable states measured by D-dimer levels, brain MRI including DWI, and TCD monitoring MES, the treat?ment and prognosis were retrospectively reviewed in 12 patients with multiple acute cerebral infarcts as the first manifes?tation of occult systemic malignancy. Results The clinical manifestations presented as localized neurological dysfunction, e.g. hemiparesis, aphasia, hemiparesthesia, dysarthria, vertigo and seizures, etc. DWI revealed multiple disseminated acute cerebral infarcts in multiple arterial territories such as the bilateral anterior or anterior plus posterior cerebral circu?lation in all patients. Eleven of 12 patients tested had elevated D-dimer. TCD detected MES in 5 of 7 patients. There were 12 patients diagnosed with occult systemic malignancy including 5 lung cancer, 3 pancreatic cancer, 1 gastric can?cer, 1 colon cancer, 1 endometrial adenocarcinoma and 1 metastatic poorly differentiated mucinous adenocarcinoma with unknown primary. Ten patients already had remote metastasis at diagnosis. The prognosis was usually poor and there were 7 cases with ischemic stroke recurrence, 4 cases with acute myocardial infarction, 3 cases died during hospitaliza?tion. Conclusions When patients present with multiple disseminated acute cerebral infarcts involving multiple arterial territories as initial manifestation, the underlying occult systemic malignancy should be considered. Hypercoagulopathy and MES might provide the clues to the diagnosis.
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<p><b>BACKGROUND</b>Endothelial dysfunction is not only an early stage of atherosclerosis, but also involved in the pathogenesis of cerebral small-vessel diseases. Patients with cerebral microbleeds (CMBs) may have arteriolosclerosis as well as systemic atherosclerosis. However, little is known about the associations among CMBs, atherosclerosis of cerebral large arteries, and endothelial function. Our study aimed to investigate the relationships among them.</p><p><b>METHODS</b>This was a cross-sectional study. Ninety patients hospitalized in Peking University First Hospital with acute ischemic stroke were enrolled consecutively between November 1, 2007 and January 31, 2008. All subjects underwent transcranial Doppler and carotid color duplex ultrasonography to record the intima-media thickness (IMT) of common carotid artery, carotid plaque, and cerebral artery stenosis. Brain magnetic resonance imaging (MRI) routine sequences and gradient recall-echo T2(*)-weighted imaging were performed to count CMBs with clinical data blindness. Endothelial function was evaluated using flow-mediated dilation (FMD) and nitroglycerin-mediated dilation (NMD) of the brachial artery. FMD and NMD were examined by an experienced vascular sonographer using a high-resolution ultrasound.</p><p><b>RESULTS</b>Thirty cases (33.3%) had CMBs with counts ranging from 1 to 30. Both FMD ((9.9 ± 4.8)% vs. (15. 2 ± 7.4)%, P = 0.001) and NMD ((13.7 ± 6.1)% vs. (19.0 ± 7.4)%, P = 0.001) were significantly decreased in CMB-positive patients than in CMB-negative patients. No significant relationships were demonstrated between CMBs and intracranial and/or extracranial artery stenosis. The frequencies of CMBs in patients with IMT≥1.0 mm, carotid plaque, and extracranial artery stenosis were 37.5%, 39.4%, and 47.6% respectively, with no significant difference, but much higher than in patients with IMT <1.0 mm (5%, P < 0.05). In Logistic regression analysis, impaired FMD (OR = 5.783, 95% CI 1.652-6.718, P = 0.007) and high pulse pressure (OR = 6.228, 95% CI 1.594-3.891, P = 0.009) were independently associated with the presence of CMBs, as well as previous ischemic stroke. In contrast, NMD was not correlated with CMBs.</p><p><b>CONCLUSIONS</b>CMBs may coexist with cerebral atherosclerosis in ischemic stroke. Endothelial dysfunction may play a role in the pathogenesis of CMBs, but may not simply reflect functional alterations of large arteries.</p>
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Aged , Female , Humans , Male , Middle Aged , Brain Ischemia , Diagnosis , Cerebral Hemorrhage , Diagnosis , Cerebral Small Vessel Diseases , Diagnosis , Cross-Sectional Studies , Magnetic Resonance ImagingABSTRACT
Objective To investigate the clinical and radiological features of the patients with cryptogenic stroke as the first manifestation of pulmonary arteriovenous malformation (PAVM),and to explore the mechanism and summarize the diagnostic strategy.Methods Transcmnial Doppler (TCD) bubble tests,non-contrast or contrast-enhanced transthoracic and transesophageal echocardiography,contrast-enhanced thoracic CT and pulmonary angiography were performed in 2 patients with cryptogenic stroke for the detection of right to left shunt (RLS) and the diagnosis of PAVM.Then interventional catheter embolization of PAVM and postoperative follow-up were arranged.Results TCD bubble tests in these 2 patients showed the following characteristics indicating RLS:spontaneous continuous shunt at rest,earlystage shunt appeared rapidly after injection,considerable large amount of shunt volume as a shower of microbubble,the extent of shunt volume not attenuated by Valsalva Maneuver (VM).One patient was diagnosed as PAVM,the other was PAVM plus patent foramen ovale (PFO).Both patients of PAVM were successfully treated with interventional catheter embolizations.Then TCD bubble test was repeated and showed significantly decreased amount of RLS.They remained asymptomatic during the 3-year and 1-year follow-up.Conclusions In patients with cryptogenic stroke due to suspected paradoxical embolism,TCD bubble test should be performed for screening RLS.Besides PFO,a traditional access to intracardiac shunt,PAVM should be considered as a new access to extracardiac shunt in paradoxical embolism.