Neurological research and practice最新文献

Background: Status epilepticus (SE) is one of the most common neurological emergencies and an acutely life-threatening condition characterized by high mortality and morbidity. Despite the well-established staged therapy of status epilepticus, especially stages 1 and 2, more than one third of patients develop (super-) refractory SE. Despite a large variety of potential treatment options for super-refractory SE, there is an unmet clinical need of potential new treatment ideas in this often desperate clinical situation. A number of studies have demonstrated the safety and efficacy of stiripentol (STP) in patients with Dravet syndrome (DS) and in children with focal epilepsy and generalized epilepsies. Some smaller series and case reports have documented the use of STP in the treatment of status epilepticus in adult patients.

Methods: We retrospectively analyzed all patients who were admitted to the Department of Neurology at Marburg University Hospital between 2013 and 2023 with a diagnosis of (super)-refractory status epilepticus and who received additional treatment of SE with STP. All patients who received STP during the SE were included, regardless of previous medication.

Results: SE ceased in 64% of 25 patients (13 female and 12 male). The mean age was 58.6 ± 21.9 years (mean ± SD). 72% had a structural epilepsy. In 20% of patients, SE was terminated by the administration of STP alone in 32% of cases, while in a further 32% of patients, the simultaneous administration of multiple anti-seizure medications (ASMs) including STP was potentially responsible for the cessation of the SE, with valproic acid (VPA), benzodiazepines and STP, being the most frequently implicated ASMs. In 12% of patients, there was at least a temporary improvement in the electroencephalogram (EEG). Stiripentol had to be discontinued in three cases due to a reduction in vigilance or hypercalcemia.

Conclusions: Stiripentol may represent a promising additional treatment option for refractory and super-refractory status epilepticus. The tolerability of this treatment has already been demonstrated in previous studies, and was also reflected in these data. Further prospective investigation in larger patient populations are necessary to ascertain the efficacy of stiripentol in SE.

Trial registration: NCT06540378, retrospectively registered.

Background/ aim: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting upper and lower motor neurons, causing progressive atrophy of muscles, hypertonia, and paralysis. This study aimed to evaluate the current evidence and effectiveness of ultrasound in investigating nerve cross-sectional area (CSA) of peripheral nerves, vagus and cervical roots in those with ALS compared with healthy controls and to pool the CSA measurements.

Methods: A systematic search was conducted on Cochrane, Clarivate Web of Science, PubMed, Scopus, and Embase for the mesh terms nerve, ultrasonography, and amyotrophic lateral sclerosis. A quality assessment was performed using the New-Ottawa scale. In addition, a double-arm meta-analysis using Review Manager 5 software version 5.4 was performed.

Results: From the seventeen studies included in this review, the overall mean difference showed that individuals with ALS had a significantly smaller CSA in comparison to healthy controls for median, ulnar, C6 root, and phrenic nerves. However, no significant difference in the CSA was found in radial, vagal, sural, and tibial nerves.

Discussion: This study confirmed results of some of the included studies regards the anatomic sites, where nerve atrophy in ALS could be detected to potentially support the diagnosis of ALS. However, we recommend further large, prospective studies to assess the diagnostic value of these anatomical sites for the diagnosis of ALS.

Conclusions: Our findings confirmed specific anatomic sites to differentiate ALS patients from healthy controls through ultrasound. However, these findings cannot be used to confirm the ALS diagnosis, but rather assist in differentiating it from other diagnoses.

Trial registration: Retrospectively registered on July 30th 2024 in PROSPERO (PROSPERO (york.ac.uk)) with ID574702.

Background: Nurse-initiated supported implementation of protocols to manage fever, hyperglycaemia (sugar) and swallowing (FeSS) following acute stroke reduced 90-day death and disability in the landmark Australian Quality in Acute Stroke Care (QASC)-Trial. An international interprofessional collaboration sought to evaluate the effects of nurse-led FeSS implementation on FeSS Protocol adherence in German stroke units.

Methods: This pre-test/post-test study was conducted in eight German stroke units between 2020 and 2022. Stroke nurses as clinical champions, supported by the project team, conducted multidisciplinary workshops discussing pre-implementation medical record audit results, barriers and facilitators to FeSS Protocol implementation, developed action plans and provided education, with ongoing support from Australia. Medical record audit data were collected by nurses, pre-implementation and three months post-implementation.

