Clinical impact of CCM mutation detection in familial cavernous angioma

Introduction and background A 3-year-old Bosnian girl with a large symptomatic brainstem and multiple supratentorial cavernous angiomas, who underwent neurosurgical treatment, is presented. As multiple cavernomas are more common in familial cases, genetic analyses and neuroradiological imaging were performed in the patient and her parents to see whether there was any evidence for inheritance. This information is important for genetic counseling and provision of medical care for at-risk relatives. Currently, no recommendation is available on how to manage these cases.Results Genetic analyses demonstrated a novel CCM1 frameshift mutation (c.1683_1684insA; p.V562SfsX6) in the child and the asymptomatic 27-year-old mother. Sensitive gradient-echo magnetic resonance imaging of the mother revealed multiple supratentorial lesions, whereas analogous imaging of the father showed no pathological findings.Conclusion This case exemplifies that seemingly sporadic cases with multiple lesions might well be hereditary and that presymptomatic genetic testing of family members may identify relatives for whom clinical and neuroradiological monitoring is indicated.     

Raised intracranial pressure,the syndrome of inappropriate antidiuretic hormone secretion,and arginine vasopressin in tuberculous meningitis

Intracranial pressure (ICP) monitored shortly after admission over a period of 1 h in 31 children with tuberculous meningitis (TBM) was significantly higher (median 22.5 mm Hg, range 8.4–50.9 mmHg) in 19 children with laboratory evidence of the syndrome of inappropriate antidiuretic hormone secretion (SIADH) than in 12 children without such evidence (median 16.2 mmHg, range 5.8–42.5 mmHg; P = 0.027). Neither plasma nor cerebrospinal fluid arginine vasopressin (AVP) was related to ICP (r = 0.33 and 0.13 respectively). Mean arterial pressure (MAP) was measured in 23 children and a moderate correlation was found with plasma AVP (r = 0.62; P = 0.0019). In TBM, plasma AVP may be secreted as a response to raised ICP in an effort to raise MAP and maintain cerebral perfusion pressure. In this setting excess fluid may be inappropriately retained, leading to hyponatremia and hypo-osmolemia.     

急性脑出血患者血清谷氨酸动态变化分析

目的通过观察脑出血（intracerebral hemorrhage，ICH）患者血清中谷氨酸的动态变化来进一步评价谷氨酸对脑出血的作用。方法选择符合条件的30例急性高血压脑实质出血患者，给予神经内科脑出血常规治疗，采用高效液相色谱法检测起病后2d内、5．6d、8-9d和15．16d的血清谷氨酸浓度，同步进行神经功能评定，同时于发病后1．3d、d5-6d及d15．16行头部CT检查了解血肿体积大小。结果脑出血患者血中谷氨酸浓度与血肿体积及神经功能缺损评分之间呈正的直线相关。经治疗后，所有病例谷氨酸水平迅速下降，在5-6d和8—9d下降速度更加明显。结论谷氨酸可能参与了自发性脑出血患者的急性期脑损伤作用。     

MRI relaxation fluctuations in acute reperfused hemorrhagic infarction.

MRI evaluations of intramyocardial hemorrhage in acute infarction have relied on T(2) and T(2)(*) shortening only. We propose a more comprehensive evaluation of hemorrhagic infarction based on the concept that fluctuations in T(2) and T(1) relaxation in acute reperfused infarction will reflect transient edema and hemoglobin oxidative denaturation to uncompartmentalized methemoglobin. Anteroapical infarction was created via percutaneous balloon in young swine (22-25 kg, N = 12). T(2), T(1), diastolic wall thickness (DWT), and the Gd-DTPA partition coefficient (lambda) were measured on days 0, 2, and 7. DWT was elevated at 1 hr postreperfusion (128% +/- 53%, P = 0.0001), and alleviated on days 2 and 7 (48% +/- 10%, P = 0.008; 53% +/- 24%, P = 0.003). T(2) and T(1) elevations were coincident with early edema (DeltaT(2) = 55% +/- 24%, P < 0.0001; DeltaT(1) = 27% +/- 18%, P < 0.04). T(2) and T(1) were nearly normal on day 2 (DeltaT(2) = 8% +/- 8%, P = 0.27; DeltaT(1) = 0% +/- 1%, P = 0.65). On day 7, T(2) increased while T(1) decreased (DeltaT(2) = 27% +/- 16%, P = 0.005; DeltaT(1) = -14% +/- 10%, P = 0.02). Lambda was elevated by >150% at all time points (P < or = 0.002). Histology verified hemorrhagic injury. T(1) and T(2) fluctuations are consistent with transient edema, as well as hemoglobin oxidative denaturation to decompartmentalized methemoglobin. This methodological development may broaden our understanding of hemorrhagic microvascular injury and improve its detection in clinical populations.     

