Traumatic Clivus Epidural Hematoma: Case Report and Review of the Literature

BACKGROUND: Traumatic clival epidural hematoma is an extremely rare reported entity. CASE DESCRIPTION: We describe the case of a 26-year-old woman involved in a car accident who presented with a Glasgow Coma Scale score of 13, bilateral abducens palsy, bilateral numbness on the mandibular territory of the trigeminal nerve, and left hypoglossal palsy. Radiological examinations revealed a clival epidural hematoma. The patient was managed conservatively, with clinical improvement of her neurological condition. This is the first traumatic clival epidural hematoma reported in an adult. From a review of the literature, we found only 8 cases. CONCLUSION: The pathophysiology of these hematomas is still a subject of debate; occipitoatlantoaxial ligamentous instability may play a role in it. In one third of the cases, bilateral cranial nerve palsies were associated. Apparently, they have a benign outcome.

Primary Angiosarcoma of the Pericardium. Case Report and Review of the Literature

Primary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Mean survival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient with localised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy and surgery.

Safety and Effectiveness of the Genous™ Endothelial Progenitor Cell-Capture Stent in the First Year Following ST-Elevation Acute Myocardial Infarction: A Single Center Experience and Review of the Literature

PURPOSE: The Genous™ stent (GS) is designed to accelerate endothelization, which is potentially useful in the pro-thrombotic environment of ST-elevation acute myocardial infarction (STEMI). We aimed to evaluate the safety and effectiveness of the GS in the first year following primary percutaneous coronary intervention (PCI) and to compare our results with the few previously published studies. METHODS AND MATERIALS: All patients admitted to a single center due to STEMI that underwent primary PCI using exclusively GS, between May 2006 and January 2012, were enrolled. The primary study endpoints were major adverse cardiac events (MACEs), defined as the composite of cardiac death, acute myocardial infarction and target vessel revascularization, at one and 12months. RESULTS: In the cohort of 109 patients (73.4% male, 59 ±12years), 24.8% were diabetic. PCI was performed in 116 lesions with angiographic success in 99.1%, using 148 GS with median diameter of 3.00mm (2.50-4.00) and median length of 15mm (9-33). Cumulative MACEs were 2.8% at one month and 6.4% at 12months. Three stent thromboses (2.8%), all subacute, and one stent restenosis (0.9%) occurred. These accounted for the four target vessel revascularizations (3.7%). At 12months, 33.9% of patients were not on dual antiplatelet therapy. CONCLUSIONS: GS was safe and effective in the first year following primary PCI in STEMI, with an apparently safer profile comparing with the previously published data. SUMMARY: We report the safety and effectiveness of the Genous™ stent (GS) in the first year following primary percutaneous coronary intervention in ST-elevation acute myocardial infarction. A comprehensive review of the few studies that have been published on this subject was included and some suggest a less safe profile of the GS. Our results and the critical review included may add information and reinforce the safety and effectiveness of the GS in ST-elevation in acute myocardial infarction.

Safety and Effectiveness of the Genous Endothelial Progenitor Cell-Capture Stent: Follow-Up to 5 Years

AIMS: To evaluate the long-term clinical outcomes following percutaneous coronary intervention (PCI) with the Genous stent in an unselected population. METHODS: All patients admitted to a single center who underwent PCI using the GS exclusively, between May 2006 and May 2012, were enrolled, and a clinical follow-up of up to 60 months was carried out. The primary endpoint of major adverse cardiac event (MACE) rate was defined as the composite of cardiac death, acute myocardial infarction (AMI), and target lesion revascularization (TLR). RESULTS: Of the 450 patients included (75.1% male; 65.5 ± 11.7 years), 28.4% were diabetic and acute coronary syndrome was the reason for PCI in 76.4%. Angioplasty was performed in 524 lesions using 597 Genous stents, with angiographic success in 97.1%. At a median of 36 months of follow-up (range, 1-75 months), MACE, AMI, TLR, stent restenosis (SR), and stent thrombosis (ST) rates were 15.6%, 8.4%, 4.4%, 3.8%, and 2.2%, respectively. Between 12 and 24 months, the TLR, SR, and ST rates practically stabilized, up to 60 months. Bifurcation lesions were independently associated with MACE, TLR, and SR. CONCLUSION: This is the first study reporting clinical results with the Genous stent up to 60 months. The Genous stent was safe and effective in the long-term, in an unselected population.

Chronic Calcified Subdural Hematoma: Case Report and Review of the Literature

Background: Calcified chronic subdural hematoma is a rare but known entity, estimated to represent 0.3-2.7% of chronic subdural hematomas. Although surgical treatment is unanimous for chronic subdural hematomas, therein lies some doubt on it being applied to calcified chronic subdural hematomas. Case Description: We report a case of a 73‑year‑old male, presenting with deterioration of motor function in his right limbs since 18 months, with computed tomography (CT) scans and magnetic resonance imaging (MRI) documenting a large subdural collection of the left hemisphere, with calcified inner membrane, which was successfully and completely removed, with progressive clinical and radiological improvement. Conclusions: We report a case where this type of rare lesion was removed with a progressive and complete resolution of the patient’s symptoms, restoring his previous neurological condition. From the cases described in the literature and our own experience with this case, we think surgical treatment in these patients, when symptomatic, is necessary and viable, frequently resulting in the patient’s improvement.

Variant Rett Syndrome in a Girl with a Pericentric X-Chromosome Inversion Leading to Epigenetic Changes and Overexpression of the MECP2 Gene

Rett syndrome is a neurodevelopmental disorder caused by mutations in the MECP2 gene. We investigated the genetic basis of disease in a female patient with a Rett-like clinical. Karyotype analysis revealed a pericentric inversion in the X chromosome -46,X,inv(X)(p22.1q28), with breakpoints in the cytobands where the MECP2 and CDKL5 genes are located. FISH analysis revealed that the MECP2 gene is not dislocated by the inversion. However, and in spite of a balanced pattern of X inactivation, this patient displayed hypomethylation and an overexpression of the MECP2 gene at the mRNA level in the lymphocytes (mean fold change: 2.55±0.38) in comparison to a group of control individuals; the expression of the CDKL5 gene was similar to that of controls (mean fold change: 0.98±0.10). No gains or losses were detected in the breakpoint regions encompassing known or suspected transcription regulatory elements. We propose that the de-regulation of MECP2 expression in this patient may be due to alterations in long-range genomic interactions caused by the inversion and hypothesize that this type of epigenetic de-regulation of the MECP2 may be present in other RTT-like patients.

Congenital Complete Atrioventricular Block. Case Report and Review of the Literature

Introduction: Congenital complete atrioventricular block (AVB) without cardiac malformation is a rare and potentially fatal condition. In most cases it is associated with maternal systemic lupus erythematosus through transplacental passage of antibodies anti-SSA/Ro and/or anti-SSB/La. Antenatal fluorinated-steroids have been successful in reversing first and second degree congenital AVB but inconsistent in third degree block. Case Report:The authors report a case of fetal bradycardia diagnosed at 24 weeks of gestation. The fetal echocardiogram revealed a second/third degree AVB without structural heart disease. Maternal anti-SSA/Ro antibodies were detected. There was no blockage improvement with maternal oral fluorinated-steroids. An elective cesarean section was performed at term with the delivery of a healthy girl that required an epicardical pacemaker on the 8th day of life. Conclusion: In this case, treatment with maternal fluorinated corticosteroids was not effective in preventing progression of the heart block.