European Journal of Neurology最新文献

{"title":"Disease Trajectories of a Large French Cohort of 142 Congenital Myopathy Patients in Adult Age","authors":"Michela Bisciglia,&nbsp;Gianmarco Severa,&nbsp;Norma Beatriz Romero,&nbsp;Michel Fardeau,&nbsp;John Rendu,&nbsp;Tanya Stojkovic,&nbsp;Pascal Laforêt,&nbsp;Bruno Eymard,&nbsp;Ana Ferreiro,&nbsp;Edoardo Malfatti,&nbsp;Anthony Béhin","doi":"10.1111/ene.70109","DOIUrl":"https://doi.org/10.1111/ene.70109","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Congenital myopathies (CMyo) are a group of rare inherited muscle disorders classified to date according to myopathological features on muscle biopsy. They usually present with an early onset, with a slow or non-progressive muscle weakness. The phenotypic spectrum is wide, ranging from severe early onset forms to milder and later onset conditions. Data regarding the disease trajectory of CMyo in adult patients are lacking. Here, we describe the clinical, myopathological, and genetic features of a large cohort of adult CMyo patients to facilitate their management in adulthood.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Global data of a cohort of 142 myopathologically and genetically defined adult patients, 76 women and 66 men, followed at Institute of Myology of the Pitié-Salpêtrière Hospital, were retrospectively analyzed focusing on muscular phenotype, cardiac, and respiratory assessment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>RYR1<i>-</i>related CMyo was the most represented entity (<i>N</i> = 65, 45%), followed by DNM2-related CMyo (<i>N</i> = 26, 18%). Eighty-two percent of patients presented with a prenatal, infancy or childhood onset, including delayed motor milestones. An adult onset, defined as &gt; 18 years (median age 43 years), was identified in 15% of patients (<i>N</i> = 18). Fifteen percent of patients were wheelchair-bound. The poorest respiratory outcome was found in SELENON-related CMyo patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This observational study provides long-term data on disease progression in CMyo. Adult CMyo patients generally presented mild motor disability at follow-up. Nevertheless, a subset of patients experienced loss of gait and severe respiratory failure. CMyo should be considered in the differential diagnosis of adult-onset myopathies due to the rare but possible late-onset forms.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70109","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143741032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Insights Into Disability and Disability Progression in People With Multiple Sclerosis Using Large-Scale Healthcare Data","authors":"Onur Dereli,&nbsp;Jochen Behringer,&nbsp;Achim Berthele,&nbsp;Alexander Hapfelmeier,&nbsp;Bernhard Hemmer,&nbsp;Christiane Gasperi","doi":"10.1111/ene.70124","DOIUrl":"https://doi.org/10.1111/ene.70124","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Identifying predictors for disability progression is crucial for managing multiple sclerosis (MS). This study aims to explore levels of disability and informative factors for disability progression in people with MS (PwMS) using healthcare data without detailed clinical information.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted a case–control/cohort study on data from Bavaria's largest health insurance organization. The dataset included records of assistive devices, nursing care, sick leaves, rehabilitation, drug therapies, and diagnoses for individuals with MS, Crohn's disease (CD), rheumatoid arthritis (RA), and controls (CTR) without these diseases. We used generalized linear models to compare healthcare service utilization between MS and other cohorts. A gradient-boosting algorithm identified informative healthcare-related factors associated with disability progression in PwMS, defined by increased nursing care utilization.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>PwMS (<i>N</i> = 11,961) demonstrated higher healthcare utilization than CD (<i>N</i> = 21,884), RA (<i>N</i> = 105,450), and CTR (<i>N</i> = 82,677) groups, even at young ages. Besides expected risk factors like age, smoking, diabetes, and psychiatric disorders, the prediction algorithm revealed that PwMS with specific gynecological disorders, upper tract infections, asthma, and thyroiditis were less likely to need higher levels of nursing care.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Leveraging healthcare data allows for an objective assessment of disability in PwMS and can identify informative factors for disability progression. Our approach can be applied to studies on disease progression in large cohorts without detailed clinical data and can be adapted to other diseases, disability measures, and healthcare systems. Higher utilization of healthcare resources even at young ages revealed an unmet need for improved treatment and management strategies for young adults with MS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70124","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143741030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Natural Progression of Pineal Cysts in Relation to Headache: A Prospective Observational Study","authors":"Victor Hvingelby,&nbsp;Eirini Tsigka,&nbsp;Rigmor Hoejland Jensen,&nbsp;Sofia Hylin,&nbsp;Tiit Mathiesen","doi":"10.1111/ene.70120","DOIUrl":"https://doi.org/10.1111/ene.70120","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Pineal cysts are a frequent, potentially incidental finding in routine imaging studies. Their relationship with headache has been a matter of some controversy. In this prospective observational study, we sought to track the clinical and radiological trajectory of pineal cysts in both patients with and without concurrent headache.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A cohort study was conducted in patients referred to the Department of Neurosurgery at Karolinska Hospital for evaluation of a pineal cyst ≥ 5 mm between June 2005 and April 2017. Patients were stratified according to headache status and classified according to standard criteria. Development in cyst size and degree of aqueduct stenosis, as well as clinical progression of headache, was tracked.