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IntroductionThe influence of various levels of sedation depth on motor evoked potentials (MEP) reproducibility in youth is still unclear because of a lack of data. We tested the hypothesis that a deeper level of total intravenous anesthesia (TIVA) [bispectral index (BIS) 40 ± 5 compared to 60 ± 5] can affect surgeon-directed MEP and their interpretation in youths.MethodsAll patients received TIVA combined with propofol and remifentanil. TIVA was initially maintained at a BIS level of 60 ± 5. The sedation anesthesia was deepened to BIS level 40 ± 5 before the skin incision. MEP were recorded and interpreted at both BIS levels. The primary endpoint was to evaluate the effect of the depth of sedation on the MEP reproducibility directed and interpreted by the surgical team in each patient separately. The secondary endpoint was to compare the relativized MEP parameters (amplitude and latency) in percentage at various levels of sedation in each patient separately. We planned to enroll 150 patients. Due to the COVID-19 pandemic, we decided to analyze the results of the first 50 patients.ResultsThe surgical team successfully recorded and interpreted MEP in all 50 enrolled patients in both levels of sedation depth without any clinical doubts. The MEP parameters at BIS level 40 ± 5, proportionally compared with the baseline, were latency 104% (97–110%) and the MEP amplitudes 84.5% (51–109%).ConclusionPreliminary data predict that deeper sedation (BIS 40 ± 5) does not affect the surgical team’s interpretation of MEP in youth patients. These results support that surgeon-directed MEP may be an alternative when neurophysiologists are unavailable.

Cerebral tumors and multiple sclerosis (MS) can show overlapping clinical and magnetic resonance imaging (MRI) features and even occur concurrently. Due to the emergence of new symptoms, not usually MS related, an MRI was conducted in a 29-year-old woman with relapsing-remitting MS and showed a significant size progression of a parieto-occipital lesion, with mild clinical correlates, such as blurred vision, difficulty in speaking, and headache. Contrast-enhanced MRI and fluorothymidine positron-emission tomography (PET) did not point toward neoplasm, a lesion biopsy, however, showed astrocytoma, which was confirmed as grade III astrocytoma after the radical resection of the tumor. In the case of an atypical lesion, a tumor should be considered in patients with MS. A small fraction of high-grade gliomas show no enhancement on MRI and no hypermetabolism on PET. Biopsy proved to be the essential step in a successful diagnostic workup. To the best of our knowledge, this is the first case of anaplastic astrocytoma with these radiological features reported in a patient with MS.

ImportanceMultiple sclerosis can also affect children. Approximately 3–10% of patients develop multiple sclerosis before the age of 16.ObjectiveThe aim of this analysis is to describe the characteristics of pediatric patients with multiple sclerosis who started their treatment with disease-modifying drugs in 2013–2020, with data obtained from the Czech National Registry of patients with multiple sclerosis.Design and SettingA method of retrospective analysis conducted with 134 pediatric patients with multiple sclerosis was used.ResultsThe findings reveal that the mean age at the date of the introduction of the first disease-modifying drugs treatment is 15.89 years, and gender does not play any role. In addition, moderate (51.6%) and mild (45.2%) relapses are predominant in these young patients. Seventy five percent of patients will not experience a confirmed progression of the expanded disability status scale within 54.7 months from starting the treatment. Furthermore, the results confirm that the first-choice treatment is interferon beta-a and glatiramer acetate, which is common for adult patients. However, some factors, such as a low efficacy or a lack of tolerance may impact on treatment discontinuation in children.ConclusionMore research should be performed on novel disease-modifying drugs for this target group.

Tumefactive demyelinating lesions belong to the rare variants of multiple sclerosis, posing a diagnostic challenge since it is difficult to distinguish them from a neoplasm or other brain lesions and they require a careful differential diagnosis. This contribution presents the case report of a young female with progressive tumefactive demyelinating brain and spinal cord lesions. An extensive diagnostic process including two brain biopsies and an autopsy did not reveal any explanatory diagnosis other than multiple sclerosis. The patient was treated by various disease-modifying treatments without significant effect and died from ascendent infection via ventriculoperitoneal shunt resulting in Staphylococcus aureus meningitis.

