This case report investigates the manifestation of cerebral amyloid angiopathy (CAA) through recurrent Transient Ischemic Attacks (TIAs) in an 82-year-old patient. Despite initial diagnostic complexities, cerebral ang...This case report investigates the manifestation of cerebral amyloid angiopathy (CAA) through recurrent Transient Ischemic Attacks (TIAs) in an 82-year-old patient. Despite initial diagnostic complexities, cerebral angiography-MRI revealed features indicative of CAA. Symptomatic treatment resulted in improvement, but the patient later developed a fatal hematoma. The discussion navigates the intricate therapeutic landscape of repetitive TIAs in the elderly with cardiovascular risk factors, emphasizing the pivotal role of cerebral MRI and meticulous bleeding risk management. The conclusion stresses the importance of incorporating SWI sequences, specifically when suspecting a cardioembolic TIA, as a diagnostic measure to explore and exclude CAA in the differential diagnosis. This case report provides valuable insights into these challenges, highlighting the need to consider CAA in relevant cases.展开更多
AIM:To report on the clinical features of patients with retinal amyloid angiopathy(RAA)who were identified to be caused by the transthyretin(TTR)Gly83Arg variant.METHODS:Case series of five patients diagnosed with RAA...AIM:To report on the clinical features of patients with retinal amyloid angiopathy(RAA)who were identified to be caused by the transthyretin(TTR)Gly83Arg variant.METHODS:Case series of five patients diagnosed with RAA was collected at Affiliated Hospital of Zunyi Medical University from January 2010 to December 2021.The clinical features,therapeutic strategies,and prognoses of all patients were reviewed.RESULTS:Five patients with a mean age of 52.00±7.23y were diagnosed as RAA.These patients were previously diagnosed with hereditary transthyretin amyloidosis caused by the TTR Gly83Arg variant.Vitreous opacity was found in all 10 eyes,and 7 eyes developed RAA 2 to 20y after the onset of hereditary transthyretin amyloidosis.The clinical manifestations were recurrent vitreous hemorrhage in 2 eyes(29%),neovascular glaucoma in 2 eyes(29%),and iris neovascularization in 1 eye(14%).Microangioma lesions were found in all affected eyes that underwent fundus fluorescein angiography(FFA)in this group of cases,and the incidence of the retinal non-perfusion area was 67%.Although no cases of retinal neovascularization were found,the prognosis of visual acuity was not ideal.CONCLUSION:This is the first report of RAA in patients with the TTR Gly83Arg variant.Complications such as RAA and glaucoma will seriously affect the visual prognosis of patients.Thereafter,regular ophthalmic follow-up of patients with hereditary transthyretin amyloidosis is essential.And FFA after vitrectomy is very important,which can help ophthalmologists detect RAA earlier and treat it in time.展开更多
BACKGROUND Cerebral proliferative angiopathy(CPA)is a rare subtype of arteriovenous malformation.It is extremely rare in pediatric patients and has serious implications for developing children.However,reports of these...BACKGROUND Cerebral proliferative angiopathy(CPA)is a rare subtype of arteriovenous malformation.It is extremely rare in pediatric patients and has serious implications for developing children.However,reports of these disorders worldwide are limited,and no uniform reference for diagnosis and treatment options exists.We report the case of a 6-year-old with CPA having predominantly neurological dysfunction and review the literature on pediatric CPA.CASE SUMMARY We report the case of a pediatric patient with CPA analyzed using digital subtraction angiography(DSA)who presented initially with a neurological disorder as the main manifestation.This case is the basis for further discussion of the clinical presentation,pathogenesis,diagnosis,and treatment of CPA in children.After the cerebral DSA,the patient was treated conservatively with sedation,fluid replacement,and blood anticoagulation.She could not cooperate with the followup magnetic resonance imaging examination because of her young age,and her family declined further treatment because of the surgery’s high risk.She was followed up for 3 months;her symptoms did not worsen.CONCLUSION This report of rare pediatric CPA can inform and advance clinical research on congenital cerebrovascular diseases.展开更多
Objective To study the changes of biomarkers in cerebrospinal fluid(CSF) in cerebral amyloid angiopathy(CAA) dementia and Alzheimer's disease.Methods Levels of amyloid protein β(Aβ42,Aβ40) and phosphorylated Ta...Objective To study the changes of biomarkers in cerebrospinal fluid(CSF) in cerebral amyloid angiopathy(CAA) dementia and Alzheimer's disease.Methods Levels of amyloid protein β(Aβ42,Aβ40) and phosphorylated Tau-protein(P-tau) in CSF and ratio of Aβ42/Aβ40 were tested in 5 cases with CAA dementia and 20 cases with Alzheimer's disease collected at Peking Union Medical College Hospital from December 2001 to March 2011.Results The levels of Aβ42,Aβ40,and P-tau in CSF and ratio of Aβ42/Aβ40 were(660.4±265.2) ng/L,(7111.0±1033.4) ng/L,(71.8±51.5) ng/L,and 0.077±0.033,respectively in CAA dementia and(663.6±365.6) ng/L,(5115.0±2931.1) ng/L,(47.7±38.8) ng/L,and 0.192±0.140,respectively in Alzheimer's disease patients.There were no statistically significant differences between CAA dementia and Alzheimer's disease in terms of these CSF biomarkers(all P>0.05).Conclusion Measurements of CSF biomarkers may not be helpful in differential diagnosis of CAA and Alzheimer's disease.展开更多
