Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
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A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. ⋯ To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression.
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Mutations in the glucocerebrosidase (GBA1) gene, the most common genetic contributor to Parkinson's disease (PD), are associated with an increased risk of PD in heterozygous and homozygous carriers. While glucocerebrosidase enzyme (GCase) activity is consistently low in Gaucher disease, there is a range of leukocyte GCase activity in healthy heterozygous GBA1 mutation carriers. ⋯ Therefore, GCase activity appears to be a possible marker of heterozygous GBA1 mutation PD, and larger studies are warranted. Prospective studies are also necessary to determine whether lower GCase activity precedes development of PD.
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Atypical meningiomas have a greater propensity to recur than benign meningiomas and the benefits of early adjuvant radiotherapy are unclear. Existing studies report conflicting results. This retrospective cohort study evaluated the role of early adjuvant radiotherapy following surgical resection of atypical meningioma. ⋯ Following GTR, early adjuvant radiotherapy was administered to 28.3% of patients and did not influence OS (5year OS 77.0% versus 75.7%, log-rank=0.075, p=0.784) or PFS (5year PFS 82.0% versus 79.3%, log-rank=0.059, p=0.808). Although extent of resection emerged as an important prognostic variable, early adjuvant radiotherapy did not influence outcome following GTR of atypical meningiomas. Prospective randomized controlled trials are planned.
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Recently, several randomized controlled trials (RCT) investigating the effectiveness of decompressive craniectomy in the context of neurocritical illnesses have been completed. Thus, a meta-analysis to update the current evidence regarding the effects of decompressive craniectomy is necessary. We searched PUBMED, EMBASE and the Cochrane Central Register of Controlled Trials. ⋯ Decompressive craniectomy significantly reduced the risk of death for patients suffering malignant MCAI (risk ratio [RR] 0.46, 95% confidence interval [CI]: 0.36-0.59, P<0.00001) in comparison with no reduction in the risk of death for patients with severe TBI (RR: 0.83, 95% CI: 0.48-1.42, P=0.49). However, there was no significant difference in the composite risk of death or dependence at the final follow-up between the decompressive craniectomy group and the conservative treatment group for either malignant MCAI or severe TBI. The present meta-analysis indicates that decompressive craniectomy can significantly reduce the risk of death for patients with malignant MCAI, although no evidence demonstrates that decompressive craniectomy is associated with a reduced risk of death or dependence for TBI patients.
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Randomized Controlled Trial
An equiosmolar study on early intracranial physiology and long term outcome in severe traumatic brain injury comparing mannitol and hypertonic saline.
The impact of hypertonic saline (HTS) on long term control of intracranial hypertension (ICH) is yet to be established. The current prospective randomized controlled study was carried out in 38 patients with severe traumatic brain injury (TBI). Over 450 episodes of refractory ICH were treated with equiosmolar boluses of 20% mannitol in 20 patients and 3.0% HTS in 18 subjects. ⋯ In-hospital mortality tended to be lower in the HTS group (3 versus 10; p=0.07) while mortality at 6 months was not different between the groups (6 versus 10; p=0.41). Dichotomized Glasgow Outcome Scale scores at 6months were comparable between the groups (p=0.21). To conclude, immediate physiological advantages seen with HTS over mannitol did not translate into long term benefit on ICP/CPP control or mortality of patients with TBI.