Misoprostol in preventing postpartum hemorrhage: a meta-analysis.

OBJECTIVE: To assess misoprostol's ability to prevent postpartum hemorrhage (PPH) where no alternatives exist. Comparison to oxytocics demonstrates how similarly misoprostol achieves a level of effectiveness-obtainable only in hospitals-in remote locations around the world. METHOD: Using the Mantel-Haenszel fixed-effects model and the DerSirmonian and Laird random-effects model, summary statistics indicated that misoprostol's excess risk of PPH was only 4% when compared to oxytocics. RESULT: This risk difference was well within the range of expected results for all uterotonic agents and does not warrant branding misoprostol as an inferior drug. CONCLUSION: Conventional uterotonic drugs should not be used to set the lowest-accepted level of effectiveness in settings where they are entirely unsuitable. Continuing to weigh the benefits of one effective drug against another only delays the distribution of misoprostol in countries where it is the only feasible choice and must be measured against no treatment at all.     

脑梗死后再发缺血性卒中与脑出血患者微血管病变及预后的比较

【目的】探讨脑梗死后再发缺血性卒中与脑出血患者的临床特点、影像学上微血管病变及预后的异同。【方法】连续收集急性复发性卒中的住院病例（初发事件为脑梗死，复发事件包括脑梗死或脑出血），详细记录临床资料。观察脑内微出血及自质病变的严重程度，随访有无严重血管事件及死亡的发生。【结果】175例再发脑梗死患者。19例复发事件为脑出血的患者进入本研究。155例（88．6％）再发脑梗死患者，以及18例（94．7％）脑出血患者存在自质病变。77例（44％）再发脑梗死患者和16例（84．2％）脑出血患者存在脑内微出血。脑出血组自质病变的严重程度及微出血的数目均明显高于再发脑梗死组（P=0．033，0．016），脑出血组的死亡率及再发脑出血的危险性明显增高（P=0．034，0．036）。【结论】严重的脑自质病变及微出血的出现与脑梗死后再发脑出血有关。多发微出血提示出血风险增高。如复发事件为脑出血，则其死亡率及再次复发脑出血的风险均明显增加。     

Nimodipine treatment of subarachnoid hemorrhage

In a pilot study of 6 patients with subarachnoid hemorrhage caused by a ruptured intracranial (grade IV (Hunt and Hess) aneurysm the hemodynamics and plasma-nimodipine concentrations have been observed during a 3-week period of treatment. We found that 3 patients developed reversible hypotension during the nimodipine treatment and that the hypotension tendency could be related to the plasmanimodipine level and not to a more or less severe sensitivity to nimodipine.

Repeated measurements of blood pressure, plasma-nimodipine and regional cerebral blood flow (rCBF) are necessary for the purpose of obtaining the optimum treatment and for evaluating the effect of treatment.     

足月新生儿颅内出血的CT诊断

经CT扫描诊断的足月新生儿颅内出血8例,就其病因、CT表现特点及CT与超声检查的比较进行讨论。提出新生儿颅内出血与孕期、胎次、分娩方式均有一定的关系,同时还应注意新生儿因脑发育不成熟在CT扫描图像上有其特殊表现。     

Computed tomographic findings in cerebral toxoplasmosis in adults

The clinical and computerized tomographic (CT) findings in 10 patients with cerebral toxoplasmosis are reviewed. All patients with cerebral toxoplasmosis were homosexuals and/or intravenous drug users, and all patients had other manifestations of AIDS. Two presented with focal seizures, 4 presented with focal neurological deficit which progressively worsened, and 4 had evidence of diffuse neurological dysfunction (altered consciousness, generalized seizures). CT showed a single lesion in 3 patients and multiple lesions in 7 patients. Two patients had hypodense nonenhancing lesions(s). Eight patients had hypodense lesions with peripheral or nodular enhancement. The lesions were more commonly located in the cerebral hemispheres and subcortical gray matter nuclear masses (thalamus, basal ganglia). The finding of a hypodense lesion with a central slightly hyperdense noncalcified region that showed dense nodular homogeneous enhancement was quite characteristic of cerebral toxoplasmosis, but this pattern may also be seen in other neurological conditions including brain lymphomas.