Spinal cord injury without radiographic abnormality (SCIWORA) in adults: MRI type predicts early neurologic outcome.

OBJECTIVES: The present study aimed to analyse the clinical and neuroimaging features of a consecutive series of adult patients with spinal cord injury without radiographic abnormality (SCIWORA) receiving early magnetic resonance imaging (MRI), and to apply the recently proposed MRI classification system. METHODS: Grade of neurologic impairment at admission and discharge was reported according to the American Spinal Injury Association Impairment Scale (AIS). A detailed analysis and categorisation of the extra- and intramedullary MRI findings was performed, and the relationship between imaging type and neurological outcome was described. RESULTS: Twenty-six adult patients (17 male and 9 female) with SCIWORA were identified (mean age of 52 years). The distribution of the initial AIS grade was 8% A (n=2), 19% B (n=5), 31% C (n=8) and 42% D (n=11) at admission and 15% (n=4) C, 58% (n=15) D and 27% (n=7) E at discharge, respectively. Type I SCIWORA was found in 23% (n=6) and type II in 77% (n=20) (IIa: 0%, IIb: 25%, IIc: 75%). The mean improvement of AIS grade in patients with type I lesions was 1.5 (median 1, range 1-3) and 0.9 (median 1, range 0-3) in type II. CONCLUSION: The findings underline the prognostic role of early MRI for adult patients with SCIWORA and support the use of the recently introduced MRI classification system. LEVEL OF EVIDENCE: Prognostic study, level III.

Inheritance of moyamoya disease in a Caucasian family.

BACKGROUND AND PURPOSE: Moyamoya disease is a very rare occlusive cerebrovascular disorder characterized by progressive stenosis or occlusion of the intracranial portion of the internal carotid artery and proximal cerebral arteries with an extensive network of fine collaterals. The aetiology and genetic susceptibility of moyamoya disease, especially in Caucasians, still remains unclear. METHODS AND RESULTS: We describe the cases of affected German father, daughter and son with juvenile stroke because of idiopathic moyamoya disease. The rare existing literature is reviewed and discussed. CONCLUSIONS: This is the first report on a father-to-child inheritance pattern in Caucasian patients with idiopathic Moyamoya disease (MMD). Our cases indicate possible genetic risk factors for the genesis of Caucasian Moyamoya disease.

Low-Profile Intra-Aneurysmal Flow Disruptor WEB 17 versus WEB Predecessor Systems for Treatment of Small Intracranial Aneurysms: Comparative Analysis of Procedural Safety and Feasibility.

BACKGROUND AND PURPOSE: The Woven EndoBridge 17 has recently been introduced to the market for facilitated endovascular treatment of small bifurcation aneurysms (≤7 mm) with low-profile microcatheters. We compared the Woven EndoBridge 17 with its predecessor versions in terms of procedural safety and feasibility. MATERIALS AND METHODS: This was a multicenter review of aneurysms ranging from 3 to 7 mm treated with the Woven EndoBridge between 2011 and 2019. Aneurysm characteristics, procedural parameters, and complications were retrospectively compared between treatment with the Woven EndoBridge 17 and a control group that was treated with its predecessor versions, using inverse probability of treatment weighting. RESULTS: Thirty-eight aneurysms treated with a Woven EndoBridge 17 (mean size, 4.9 ± 1.5 mm) and 70 treated with a predecessor version of the Woven EndoBridge 17 (mean size, 5.6 ± 1.4 mm) were included. The predecessor version of the Woven EndoBridge 17 had a higher failure rate (10.3%) than the Woven EndoBridge 17 (0%, P = .05). Additional stent placement was performed more often with the predecessor version of the Woven EndoBridge 17 (10.0%) than with the Woven EndoBridge 17 (2.6%, adjusted P = .005). The predecessor version of the Woven EndoBridge 17 was associated with a higher thromboembolic event rate (14.3%) than the Woven EndoBridge 17 (5.3%, adjusted P = .002). Neurologic complications (Woven EndoBridge 17: 2.6%; predecessor version of the Woven EndoBridge 17: 2.9%, adjusted P = 1.0) and immediate complete aneurysm occlusion rates (Woven EndoBridge 17: 57.9%; predecessor version of the Woven EndoBridge 17: 54.3%, adjusted P = .21) did not differ significantly between groups. CONCLUSIONS: In the current study, the Woven EndoBridge 17 was associated with a potentially lower thromboembolic event rate than the predecessor version of the Woven EndoBridge 17, without compromising the immediate aneurysm occlusion rate. Long-term clinical and angiographic outcome analysis will be necessary to draw a definite conclusion.