Purpose: We propose an analysis of dyscirculatory angiopathy of Alzheimer’s type (DAAT) endovascular treatment method based on transcatheter revascularization and recovery of collateral and microvascular bed of the b...Purpose: We propose an analysis of dyscirculatory angiopathy of Alzheimer’s type (DAAT) endovascular treatment method based on transcatheter revascularization and recovery of collateral and microvascular bed of the brain by means of low-energy transluminal laser irradiation as well as its comparison with traditional Alzheimer’s disease (AD) treatment methods. Methods: The research involved 81 patients aged 34 - 79 (average age 67). 46 (46.8%) patients were treated using endovascular method—Test Group. 35 (43.2%) patients were given conventional treatment—Control Group. Patients were subdivided: Group (CDR-0): 9 (11.1%), pre-clinical stage or increased AD risk;Group (CDR-1): 24 (29.6%), mild dementia and cognitive impairment;Group (CDR-2): 31 (38.3%), moderate dementia and persistent cognitive impairment;Group (CDR-3): 17 (21.0%), severe dementia and cognitive impairment. Research plan included CT or MRI with subsequent temporal lobes volume calculation, brain scintigraphy (SG), rheoencephalography (REG), and cerebral MUGA. There were indications and contraindications for treatment in Test Group. In Group CDR-0, endovascular intervention was prophylactic, against the background of increasing memory impairment;in Groups CDR-1, CDR-2, CDR-3, it was conducted in 1 to 12 years period from AD symptoms appear-ance. Conservative treatment with Memantin and Rivastigmine was carried out in Control Group. Results: In Test Group, positive outcome accompanied by prolonged dementia decline, cognitive impairment decrease, and patients’ transition to CDR group of an earlier stage, was obtained in all cases. In Control Group, patients’ temporary stabilization in their own CDR group was achieved. Conclusions: Endovascular treatment of patients with AD different stages can not only reduce DAAT phenomena but can also cause AD regression possibly accompanied by regenerative processes in the cerebral tissue. Conservative treatment only allows stabilizing the patient’s condition for a while.展开更多
BACKGROUND Cerebral proliferative angiopathy(CPA)is a rare vascular disease characterized by the presence of diffuse vascular proliferation,progressive vascular hyperflow and vasodilation of multiple vessels in the no...BACKGROUND Cerebral proliferative angiopathy(CPA)is a rare vascular disease characterized by the presence of diffuse vascular proliferation,progressive vascular hyperflow and vasodilation of multiple vessels in the normal brain parenchyma.Unlike cerebral arteriovenous malformations,CPA has a mixed appearance between that of lesions with cell proliferation and endothelial proliferation.To date,the pathogenesis of CPA is unclear,in which changes induced by cortical ischemia in the elastic layer of the blood supply artery and smooth muscle cells may be involved.CASE SUMMARY In this article,we retrospectively analyzed a case of hemorrhagic transformation of ischemic CPA diagnosed by digital subtraction angiography and reviewed the related literature for further exploration of its pathogenesis,diagnosis and treatment.CONCLUSION The information in the present case report may facilitate further clinical research on this cerebrovascular disease.展开更多
Purpose: We assess the significance of dyscirculatory angiopathy of Alzheimer’s type (DAAT) in identify- ing the predisposition to the development and diagnosis of Alzheimer’s disease (AD) different stages. Meth- od...Purpose: We assess the significance of dyscirculatory angiopathy of Alzheimer’s type (DAAT) in identify- ing the predisposition to the development and diagnosis of Alzheimer’s disease (AD) different stages. Meth- ods: 108 patients took part in the research:1) 49 aged 34-79 suffering from AD or running an increased risk of its development (those not diagnosed with AD but having growing memory disorders without any mani- festations of dementia or specific cognitive impairments, and having 2 or more immediate relatives with AD) - Test Group;2) 59 aged 28-78 suffering from different types of brain lesions accompanied by dementia but not suffering from AD or corresponding to their age norm - Control Group. All the patients underwent MRI, CT with subsequent calculation of the temporal lobes atrophy degree, brain scintigraphy (SG), rheoencepha- lography (REG), and MUGA. Results: Characteristic features of patients with an increased risk of AD as well as at its various stages are: 1) Temporal lobes and hippocampus atrophy ranging from 4% among those with an increased