Background: Toxic epidermal necrolysis (TEN) is a rare life-threatening disease that mainly affects the skin and mucous membranes, resulting from a toxic delayed-type hypersensitivity (DTH) reaction (type IV reaction) to the presence of foreign antigens such as drugs. The clinical symptoms are caused by pathophysiological processes leading to massive apoptosis of keratinocytes in the dermo-epidermal junction. This results in the formation of a bulla and subsequent separation of the entire epidermis with the exposure of the dermis. The current approach in the local therapy of TEN prefers the use of biological dressings, which helps provide several critical requirements for defect healing; in particular, it helps in the acceleration of the spontaneous wound closure (re-epithelialization) of the skin defect and the reduction of the risk of development of various complications and infections, such as the risk of pathological scar maturation. This paper is a case report of the use of a lyophilized amniotic membrane (AM) for accelerating wound healing in a patient with TEN.Case Presentation: We report a case of an 8-year-old girl transferred to our center with a histologically confirmed diagnosis of TEN. Despite the application of immunosuppressive therapy consisting of corticosteroids and intravenous immunoglobulins, we have observed disease progression and exfoliation of up to 60% of the total body surface area (TBSA). In the facial area, which is cosmetically privileged, we decided to use the lyophilized amniotic membrane (Amnioderm®) to cover up approximately 2% of the TBSA. Within 2 days after the application, we observed accelerated reepithelialisation, with rapid wound closure. We have not observed any side effects nor infections during the subsequent phases of wound healing. Skin defects in non-facial areas of the body were treated with synthetic dressings. When compared to the areas covered with the lyophilized AM, the healing process was prolonged.Conclusions: To our knowledge, this is the first case study using a lyophilized amniotic membrane in the treatment of a patient with TEN. The AM application in the cosmetically-privileged area (face), proved to be very efficient in the treatment of TEN patients. The use of this allogeneic material demonstrated excellent biocompatibility and caused a unique acceleration of epithelialization and wound healing, yielding also excellent long-term results. The current study opens broad possibilities for clinical application of the used material, the improvement of current therapy of patients with TEN and better outcomes and recovery of patients.

Background: Multiple sclerosis (MS) is a chronic inflammatory and neurodegenerative disease of the central nervous system. Well-established drugs used for MS patients after the first demyelinating event in the Czech Republic include glatiramer acetate (GA), interferon beta-1a (IFNβ-1a), IFN beta-1b (IFNβ-1b), peginterferon beta-1a (peg-IFNβ-1a), and teriflunomide.Objective: The objective of this observational study was to compare the effectiveness of the abovementioned drugs in patients with MS who initiated their therapy after the first demyelinating event. Patients were followed for up to 2 years in real clinical practice in the Czech Republic.Methods: A total of 1,654 MS patients treated after the first demyelinating event and followed up for 2 years were enrolled. Evaluation parameters (endpoints) included the annualized relapse rate (ARR), time to next relapse, change in the Expanded Disability Status Scale (EDSS) score, and time of confirmed disease progression (CDP). When patients ended the therapy before the observational period, the reason for ending the therapy among different treatments was compared.Results: No significant difference was found among the groups of patients treated with IFNβ-1a/1b, GA, or teriflunomide for the following parameters: time to the first relapse, change in the EDSS score, and the proportion of patients with CDP. Compared to IFNβ-1a (44 mcg), a significant increase in the percentage of relapse-free patients was found for GA, but this treatment effect was not confirmed by the validation analysis. Compared to the other drugs, there was a significant difference in the reasons for terminating GA therapy.Conclusion: Small differences were found among GA, IFNβ and teriflunomide therapies, with no significant impact on the final outcome after 2 years. Therefore, in clinical practice, we recommend choosing the drug based on individual potential risk from long-term therapy and on patient preferences and clinical characteristics.