The influence of genetics on intracranial aneurysm formation and rupture: current knowledge and its possible impact on future treatment.

The etiology of intracranial aneurysm formation and rupture remains mostly unknown, but lately several studies have increasingly supported the role of genetic factors. In reports so far, genome-wide linkage studies suggest several susceptibility loci that may contain one or more predisposing genes. Depending on the examined ethnic population, several different non-matching chromosomal regions have been found. Studies of several candidate genes report association with intracranial aneurysms. To date, no single gene has been identified as responsible for intracranial aneurysm formation or rupture. In addition to the well-published environmental factors, such as alcohol intake, hypertension and smoking, only the recent progress in molecular genetics enables us to investigate the possible genetic determinants of this disease. Although a familial predisposition is the strongest risk factor for the development of intracranial aneurysms, the mode of Mendelian inheritance is uncertain in most families. Therefore, multiple genetic susceptibilities in conjunction with the environmental factors are considered to act together in the disease's etiology. Accordingly, researchers performed linkage studies and case-control association studies for the genetic analysis and have identified several genes to be susceptible to intracranial aneurysms. The identification of susceptible genes may lead to the understanding of the mechanism of formation and rupture and possibly lead to the development of a pharmacological therapy. Furthermore, should it be possible to identify a genetic marker associated with an increased risk of formation and rupture of an intracranial aneurysm, the necessity for screening and urgency of treatment could be determined more easily. In this review we summarize the current knowledge of intracranial aneurysm genetics and also discuss the method to detect the causalities. In view of the recent advances made in this field, we also give an outlook on possible future genetically engineered therapies, whose development are well underway.

Navigated Transtubular Extraforaminal Decompression of the L5 Nerve Root at the Lumbosacral Junction: Clinical Data, Radiographic Features, and Outcome Analysis.

Purpose. Extraforaminal decompression of the L5 nerve root remains a challenge due to anatomic constraints, severe level-degeneration, and variable anatomy. The purpose of this study is to introduce the use of navigation for transmuscular transtubular decompression at the L5/S1 level and report on radiological features and clinical outcome. Methods. Ten patients who underwent a navigation-assisted extraforaminal decompression of the L5 nerve root were retrospectively analyzed. Results. Six patients had an extraforaminal herniated disc and four had a foraminal stenosis. The distance between the L5 transverse process and the para-articular notch of the sacrum was 12.1 mm in patients with a herniated disc and 8.1 mm in those with a foraminal stenosis. One patient had an early recurrence and another developed dysesthesia that resolved after 3 months. There was a significant improvement from preoperative to postoperative NRS with the results being sustainable at follow-up. ODI was also significantly improved after surgery. According to the Macnab grading scale, excellent or good outcomes were obtained in 8 patients and fair ones in 2. Conclusions. The navigated transmuscular transtubular approach to the lumbosacral junction allows for optimal placement of the retractor and excellent orientation particularly for foraminal stenosis or in cases of complex anatomy.

Diagnostic and microsurgical presentation of intracranial angiolipomas. Case report and review of the literature.