Results: In 771 (pre-implementation) and 679 (post-implementation) patients there were improvements in overall FeSS adherence (pre 20%, post 28%; adjusted difference in proportions (95% CI) 11%, (5.1%, 16%); p < 0.001), adherence to hyperglycaemia (pre 43%, post 55%; adjusted difference 23%, (17%, 29%); p < 0.001) and swallowing (pre 52%, post 61%; adjusted difference 11%, (5.2%, 17%); p < 0.001) but not fever protocol (pre 76%, post 78%; adjusted difference 1.5%, (-2.6%, 5.7%); p = 0.474). Improvements also were noted in administration of anti-pyretics (pre 29%, post 59%; adjusted difference 32%, (20%, 44%); p < 0.001); and insulin (pre 41%, post 60%; adjusted difference 14%, (1.1%, 28%); p < 0.034) both within one hour, as well as in performing a swallow screen within 24 h of admission (pre 65%, post 74%; adjusted difference 18% (8.8%, 26%); p < 0.001).

Conclusions: Supported implementation of the FeSS Protocols significantly improved acute care for post stroke complications of fever, hyperglycaemia and dysphagia in terms of higher adherence and shorter time to treatment.

Trial registration: As this is a pre-test/post-test study and does not meet the WHO/ICMJE definition of a clinical trial, registration was not required.

Background: Optimal blood pressure management during endovascular stroke treatment is not certain. We hypothesized that time or proportion of intraprocedural systolic blood pressure spent in a range around admission blood pressure might be associated with better clinical outcome.

Methods: We conducted a retrospective observational study at a single center at a university hospital, which included patients from August 2018 to September 2020 suffering from acute ischemic stroke with anterior circulation vessel occlusion and treated with endovascular therapy. Time and proportion of procedure time where systolic blood pressure (SBP) was near the baseline SBP on admission (bSBP) were used as exposure variables. The primary outcome was the occurrence of mRS score 0-2 three months after stroke. The primary analysis was performed by fitting a logistic regression model adjusted for baseline NIHSS, pre-stroke mRS, mTICI score, intubation, age and sex.

Results: We included 589 patients in the analysis. Mean (SD) age was 76 (12) years, 315 were women (53%) and mean (SD) NIHSS score at admission was 15 (7.5). Mean (SD) bSBP was 167 (28) mmHg and mean (SD) intraprocedural SBP was 147 (21) mmHg. The proportion of time where intraprocedural SBP was in range of bSBP ± 20% was associated with a slightly higher odds of achieving favorable outcome (adjusted OR, 1.007; 95% CI, 1.0003-1.013).

Conclusion: A higher proportion of intraprocedural time with systolic blood pressure in range of ± 20% of the admission level is associated with higher odds of favorable functional outcome.

Trial registration: Not applicable.

Guillain-Barré syndrome is the most common acute inflammatory demyelinating peripheral nerve condition. Occasionally, other autoimmune conditions can mimic Guillain-Barré syndrome but may require different diagnostic workup and treatment. We report here two patients with Evans syndrome, a rare hematological autoimmune condition who developed a subacute inflammatory radiculopathy. Similarities and distinguishing clinical and diagnostic features are discussed.

As a chronic inflammatory disease of the central nervous system, multiple sclerosis (MS) is of great individual health and socio-economic significance. To date, there is no prognostic model that is used in routine clinical care to predict the very heterogeneous course of the disease. Despite several research groups working on different prognostic models using traditional statistics, machine learning and/or artificial intelligence approaches, the use of published models in clinical decision making is limited because of poor model performance, lack of transferability and/or lack of validated models. To provide a systematic overview, we conducted a "Cochrane review" that assessed 75 published prediction models using relevant checklists (CHARMS, PROBAST, TRIPOD). We have summarized the relevant points from this analysis here so that the use of prognostic models for therapy decisions in clinical routine can be successful in the future.

Immunoglobulin G4 (IgG4) related hypertrophic pachymeningitis of the spinal cord is a rare condition, characterized by infiltration of the spinal meninges with IgG4-producing plasma cells and subsequent hypertrophic fibrosis. Here, we report on a 65-year-old woman with IgG4 associated hypertrophic spinal pachymeningitis, in whom cerebrospinal fluid (CSF) analysis was a decisive diagnostic tool. Not only could we demonstrate an intrathecal IgG4 production, but also IgG4 positive plasma cells in CSF. Following decompressive surgery, diagnosis of IgG4 associated hypertrophic pachymeningitis was confirmed histologically. Surgery and immunosuppressive therapy with rituximab were associated with clinical improvement. This case highlights CSF analyses as diagnostic tool for detection of IgG4 related hypertrophic pachymeningitis.