risk of AD to 62% among those at its advanced stages;2) DAAT manifestations: reduction of the capillary bed in the temporal and frontoparietal regions with the development of multiple arterioven- ous shunts of the same localization and correspondent early venous discharge accompanied by venous stasis on the border of the frontal and parietal region;3) DAAT phenomena equally develop both among those with an increased risk of developing AD and those at various AD stages. Similar changes are not observed among Control Group patients with other brain lesions, regardless of the severity of dementia, as well as among practically healthy people of the corresponding age group. Conclusion: Timely identification of the above- mentioned changes can reveal a predisposition to AD development long before its initial manifestations, and it allows differentiating AD from other diseases attended by dementia. In both cases, timely diagnosis allows beginning timely treatment and thus achieving more stable results.展开更多
Objective: To evaluate the incidence of cervical angiopathy caused by radiation therapy for head and neck cancer. Methods: Segments of 57 cervical arteries were obtained during surgery for head and neck malignant tumo...Objective: To evaluate the incidence of cervical angiopathy caused by radiation therapy for head and neck cancer. Methods: Segments of 57 cervical arteries were obtained during surgery for head and neck malignant tumors and divided into two groups (irradiated group and non-irradiated group) based on the treatment prior to vascular resection. In order to evaluate vascular injury after radiation therapy, we examined the degree of medial atrophy, medial fibrosis, smooth muscle cell (SMC) differentiation in the media and intima, intimal hyperplasia and endothelial cell (EC) injury. Sections of arterial segments were stained with hematoxylin-eosin, Elastica van Gieson and Masson’s trichrome, and immunohistochemistry for α-smooth muscle actin (α-SMA), smoothelin, S100A4 and CD31 in the resected vessels was conducted. Results: The median interval between the completion of radiation therapy and vascular resection was nine months. No significant differences were observed between the two groups in terms of medial atrophy, medial fibrosis and intimal hyperplasia. The ratio of the smoothelin-positive area per α-SMA-positive area in the media and the S100A4-positive proportion in the intima, indicating the degree of differentiation of the medial SMC and dedifferentiation of the intimal SMC, respectively, showed no significant differences, despite the tendency toward a lower smoothelin-positive area per α-SMA-positive area in the media of the irradiated arteries. The EC coverage revealed on CD31 immunohistochemistry was significantly decreased, with mural thrombus adhesion, in the irradiated group. Conclusions: The ECs of small arteries are damaged by irradiation. Although we did not confirm the statistical significance of medial SMC dedifferentiation, a decreased expression of smoothelin tended to be observed in the media of the irradiated arteries. Our findings provide histopathological evidence of irradiation angiopathy in head and neck cancer and may help to improve the surgical safety of microvascular anastomosis and determine the treatment strategy for head and neck tumors.展开更多
BACKGROUND Diagnosis of a dementia subtype can be complex and often requires comprehensive cognitive assessment and dedicated neuroimaging.Clinicians are prone to cognitive biases when reviewing such images.We present...BACKGROUND Diagnosis of a dementia subtype can be complex and often requires comprehensive cognitive assessment and dedicated neuroimaging.Clinicians are prone to cognitive biases when reviewing such images.We present a case of cognitive impairment and demonstrate that initial imaging may have resulted in misleading the diagnosis due to such cognitive biases.CASE SUMMARY A 76-year-old man with no cognitive impairment presented with acute onset word finding difficulty with unremarkable blood tests and neurological examination.Magnetic resonance imaging(MRI)demonstrated multiple foci of periventricular and subcortical microhaemorrhage,consistent with cerebral amyloid angiopathy(CAA).Cognitive assessment of this patient demonstrated marked impairment mainly in verbal fluency and memory.However,processing speed and executive function are most affected in CAA,whereas episodic memory is relatively preserved,unlike in other causes of cognitive impairment,such as Alzheimer’s dementia(AD).This raised the question of an underlying diagnosis of dementia.Repeat MRI with dedicated coronal views demonstrated mesial temporal lobe atrophy which is consistent with AD.CONCLUSION MRI brain can occasionally result in diagnostic overshadowing,and the application of artificial intelligence to medical imaging may overcome such cognitive biases.展开更多
