Background and purpose
Occlusive cerebrovascular moyamoya disease (MMD) is rare and has been characterized mainly in Asian countries, so far. In recent years, MMD has been increasingly reported ...worldwide, raising the question whether its clinical presentation would vary amongst different ethnic backgrounds. Here, a homogeneous series of 153 patients with MMD are reported and the specific clinical features of this rare disease amongst European Caucasians are highlighted.
Methods
A total of 153 European Caucasians with MMD who were treated in our institution between 1997 and 2014 were retrospectively identified. Demographic data, clinical symptoms, angiographic characteristics and functional hemodynamic studies were analyzed.
Results
Moyamoya disease presented with a female predominance of 2,9:1.,78% presented with a typical MMD, 17% with a unilateral MMD and 5% with an atypical MMD. 16% of our patients belonged to the pediatric population. Overall, 81% and 8.5% of our cohort presented initially with ischaemic and hemorrhagic manifestation, respectively. The rate of hemorrhagic manifestation of MMD amongst the pediatric group was slightly higher (12%). Angiographic analysis revealed steno‐occlusive involvement of the posterior circulation in 34% with a higher involvement in pediatric patients (64%) compared to adults (28%).
Conclusions
The characterization of our homogeneous European Caucasian cohort reveals several significant differences compared to Asian cohorts. In contrast, MMD presents similarly amongst European and North American cohorts, suggesting that non‐Asian MMD is characterized by distinct clinical features.
Objective: To evaluate the results of surgical treatment options for chronic subdural haematoma in contemporary neurosurgery according to evidence based criteria. Methods: A review based on a Medline ...search from 1981 to October 2001 using the phrases “subdural haematoma” and “subdural haematoma AND chronic”. Articles selected for evaluation had at least 10 patients and less than 10% of patients were lost to follow up. The articles were classified by three classes of evidence according to criteria of the American Academy of Neurology. Strength of recommendation for different treatment options was derived from the resulting degrees of certainty. Results: 48 publications were reviewed. There was no article that provided class I evidence. Six articles met criteria for class II evidence and the remainder provided class III evidence. Evaluation of the results showed that twist drill and burr hole craniostomy are safer than craniotomy; burr hole craniostomy and craniotomy are the most effective procedures; and burr hole craniostomy has the best cure to complication ratio (type C recommendation). Irrigation lowers the risk of recurrence in twist drill craniostomy and does not increase the risk of infection (type C recommendation). Drainage reduces the risk of recurrence in burr hole craniostomy, and a frontal position of the drain reduces the risk of recurrence (type B recommendation). Drainage reduces the risk of recurrence in twist drill craniostomy, and the use of a drain does not increase the risk of infection (type C recommendation). Burr hole craniostomy appears to be more effective in treating recurrent haematomas than twist drill craniostomy, and craniotomy should be considered the treatment of last choice for recurrences (type C recommendation). Conclusions: The three principal techniques—twist drill craniostomy, burr hole craniostomy, and craniotomy—used in contemporary neurosurgery for chronic subdural haematoma have different profiles for morbidity, mortality, recurrence rate, and cure rate. Twist drill and burr hole craniostomy can be considered first tier treatment, while craniotomy may be used as second tier treatment. A cumulative summary of data shows that, overall, the postoperative outcome of chronic subdural haematoma has not improved substantially over the past 20 years.
Background
Moyamoya disease (MMD) may be graded based on DSA, the presence of ischemia in MRI and cerebrovascular reserve capacity allowing the prediction of ischemic symptoms in patients. Cerebral ...ischemia represents a severe complication in revascularization surgery. Focusing on different clinical features of hemodynamic impairment, MMD grading may allow prediction of ischemic complications. It was the aim to analyze whether MMD grading stratifies for ischemic complications in revascularization surgery for MMD.
Method
In 37 MMD patients a bilateral, standardized, one-staged revascularization approach consisting of STA-MCA bypass/encephalomyosynangiosis (EMS) and single EMS on the contralateral hemisphere was performed. Clinical data including DSA, MRI and rCBF (Xenon-CT) studies were assessed and used for grading MMD. All patients were observed on the ICU for at least 24 h and received CT imaging on the first postoperative day and in case of neurological deterioration. Ischemic complications were analyzed until the day of discharge and at 6-month follow-up.
Results
Grading of MMD revealed 11 hemispheres (15 %) as grade I, 33 hemispheres (44 %) as grade II and 30 hemispheres (41 %) as grade III. Eight ischemic complications were observed (11 %). MMD grading demonstrated a significant correlation with ischemic complications: 0 complications in grade I, 3 in grade II (9 %) and 5 in grade III hemispheres (16 %;
p
< 0.05, Fisher’s exact test).
Conclusions
The proposed grading system allows to stratify for ischemic complications in MMD patients that receive bilateral, one-staged revascularization surgery. Future studies will have to investigate its use for predicting ischemic complications in other revascularization strategies for MMD.
The best revascularization strategy for moyamoya disease (MMD) remains unknown. Our aim was to characterize angiographic revascularization effects of a bilateral standardized revascularization ...approach, consisting of superficial temporal artery (STA)-middle cerebral artery (MCA) bypass and encephalomyosynangiosis (EMS) on one hemisphere and single EMS on the contralateral hemisphere of each patient, and to compare the effects of both revascularization strategies on cerebral hemodynamics.
In 30 patients (18 females/12 males, age 8-63 years), standardized revascularization was performed. Digital subtraction angiography was performed preoperatively and at 7 days, 6 months and 12 months postoperatively. STA-MCA and EMS functions were graded I (poor), II (medium) or III (extensive) according to angiographic aspects. In 20 patients, cerebrovascular reserve capacity (CVRC) was assessed pre- and postoperatively (at 12 months) using xenon CT.