Objective: Robotic arms are innovative assistive devices for ALS patients with progressive motor deficits of arms and hands. The objective was to explore the patients´ expectations towards a robotic arm system and to assess the actual experiences after the provision of the device.

Methods: A prospective observational study was conducted at 9 ALS centers in Germany. ALS-related functional deficits were assessed using the ALS-Functional Rating Scale-revised (ALSFRS-R). Motor deficit of the upper limbs was determined using a subscore of three arm-related items of the ALSFRS-R (items 4-6; range 0-12 points). User expectations before provision (expectation group, n = 85) and user experiences after provision (experience group, n = 14) with the device (JACO Assistive Robotic Device, Kinova, Boisbriand, QC, Canada) were assessed.

Results: In the total cohort, mean ALSFRS-R subscore for arm function was 1.7 (SD: 2.0, 0-9) demonstrating a severe functional deficit of the upper limbs. In the expectation group (n = 85), the following use cases of the robotic arm have been prioritized: handling objects (89%), close-body movements (88%), pressing buttons (87%), serving drinks (86%), and opening cabinets and doors (85%). In the experience group (n = 14), handling objects (79%), serving drinks (79%), near-body movements (71%), pushing buttons (71%), serving food (64%), and opening doors (64%) were the most frequent used cases. Most patients used the device daily (71.4%, n = 10), and 28.6% (n = 4) several times a week. All patients of the experience group found the device helpful, felt safe while using the device, and were satisfied with its reliability. NPS of the assistive robotic arm revealed 64% "promoters" (strong recommendation), 29% "indifferents" (uncertain recommendation) and 7% "detractors" (no recommendation). Total NPS was + 57 demonstrating strong patient satisfaction.

Conclusions: Initiation of procurement with a robotic assistive arm was confined to patients with severe functional deficit of the upper limbs. User experience underlined the wide spectrum of use cases of assistive robotic arms in ALS. The positive user experience together with high satisfaction underscore that robotic arm systems serve as a valuable treatment option in ALS patients with severe motor deficits of the arms.

Background: Individuals with Parkinson's disease (PD) report a diminished perceived functional autonomy as their condition progresses. For those seeking emergency care, it is unknown whether the patient-physician relationship is instrumental in respecting patient autonomy. This study evaluated patient autonomy ideals in individuals with PD requiring emergency care and the perceived support for autonomy from emergency department physicians.

Method: Individuals with PD (n = 36, average age 78.1 years) were surveyed using the Ideal Patient Autonomy questionnaire (IPA) and the Health Care Climate Questionnaire (HCCQ). A multivariable regression analysis assessed whether patients' Hoehn and Yahr stage and IPA questionnaire results predicted HCCQ items.

Results: The IPA questionnaire revealed that individuals with PD in need of emergency care emphasize the significance of medical expertise (IPA 'doctor should decide' theme 0.71) in decision-making and their desire to be fully informed about all potential risks (IPA 'obligatory risk information' theme 0.71). The average HCCQ values showed a decreasing trend across Hoehn and Yahr stages 1 to 5: 6.19, 6.03, 5.83, 5.80, and 5.23, respectively. HY scale values also influenced HCCQ items related to the physician's role.

Conclusion: In our cohort, individuals with Parkinson's disease tend to rely on medical expertise for decision-making and prioritize complete risk information during emergency care. However, this autonomy support diminishes as functional disability levels increase.

Background: Atrophy of white and grey matter volumes occurs early in the brains of people with multiple sclerosis (pwMS) and has great clinical relevance. In clinical trials, brain atrophy can be quantified by magnetic resonance imaging (MRI) with automated software tools.

Methods: In this study, we analyze volumes of various brain regions with the software "md brain" based on routine MRI scans of 53 pwMS in a real-world setting. We compare brain volumes of pwMS with an EDSS ≥ 3.5 and a disease duration ≥ 10 years to the brain volumes of pwMS with an EDSS < 3.5 and a disease duration < 10 years as well as with or without immunotherapy.

Results: pwMS with an EDSS ≥ 3.5 and a disease duration ≥ 10 years had significantly lower volumes of the total brain, the grey matter and of the frontal, temporal, parietal and occipital lobe regions as compared to pwMS with an EDSS < 3.5 and a disease duration < 10 years. Regional brain volumes were significantly lower in pwMS without immunotherapy.

Conclusions: The study showed that higher EDSS, longer disease duration and absence of immunotherapy was associated with lower volumes in a number of brain regions. Further real-world studies may include larger patient cohorts in longitudinal analyses.