Objective: The purpose of this study was to differentiate between cerebral amyloid angiopathy (CAA) and hypertension (HTN) based on hemorrhage pattern interpretation. Methods: From June 1994 to Oct., 2000, 83 patients...Objective: The purpose of this study was to differentiate between cerebral amyloid angiopathy (CAA) and hypertension (HTN) based on hemorrhage pattern interpretation. Methods: From June 1994 to Oct., 2000, 83 patients admitted to our service with acute intracerebral hemorrhage (ICH) were investigated retrospectively; 41 patients with histologically proven diagnosis of cerebral amyloid angiography and 42 patients with clear history of hypertension were investigated. Results: Patients with a CAA-related ICH were significantly older than patients with a HTN-related ICH (74.0 years vs 66.5 years, P<0.05). There was a significantly higher number of hematomas≥30ml in CAA (85.3%) when compared with HTN (59.5%). No basal ganglional hemorrhage was seen in CAA, but in 40.5% in HTN. In CAA-related ICH, subarachnoid hemorrhage (SAH) was seen in 26 patients (63.4%) compared to only 11 patients (26.2%) in HTN-related ICH. Intraventricular hemorrhage was seen in 24.4% in CAA, and in 26.2% in HTN. Typical features of CAA-related ICH included lobar distribution affecting mainly the lobar superficial areas, lobulated appearance, rupture into the subarachnoid space, and secondary IVH from the lobar hemorrhage. More specifically, multiplicity of hemorrhage, bilaterality, and repeated episodes also strongly suggest the diagnosis of CAA. Multiple hemorrhages, defined as 2 or more separate hematomas in multiple lobes, accounted for 17.1% in CAA-related ICH. Conclusion: There are certain features in CAA on CT and MRI and in clinical settings. To some extent, these features may contribute to distinguishing CAA from HTN related ICH.展开更多
Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura(TTP)-like syndrome suggest a survival benefit to early treatment with plasma exchange. The following is a report of the eighth such...Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura(TTP)-like syndrome suggest a survival benefit to early treatment with plasma exchange. The following is a report of the eighth such case in the last ten years. A 44-yearold lady known for lupus presented with the nephrotic syndrome and a renal biopsy was consistent with class 4G lupus nephritis. She was given high-dose steroids and cytotoxic therapy, but her induction therapy was complicated by the classic pentad of TTP. She was subsequently treated with another course of high-dose steroids, a different cytotoxic agent, and plasma exchange, with clinical resolution shortly thereafter. Similar to seven recently reported cases of microangiopathy in lupus, this lady's TTP-like syndrome improved dramatically after initiation of plasma exchange, despite not having a severely deficient ADAMTS13. This has implications on both current clinical practice and on the pathogenesis of TTP-like syndromes in lupus.展开更多
Background Cerebral amyloid angiopathy is a common cause of subcortical hemorrhage in older adults.Although open hematoma removal may be performed for severe subcortical hemorrhage,its safety in patients with cerebral...Background Cerebral amyloid angiopathy is a common cause of subcortical hemorrhage in older adults.Although open hematoma removal may be performed for severe subcortical hemorrhage,its safety in patients with cerebral amyloid angiopathy has not been established,and postoperative rebleeding may occur.Therefore,this study aimed to investigate factors associated with postoperative rebleeding.Methods Out of 145 consecutive patients who had undergone craniotomy for surgical removal of subcortical intracerebral hemorrhage between April 2010 and August 2019 at a single institution in Japan,we examined 109 patients with subcortical hemorrhage who met the inclusion criteria.After excluding 30 patients whose tissue samples were unsuitable for the study,the final study cohort comprised 79 patients.Results Of the 79 patients,50(63%)were diagnosed with cerebral amyloid angiopathy(cerebral amyloid angiopathy group)and 29(37%)were not diagnosed with noncerebral amyloid angiopathy(noncerebral amyloid angiopathy group).Postoperative rebleeding occurred in 12 patients(24%)in the cerebral amyloid angiopathy group and in 2 patients(7%)in the noncerebral amyloid angiopathy group.Preoperative prothrombin time-international normalized ratio and intraoperative bleeding volume were significantly associated with postoperative rebleeding in the cerebral amyloid angiopathy group(odds ratio=42.4,95%confidence interval=1.14-1578;p=0.042 and odds ratio=1.005,95%confidence interval=1.001-1.008;p=0.007,respectively).Conclusions Patients with cerebral amyloid angiopathy-related cerebral hemorrhage who are receiving antithrombotic therapy,particularly warfarin therapy,are at a high risk of postoperative rebleeding.Trial registration Registry and Registration Number of the study:19-220,2019/12/23,retrospectively registered.展开更多
Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related an...Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive;consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.展开更多