Angiolipomas (ALs) are hamartomas composed of abnormally differentiated vessels and mature adipose tissue. Although they are most commonly found in peripheral tissues, ALs sometimes grow in the spinal epidural space. Intracranial ALs (ICALs) are rare: only seven cases have been reported in the literature. The authors describe the case of a 70-year-old woman who presented with ocular symptoms from a clinically and radiologically progressing parasellar ICAL. The radiological as well as the microsurgical findings are illustrated and compared with the seven previously published cases. The most frequent location of ALs is the skull base, especially the parasellar region. Other ICALs were diagnosed as components of cerebral arteriovenous malformations and were not symptomatic by themselves. Neuroradiological studies of ICALs usually demonstrate the characteristics of both adipose and vascular tissues. However, a review of the literature shows that the diagnosis had not been suspected preoperatively in any of the cases. Operative descriptions emphasize that most neurosurgeons were caught off guard by the profuse bleeding and the unusual relationship of this unexpected lesion to the cavernous sinus, so that removal was rarely complete. The authors conclude that preoperative diagnosis of ICALs is achievable based on magnetic resonance analysis, which should help optimize the microsurgical management of these lesions.

Heparin-induced thrombocytopenia: a critical risk/benefit analysis of patients in intensive care treated with R-hirudin.

Patients in intensive care may be at high risk of in vivo platelet activation because comorbid conditions, such as infections, septicemia, shock, disseminated intravascular coagulation, and cancer represent procoagulant states. Hyperreactivity of platelets with or without a decline of cell count may result in thromboembolic complications potentially associated with the phenomenon of heparin-induced thrombocytopenia. We analyzed the data of 10 patients highly suspected of having heparin-induced thrombocytopenia during their intensive care treatment of 29 plus or minus 22 days. In seven patients, thrombocytopenia coincided with thromboembolic complications. Six patients had additionally undergone fibrinolytic therapy before starting activated partial thromboplastin time-adapted alternative anticoagulation with r-hirudin. In three patients, the platelet count decreased without a clinical manifestation, of heparin-induced thrombocytopenia. R-Hirudin treatment monitored by activated partial thromboplastin time and prothrombin time (PT) was effective and safe. The target value for activated partial thromboplastin time was a twofold prolongation. In four of five patients with deep venous thrombosis, a partial recanalization of the lower extremity could be achieved. Three patients with pulmonary embolism associated with deep venous thrombosis in two cases and in one additional case with an acute myocardial infarction did clinically profit from fibrinolysis with recombinant tissue plasminogen activator (rtPA) and r-hirudin treatment. Two lethal events probably caused by the underlying multimorbidity could not be prevented. No recurrence of thrombosis occurred, and there were no severe bleeding complications attributed to r-hirudin treatment. Platelet counts were significantly reduced on day 9.4 plus or minus 6.4 of heparin administration in all cases (>50% decrease related to the initial values) from 224,000 plus or minus 126,000/microL to 96,000 plus or minus 61,000/microL, and increased during rhirudin treatment to mean values of 224,000 plus or minus 126,000/microL. The heparin-induced platelet activation assay (HIPAA) assay was positive in 8/10 cases, whereas the PF4 enzyme-linked immunosorbent assay showed a positive result in four of eight analyzed cases. In four cases, the assays were concordantly positive. The PF4 enzyme-linked immunosorbent assay was not performed in two cases.

Meta-analysis of microarray gene expression studies on intracranial aneurysms.

The rupture of intracranial aneurysms (IAs) is one of the most devastating neurological conditions known to date. Although treatment has changed dramatically throughout the last decades, the outcome of patients still has a poor prognosis. Besides environmental factors, genomics seem to be a very important factor in the genesis of this disease. Different approaches to decrypt genomic causes were pursued throughout the last years. Microarray gene expression studies comparing aneurysmal and healthy tissue seem to be one of the most promising approaches. However, large amounts of data created with each study, make a comparison or interpretation of results difficult. We analyzed microarray gene expression studies on IAs (vs. control tissue) and compared lists of genes with altered expression provided by the authors. Additionally functional pathway analysis was performed. We identified five microarray gene expression studies analyzing a total of 60 samples of IA tissue (30 ruptured IA, 30 unruptured IA). A total of 507 genes with altered expression were listed, of which 57 showed differences in more than two studies and seven in more than three studies (BCL2, COL1A2, COL3A1, COL5A2, CXCL12, TIMP4, TNC). The meta-analysis of five microarray gene expression studies on IAs revealed seven genes that are very likely to be involved in the genesis of IAs. Further analysis of these genes might provide valuable information on mechanisms causing this disease.