Degeneration of the Cerebellum in Huntington's Disease (HD): Possible Relevance for the Clinical Picture and Potential Gateway to Pathological Mechanisms of the Disease Process

Huntington's disease (HD) is a polyglutamine disease and characterized neuropathologically by degeneration of the striatum and select layers of the neo‐ and allocortex. In the present study, we performed a systematic investigation of the cerebellum in eight clinically diagnosed and genetically confirmed HD patients. The cerebellum of all HD patients showed a considerable atrophy, as well as a consistent loss of Purkinje cells and nerve cells of the fastigial, globose, emboliform and dentate nuclei. This pathology was obvious already in HD brains assigned Vonsattel grade 2 striatal atrophy and did not correlate with the extent and distribution of striatal atrophy. Therefore, our findings suggest (i) that the cerebellum degenerates early during HD and independently from the striatal atrophy and (ii) that the onset of the pathological process of HD is multifocal. Degeneration of the cerebellum might contribute significantly to poorly understood symptoms occurring in HD such as impaired rapid alternating movements and fine motor skills, dysarthria, ataxia and postural instability, gait and stance imbalance, broad‐based gait and stance, while the morphological alterations (ie ballooned neurons, torpedo‐like axonal inclusions) observed in the majority of surviving nerve cells may represent a gateway to the unknown mechanisms of the pathological process of HD.     

Cavity length control for Fourier domain mode locked (FDML) lasers with µm precision

In highly dispersion compensated Fourier domain mode locked (FDML) lasers, an ultra-low noise operation can only be achieved by extremely precise and stable matching of the filter tuning period and light circulation time in the cavity. We present a robust and high precision closed-loop control algorithm and an actively cavity length controlled FDML laser. The cavity length control achieves a stability of ∼0.18 mHz at a sweep repetition rate of ∼418 kHz which corresponds to a ratio of 4×10⁻¹⁰. Furthermore, we prove that the rapid change of the cavity length has no negative impact on the quality of optical coherence tomography using the FDML laser as light source.     

Convalescent plasma therapy in patients with COVID-19

IntroductionPassive antibody therapy has been used to immunize vulnerable people against infectious agents. In this study, we aim to investigate the efficacy of convalescent plasma (CP) in the treatment of severe and critically ill patients diagnosed with COVID-19.MethodThe data of severe or critically ill COVID-19 patients who received anti-SARS-CoV-2 antibody-containing CP along with the antiviral treatment (n = 888) and an age-gender, comorbidity, and other COVID-19 treatments matched severe or critically ill COVID-19 patients at 1:1 ratio (n = 888) were analyzed retrospectively.ResultsDuration in the intensive care unit (ICU), the rate of mechanical ventilation (MV) support and vasopressor support were lower in CP group compared with the control group (p = 0.001, p = 0.02, p = 0.001, respectively). The case fatality rate (CFR) was 24.7 % in the CP group, and it was 27.7 % in the control group. Administration of CP 20 days after the COVID-19 diagnosis or COVID-19 related symptoms were associated with a higher rate of MV support compared with the first 3 interval groups (≤5 days, 6?10 days, 11?15 days) (p=0.001).ConclusionCP therapy seems to be effective for a better course of COVID-19 in severe and critically ill patients.     

Pulmonary arteriovenous malformation in children

Although pulmonary arteriovenous malformations (PAVM) are relatively rare in children, they are important in the differential diagnosis of common pulmonary problems, such as hypoxemia, hemoptysis and dyspnea on exertion. We report the cases of two PAVM patients with different presentations and describe the treatment strategies.     

Post-partum sacral fracture associated with heparin treatment

We report the case of a 19-year-old woman who consulted for low-back pain 3 weeks after her first delivery. This young woman had a personal history of protein C deficiency and was treated daily during her pregnancy with low-molecular-weight heparin. Her body mass index was 34 and she only gained 10 kg during her pregnancy. Since the delivery - which occurred without any complication - she had suffered from a gradually increasing right-buttock pain and limp. Magnetic resonance imaging (MRI) revealed a fracture of the right sacral ala. After analgesia and 1 month of home relative bed rest, the patient recovered her functional capacities. Regarding our patient, who had no potential clinical risk factors for osteoporosis, the causal effect of heparin is thus possible but not certain. This case report illustrates the fact that clinicians should have a high suspicion of pelvic fracture in post-partum women, even in very young ones, presenting sudden onset of low back and pelvic pain, especially when they have received heparin during pregnancy. MRI seems to be the key exam because it is able to detect and stage fractures or microfractures.     

Application of transcatheter valves for aortic valve replacement in pediatric patients: A case series

• Diabetic patients are frequently affected by coronary artery disease (CAD) and are at increased risk of CAD‐related adverse events, even after drug‐eluting stent (DES) implantation. If currently available DES have similar safety and efficacy in diabetic and nondiabetic patients is still debated.
• This prospective, multicenter registry showed similar 3‐year outcome in patients undergoing different DES implantation, although diabetic patients, especially those requiring insulin treatment, had significantly higher risk of adverse events than nondiabetic patients.
• Specific efforts to improve the performance of DES in diabetic patients are mandatory to adequately address the unsolved issue of diabetic patients affected by CAD.