Cerebral amyloid angiopathy(CAA)is characterized by the deposition of amyloid β-protein(Aβ)in the leptomeningeal and cortical blood vessels,which is an age-dependent risk factor for intracerebral hemorrhage(ICH),isc...Cerebral amyloid angiopathy(CAA)is characterized by the deposition of amyloid β-protein(Aβ)in the leptomeningeal and cortical blood vessels,which is an age-dependent risk factor for intracerebral hemorrhage(ICH),ischemic stroke and contributes to cerebrovascular dysfunction leading to cognitive impairment.However clinical prevention and treatment of the disease is very difficult because of its occult onset and severity of the symptoms.In recent years,many anti-amyloid β immunotherapies have not demonstrated clinical efficacy in subjects with Alzheimer’s disease(AD),and the failure may be due to the deposition of Aβ in the cerebrovascular export pathway resulting in further damage to blood vessels and aggravating CAA.So decreased clearance of Aβ in blood vessels plays a crucial role in the development of CAA and AD,and identification of the molecular pathways involved will provide new targets for treatment.In this review,we mainly describe the mechanisms of Aβ clearance through vessels,especially in terms of some proteins and receptors involved in this process.展开更多
文摘This case report investigates the manifestation of cerebral amyloid angiopathy (CAA) through recurrent Transient Ischemic Attacks (TIAs) in an 82-year-old patient. Despite initial diagnostic complexities, cerebral angiography-MRI revealed features indicative of CAA. Symptomatic treatment resulted in improvement, but the patient later developed a fatal hematoma. The discussion navigates the intricate therapeutic landscape of repetitive TIAs in the elderly with cardiovascular risk factors, emphasizing the pivotal role of cerebral MRI and meticulous bleeding risk management. The conclusion stresses the importance of incorporating SWI sequences, specifically when suspecting a cardioembolic TIA, as a diagnostic measure to explore and exclude CAA in the differential diagnosis. This case report provides valuable insights into these challenges, highlighting the need to consider CAA in relevant cases.
基金Supported by the National Natural Science Foundation of China(No.31871261)the Guizhou Science and Technology Cooperation Foundation[No.ZK(2021)general 423]the Research Initiation Fund for Masters in Affiliated Hospital of Zunyi Medical University(No.2016-43)。
文摘AIM:To report on the clinical features of patients with retinal amyloid angiopathy(RAA)who were identified to be caused by the transthyretin(TTR)Gly83Arg variant.METHODS:Case series of five patients diagnosed with RAA was collected at Affiliated Hospital of Zunyi Medical University from January 2010 to December 2021.The clinical features,therapeutic strategies,and prognoses of all patients were reviewed.RESULTS:Five patients with a mean age of 52.00±7.23y were diagnosed as RAA.These patients were previously diagnosed with hereditary transthyretin amyloidosis caused by the TTR Gly83Arg variant.Vitreous opacity was found in all 10 eyes,and 7 eyes developed RAA 2 to 20y after the onset of hereditary transthyretin amyloidosis.The clinical manifestations were recurrent vitreous hemorrhage in 2 eyes(29%),neovascular glaucoma in 2 eyes(29%),and iris neovascularization in 1 eye(14%).Microangioma lesions were found in all affected eyes that underwent fundus fluorescein angiography(FFA)in this group of cases,and the incidence of the retinal non-perfusion area was 67%.Although no cases of retinal neovascularization were found,the prognosis of visual acuity was not ideal.CONCLUSION:This is the first report of RAA in patients with the TTR Gly83Arg variant.Complications such as RAA and glaucoma will seriously affect the visual prognosis of patients.Thereafter,regular ophthalmic follow-up of patients with hereditary transthyretin amyloidosis is essential.And FFA after vitrectomy is very important,which can help ophthalmologists detect RAA earlier and treat it in time.
文摘BACKGROUND Cerebral proliferative angiopathy(CPA)is a rare subtype of arteriovenous malformation.It is extremely rare in pediatric patients and has serious implications for developing children.However,reports of these disorders worldwide are limited,and no uniform reference for diagnosis and treatment options exists.We report the case of a 6-year-old with CPA having predominantly neurological dysfunction and review the literature on pediatric CPA.CASE SUMMARY We report the case of a pediatric patient with CPA analyzed using digital subtraction angiography(DSA)who presented initially with a neurological disorder as the main manifestation.This case is the basis for further discussion of the clinical presentation,pathogenesis,diagnosis,and treatment of CPA in children.After the cerebral DSA,the patient was treated conservatively with sedation,fluid replacement,and blood anticoagulation.She could not cooperate with the followup magnetic resonance imaging examination because of her young age,and her family declined further treatment because of the surgery’s high risk.She was followed up for 3 months;her symptoms did not worsen.CONCLUSION This report of rare pediatric CPA can inform and advance clinical research on congenital cerebrovascular diseases.