After 12 months, STA-MCA/EMS function was grade 1 in 40/40%, grade 2 in 27/26%, and grade 3 in 27/10% of hemispheres, respectively. Twelve months after surgery, single EMS showed grade I in 37%, grade II in 27%, and grade III in 20% of hemispheres. Combined revascularization improved CVRC significantly compared to preoperative measurement (preoperative: 16.5 ± 34.6% vs. postoperative: 60.8 ± 64.22%; p < 0.05). Single EMS did not improve CVRC significantly (preoperative: 21.8 ± 35.9% vs. postoperative: 34.8 ± 63.0%; p < 0.05).
Combined and indirect revascularization may be successfully applied in a bilateral standardized approach. STA-MCA/EMS is superior to single EMS in restoring CVRC in adult MMD patients.
Moyamoya disease (MMD) is graded based on digital subtraction angiography (DSA) with limited clinical applications. The aim was to identify clinically relevant parameters that may be used to develop ...a novel MMD grading system.
In 40 MMD patients bilateral revascularization surgery was performed. Clinical data including DSA, MRI and regional cerebral blood flow studies were assessed. χ(2) test corrected for dependency of measurements at the same subject and analysis of receiver operating characteristics were used to identify key parameters. Grading system included: DSA (stenosis/occlusion = 1 point; stenosis/occlusion + intracranial compensation = 2 points; stenosis/occlusion + intracranial compensation + extra-intracranial compensation = 3 points), MRI (no sign of ischemia = 0 points; signs of ischemia = 1 point) and cerebrovascular reserve capacity (CVRC > -5% = 0 points; CVRC < -5% = 2 points). MMD grade I referred to 1-2 points, grade II to 3-4 and grade III to 5-6 points.
DSA, MRI and CVRC were dependent factors associated with the occurrence of clinical symptoms. Receiver operating characteristics analysis indentified the grading system as superior to each single parameter in predicting clinical symptoms. Fourteen hemispheres were graded as mild (grade I), 35 as moderate (grade II) and 31 as severe (grade III); 21% of grade I, 63% of grade II and 93% of grade III hemispheres were clinically symptomatic.
The proposed grading system allows to stratify for clinical symptomatology in MMD patients. Future studies will have to investigate its value for assessing clinical symptoms and treatment risks.
Subarachnoidal release of inflammatory cytokines (interleukin (IL)-1β, IL-6, and tumour necrosis factor (TNF)-α) was characterised in 35 patients with subarachnoid haemorrhage (SAH) and control ...subjects and compared with development of complicating haemodynamic abnormalities in basal cerebral arteries and clinical outcome. Serial analysis allowed the observation of a subacute response profile of these key mediators of inflammation in the subarachnoidal space. This compartmentalised inflammatory host response was closely associated in time and extent with development of increased blood flow velocities in the basal cerebral vessels as recorded by transcranial Doppler sonography. Moreover, intrathecal secretion of inflammatory cytokines was significantly increased in patients with poor clinical outcome. Together, these findings suggest a role of excessive compartmentalised inflammatory host response in pathogenesis of cerebrovascular complications after SAH.
The aim of this prospective study was to evaluate the significance of growth factors as determinants of the pathological degree of neovascularisation found in the parietal neomembrane of chronic ...subdural hematoma (CSH). Thus far the pathogenesis of the vascularisation has not been elucidated.
The concentrations of growth factors, i.e. vascular endothelial derived growth factor (VEGF), basic fibroblast growth factor (bFGF) and platelet derived growth factor (PDGF) were determined using ELISA technique in hematoma fluid and serum of 20 patients with uni- or bilateral CSH. For comparison, growth factor concentrations were determined in cerebrospinal fluid (CSF) of patients undergoing diagnostic myelography.
Concentrations of VEGF and bFGF were significantly (p < 0.001) increased in the hematoma fluid as compared with serum (VEGFh = 8.142 pg/ml, bFGFh = 8.7 pg/ml versus VEGFS = 368 pg/ml, bFGF, = 1.8 pg/ml). In contrast, PDGF concentration was significantly (p < 0.001) lower in the hematoma (PDGFh = 3,456 pg/ml versus PDGF, = 31,937 pg/ml). The serum levels for VEGF, bFGF and PDGF in CSH patients lay within the range of normal volunteers. No growth factors were found in normal CSF.
These results reveal a specific distribution pattern of growth factors in CSH patients. This pattern suggests that CSH may be considered a member of the angiogenic disease family.
Vascular endothelial growth factor (VEGF) plays a fundamental role in mediating tumor angiogenesis and tumor growth. Here we investigate the direct effect of a novel small molecule inhibitor of the ...Flk-1-mediated signal transduction pathway of VEGF, SU5416, on tumor angiogenesis and microhemodynamics of an experimental glioblastoma by using intravital multifluorescence videomicroscopy. SU5416 treatment significantly suppressed tumor growth. In parallel, SU5416 demonstrated a potent antiangiogenic activity, resulting in a significant reduction of both the total and functional vascular density of the tumor microvasculature, which indicates an impaired vascularization as well as significant perfusion failure in treated tumors. This malperfusion was not compensated for by changes in vessel diameter or recruitment of nonperfused vessels. Analyses of the tumor microcirculation revealed significant microhemodynamic changes after angiogenesis blockage such as a higher red blood cell velocity and blood flow in remnant tumor vessels when compared with controls. Our results demonstrate that the novel antiangiogenic concept of targeting the tyrosine kinase of Flk-1/KDR by means of a small molecule inhibitor represents an efficient strategy to control growth and progression of angiogenesis-dependent tumors. This study provides insight into microvascular consequences of Flk-1/KDR targeting in vivo and may have important implications for the future treatment of angiogenesis-dependent neoplasms.
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