文摘Objective To study the changes of biomarkers in cerebrospinal fluid(CSF) in cerebral amyloid angiopathy(CAA) dementia and Alzheimer's disease.Methods Levels of amyloid protein β(Aβ42,Aβ40) and phosphorylated Tau-protein(P-tau) in CSF and ratio of Aβ42/Aβ40 were tested in 5 cases with CAA dementia and 20 cases with Alzheimer's disease collected at Peking Union Medical College Hospital from December 2001 to March 2011.Results The levels of Aβ42,Aβ40,and P-tau in CSF and ratio of Aβ42/Aβ40 were(660.4±265.2) ng/L,(7111.0±1033.4) ng/L,(71.8±51.5) ng/L,and 0.077±0.033,respectively in CAA dementia and(663.6±365.6) ng/L,(5115.0±2931.1) ng/L,(47.7±38.8) ng/L,and 0.192±0.140,respectively in Alzheimer's disease patients.There were no statistically significant differences between CAA dementia and Alzheimer's disease in terms of these CSF biomarkers(all P>0.05).Conclusion Measurements of CSF biomarkers may not be helpful in differential diagnosis of CAA and Alzheimer's disease.
文摘Purpose: We propose an analysis of dyscirculatory angiopathy of Alzheimer’s type (DAAT) endovascular treatment method based on transcatheter revascularization and recovery of collateral and microvascular bed of the brain by means of low-energy transluminal laser irradiation as well as its comparison with traditional Alzheimer’s disease (AD) treatment methods. Methods: The research involved 81 patients aged 34 - 79 (average age 67). 46 (46.8%) patients were treated using endovascular method—Test Group. 35 (43.2%) patients were given conventional treatment—Control Group. Patients were subdivided: Group (CDR-0): 9 (11.1%), pre-clinical stage or increased AD risk;Group (CDR-1): 24 (29.6%), mild dementia and cognitive impairment;Group (CDR-2): 31 (38.3%), moderate dementia and persistent cognitive impairment;Group (CDR-3): 17 (21.0%), severe dementia and cognitive impairment. Research plan included CT or MRI with subsequent temporal lobes volume calculation, brain scintigraphy (SG), rheoencephalography (REG), and cerebral MUGA. There were indications and contraindications for treatment in Test Group. In Group CDR-0, endovascular intervention was prophylactic, against the background of increasing memory impairment;in Groups CDR-1, CDR-2, CDR-3, it was conducted in 1 to 12 years period from AD symptoms appear-ance. Conservative treatment with Memantin and Rivastigmine was carried out in Control Group. Results: In Test Group, positive outcome accompanied by prolonged dementia decline, cognitive impairment decrease, and patients’ transition to CDR group of an earlier stage, was obtained in all cases. In Control Group, patients’ temporary stabilization in their own CDR group was achieved. Conclusions: Endovascular treatment of patients with AD different stages can not only reduce DAAT phenomena but can also cause AD regression possibly accompanied by regenerative processes in the cerebral tissue. Conservative treatment only allows stabilizing the patient’s condition for a while.
基金Supported by National Natural Science Foundation of China,No.81771154.
文摘BACKGROUND Cerebral proliferative angiopathy(CPA)is a rare vascular disease characterized by the presence of diffuse vascular proliferation,progressive vascular hyperflow and vasodilation of multiple vessels in the normal brain parenchyma.Unlike cerebral arteriovenous malformations,CPA has a mixed appearance between that of lesions with cell proliferation and endothelial proliferation.To date,the pathogenesis of CPA is unclear,in which changes induced by cortical ischemia in the elastic layer of the blood supply artery and smooth muscle cells may be involved.CASE SUMMARY In this article,we retrospectively analyzed a case of hemorrhagic transformation of ischemic CPA diagnosed by digital subtraction angiography and reviewed the related literature for further exploration of its pathogenesis,diagnosis and treatment.CONCLUSION The information in the present case report may facilitate further clinical research on this cerebrovascular disease.
文摘Purpose: We assess the significance of dyscirculatory angiopathy of Alzheimer’s type (DAAT) in identify- ing the predisposition to the development and diagnosis of Alzheimer’s disease (AD) different stages. Meth- ods: 108 patients took part in the research:1) 49 aged 34-79 suffering from AD or running an increased risk of its development (those not diagnosed with AD but having growing memory disorders without any mani- festations of dementia or specific cognitive impairments, and having 2 or more immediate relatives with AD) - Test Group;2) 59 aged 28-78 suffering from different types of brain lesions accompanied by dementia but not suffering from AD or corresponding to their age norm - Control Group. All the patients underwent MRI, CT with subsequent calculation of the temporal lobes atrophy degree, brain scintigraphy (SG), rheoencepha- lography (REG), and MUGA. Results: Characteristic features of patients with an increased risk of AD as well as at its various stages are: 1) Temporal lobes and hippocampus atrophy ranging from 4% among those with an increased risk of AD to 62% among those at its advanced stages;2) DAAT manifestations: reduction of the capillary bed in the temporal and frontoparietal regions with the development of multiple arterioven- ous shunts of the same localization and correspondent early venous discharge accompanied by venous stasis on the border of the frontal and parietal region;3) DAAT phenomena equally develop both among those with an increased risk of developing AD and those at various AD stages. Similar changes are not observed among Control Group patients with other brain lesions, regardless of the severity of dementia, as well as among practically healthy people of the corresponding age group. Conclusion: Timely identification of the above- mentioned changes can reveal a predisposition to AD development long before its initial manifestations, and it allows differentiating AD from other diseases attended by dementia. In both cases, timely diagnosis allows beginning timely treatment and thus achieving more stable results.
文摘Objective: To evaluate the incidence of cervical angiopathy caused by radiation therapy for head and neck cancer. Methods: Segments of 57 cervical arteries were obtained during surgery for head and neck malignant tumors and divided into two groups (irradiated group and non-irradiated group) based on the treatment prior to vascular resection. In order to evaluate vascular injury after radiation therapy, we examined the degree of medial atrophy, medial fibrosis, smooth muscle cell (SMC) differentiation in the media and intima, intimal hyperplasia and endothelial cell (EC) injury. Sections of arterial segments were stained with hematoxylin-eosin, Elastica van Gieson and Masson’s trichrome, and immunohistochemistry for α-smooth muscle actin (α-SMA), smoothelin, S100A4 and CD31 in the resected vessels was conducted. Results: The median interval between the completion of radiation therapy and vascular resection was nine months. No significant differences were observed between the two groups in terms of medial atrophy, medial fibrosis and intimal hyperplasia. The ratio of the smoothelin-positive area per α-SMA-positive area in the media and the S100A4-positive proportion in the intima, indicating the degree of differentiation of the medial SMC and dedifferentiation of the intimal SMC, respectively, showed no significant differences, despite the tendency toward a lower smoothelin-positive area per α-SMA-positive area in the media of the irradiated arteries. The EC coverage revealed on CD31 immunohistochemistry was significantly decreased, with mural thrombus adhesion, in the irradiated group. Conclusions: The ECs of small arteries are damaged by irradiation. Although we did not confirm the statistical significance of medial SMC dedifferentiation, a decreased expression of smoothelin tended to be observed in the media of the irradiated arteries. Our findings provide histopathological evidence of irradiation angiopathy in head and neck cancer and may help to improve the surgical safety of microvascular anastomosis and determine the treatment strategy for head and neck tumors.
文摘BACKGROUND Diagnosis of a dementia subtype can be complex and often requires comprehensive cognitive assessment and dedicated neuroimaging.Clinicians are prone to cognitive biases when reviewing such images.We present a case of cognitive impairment and demonstrate that initial imaging may have resulted in misleading the diagnosis due to such cognitive biases.CASE SUMMARY A 76-year-old man with no cognitive impairment presented with acute onset word finding difficulty with unremarkable blood tests and neurological examination.Magnetic resonance imaging(MRI)demonstrated multiple foci of periventricular and subcortical microhaemorrhage,consistent with cerebral amyloid angiopathy(CAA).Cognitive assessment of this patient demonstrated marked impairment mainly in verbal fluency and memory.However,processing speed and executive function are most affected in CAA,whereas episodic memory is relatively preserved,unlike in other causes of cognitive impairment,such as Alzheimer’s dementia(AD).This raised the question of an underlying diagnosis of dementia.Repeat MRI with dedicated coronal views demonstrated mesial temporal lobe atrophy which is consistent with AD.CONCLUSION MRI brain can occasionally result in diagnostic overshadowing,and the application of artificial intelligence to medical imaging may overcome such cognitive biases.
文摘Objective: The purpose of this study was to differentiate between cerebral amyloid angiopathy (CAA) and hypertension (HTN) based on hemorrhage pattern interpretation. Methods: From June 1994 to Oct., 2000, 83 patients admitted to our service with acute intracerebral hemorrhage (ICH) were investigated retrospectively; 41 patients with histologically proven diagnosis of cerebral amyloid angiography and 42 patients with clear history of hypertension were investigated. Results: Patients with a CAA-related ICH were significantly older than patients with a HTN-related ICH (74.0 years vs 66.5 years, P<0.05). There was a significantly higher number of hematomas≥30ml in CAA (85.3%) when compared with HTN (59.5%). No basal ganglional hemorrhage was seen in CAA, but in 40.5% in HTN. In CAA-related ICH, subarachnoid hemorrhage (SAH) was seen in 26 patients (63.4%) compared to only 11 patients (26.2%) in HTN-related ICH. Intraventricular hemorrhage was seen in 24.4% in CAA, and in 26.2% in HTN. Typical features of CAA-related ICH included lobar distribution affecting mainly the lobar superficial areas, lobulated appearance, rupture into the subarachnoid space, and secondary IVH from the lobar hemorrhage. More specifically, multiplicity of hemorrhage, bilaterality, and repeated episodes also strongly suggest the diagnosis of CAA. Multiple hemorrhages, defined as 2 or more separate hematomas in multiple lobes, accounted for 17.1% in CAA-related ICH. Conclusion: There are certain features in CAA on CT and MRI and in clinical settings. To some extent, these features may contribute to distinguishing CAA from HTN related ICH.
文摘Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura(TTP)-like syndrome suggest a survival benefit to early treatment with plasma exchange. The following is a report of the eighth such case in the last ten years. A 44-yearold lady known for lupus presented with the nephrotic syndrome and a renal biopsy was consistent with class 4G lupus nephritis. She was given high-dose steroids and cytotoxic therapy, but her induction therapy was complicated by the classic pentad of TTP. She was subsequently treated with another course of high-dose steroids, a different cytotoxic agent, and plasma exchange, with clinical resolution shortly thereafter. Similar to seven recently reported cases of microangiopathy in lupus, this lady's TTP-like syndrome improved dramatically after initiation of plasma exchange, despite not having a severely deficient ADAMTS13. This has implications on both current clinical practice and on the pathogenesis of TTP-like syndromes in lupus.
基金The Hidaka Research Projects(grant number:01-D-1-07)
文摘Background Cerebral amyloid angiopathy is a common cause of subcortical hemorrhage in older adults.Although open hematoma removal may be performed for severe subcortical hemorrhage,its safety in patients with cerebral amyloid angiopathy has not been established,and postoperative rebleeding may occur.Therefore,this study aimed to investigate factors associated with postoperative rebleeding.Methods Out of 145 consecutive patients who had undergone craniotomy for surgical removal of subcortical intracerebral hemorrhage between April 2010 and August 2019 at a single institution in Japan,we examined 109 patients with subcortical hemorrhage who met the inclusion criteria.After excluding 30 patients whose tissue samples were unsuitable for the study,the final study cohort comprised 79 patients.Results Of the 79 patients,50(63%)were diagnosed with cerebral amyloid angiopathy(cerebral amyloid angiopathy group)and 29(37%)were not diagnosed with noncerebral amyloid angiopathy(noncerebral amyloid angiopathy group).Postoperative rebleeding occurred in 12 patients(24%)in the cerebral amyloid angiopathy group and in 2 patients(7%)in the noncerebral amyloid angiopathy group.Preoperative prothrombin time-international normalized ratio and intraoperative bleeding volume were significantly associated with postoperative rebleeding in the cerebral amyloid angiopathy group(odds ratio=42.4,95%confidence interval=1.14-1578;p=0.042 and odds ratio=1.005,95%confidence interval=1.001-1.008;p=0.007,respectively).Conclusions Patients with cerebral amyloid angiopathy-related cerebral hemorrhage who are receiving antithrombotic therapy,particularly warfarin therapy,are at a high risk of postoperative rebleeding.Trial registration Registry and Registration Number of the study:19-220,2019/12/23,retrospectively registered.
基金National Key Research and Development Program of China(No.2016YFC1300500-505)。
文摘Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive;consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.
基金This work was supported by national natural science grant(81570035).
文摘Cerebral amyloid angiopathy(CAA)is characterized by the deposition of amyloid β-protein(Aβ)in the leptomeningeal and cortical blood vessels,which is an age-dependent risk factor for intracerebral hemorrhage(ICH),ischemic stroke and contributes to cerebrovascular dysfunction leading to cognitive impairment.However clinical prevention and treatment of the disease is very difficult because of its occult onset and severity of the symptoms.In recent years,many anti-amyloid β immunotherapies have not demonstrated clinical efficacy in subjects with Alzheimer’s disease(AD),and the failure may be due to the deposition of Aβ in the cerebrovascular export pathway resulting in further damage to blood vessels and aggravating CAA.So decreased clearance of Aβ in blood vessels plays a crucial role in the development of CAA and AD,and identification of the molecular pathways involved will provide new targets for treatment.In this review,we mainly describe the mechanisms of Aβ clearance through vessels,especially in terms of some proteins and receptors involved in this process.