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>157 cysts were identified (50 males and 107 females, mean age: 37 years), 103 of which were in patients primarily assessed for headaches. Cyst size remained stable over the follow-up period. There was no relationship between cyst size, the presence or progression of headache, nor with amenability to treatment. No relationship between the mass effect of the quadrigeminal plate and headache was observed. Of 13 patients treated with melatonin, 7 (53%) reported a subsequent decrease in headache severity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>A major proportion of seemingly incidental pineal cysts referred for neurosurgical evaluation were detected during work-up for headache. Furthermore, the size and symptoms associated with cysts remain stable over time, barring intervention. The therapeutic implications and relationship with other factors such as melatonin remain to be explored.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70120","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143690203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aetiology of Acute Respiratory Insufficiency in Patients With Ischaemic Stroke Studied by Chest CT Scan","authors":"Omid Shirvani,&nbsp;Patricia Fischbein,&nbsp;Zeynep Bendella,&nbsp;Piergiorgio Profico,&nbsp;Franziska Dorn,&nbsp;Gabor C. Petzold,&nbsp;Sebastian Stösser","doi":"10.1111/ene.70125","DOIUrl":"https://doi.org/10.1111/ene.70125","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Acute respiratory insufficiency (ARI) is considered a serious life-threatening complication after ischaemic stroke. The aim of this study was to identify the most common aetiologies of ARI after stroke and their association with patients' outcome.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This retrospective study was conducted at the University Hospital Bonn, involving patients with acute ischaemic stroke who underwent chest CT scans for ARI between 2017 and 2022. We collected clinical and demographic data, laboratory parameters, vital signs, as well as outcome parameters. CT scans were reviewed by a radiologist. The dataset was analysed to identify the most frequent aetiologies and their associations to outcome parameters.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We included 236 patients with a median age of 75 years and a median NIHSS score of 11. In-hospital mortality accounted for 30.5%. The most frequent pulmonary conditions on CT, in order of prevalence, included bronchitis/bronchiolitis (66.1%), atelectasis (66.1%), pleural effusion (60.6%), pneumonia (53%), pulmonary oedema (37.3%), and pulmonary artery embolism (27.5%). Bronchitis/bronchiolitis was an independent risk factor for mortality (OR = 3.17, 95% CI: 1.11–8.79, <i>p</i> = 0.03). A higher number of pulmonary conditions decreased the likelihood of discharge to home, and non-survivors had worse vital/laboratory parameters.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>We identified six key pulmonary aetiologies of ARI after ischaemic stroke, with bronchitis/bronchiolitis notably linked to in-hospital mortality in our study cohort. An increased number of these acute pulmonary conditions decreased the likelihood of discharge to home. Early chest CT/CT-angiography may help to identify patients at high risk for in-hospital mortality and to initiate appropriate treatment early.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70125","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143690202","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Preventing Cardiomyopathy in Duchenne Muscular Dystrophy: Long-Term Follow-Up of Patients in the Randomised, Placebo-Controlled Drug-Trial of Perindopril and Bisoprolol","authors":"John P. Bourke,&nbsp;Andrew Bryant,&nbsp;Gregory Landon,&nbsp;Alexis Burn,&nbsp;Stefan Spinty,&nbsp;Ros Quinlivan,&nbsp;Zoya Alhaswani,&nbsp;Thomas Chadwick,&nbsp;Francesco Muntoni,&nbsp;Michela Guglieri,&nbsp;DMD Heart Study Group","doi":"10.1111/ene.70097","DOIUrl":"https://doi.org/10.1111/ene.70097","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>It is uncertain whether using cardiac drugs prophylactically in combinations for DMD is better than ACE-inhibitor alone. Our previous study showed no differences in left ventricular function between perindopril-bisoprolol and matched placebo after 36 months.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This study aimed to determine whether heart measures diverged after 60-month total follow-up. All participants had commenced open-label perindopril and bisoprolol when the original study ended. All were reconsented for access to heart measures, undertaken as part of their clinical care. The primary outcome was the change in echo-measured ventricular ejection fraction from baseline according to original randomization.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 75 participants reported originally, 65 (aged 16 ± 2.5 years) were re-recruited and had data for analysis. Adjusted primary outcomes included 44 participants (original arms: ‘active’ 21; ‘placebo’ 23), 48 for secondary outcomes, and 65 for ‘headcount’ analysis of those with ventricular dysfunction. Absolute LVEF% values reduced in both groups (‘active’: 62.5% ± 5.6% to 53.8% ± 4.0%; ‘placebo’: 60.6% ± 4.9% to 50.4% ± 8.5%). Despite trends favoring earlier introduction of therapy, change from baseline was similar between groups (adjusted mean difference: -7.7 (95% CI -16.4 to1.0%)). However, more in the ‘placebo’ arm had died, had reduced LVEF%, and were taking additional heart medications.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>While some patients may have benefited from ‘early’ (active) as opposed to ‘delayed’ (placebo) initiation of perindopril and bisoprolol, group-mean ventricular function did not differ between study arms after 60 months. Small numbers, absence of a control group, insensitivity of echo-ejection fraction, and additional drug use probably prevented divergence between groups.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70097","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143690204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Peripheral Nerve Involvement in Friedreich's Ataxia Characterized by Quantitative Magnetic Resonance Neurography","authors":"Heike Jacobi,&nbsp;Markus Weiler,&nbsp;Georges Sam,&nbsp;Sabine Heiland,&nbsp;John M. Hayes,&nbsp;Martin Bendszus,&nbsp;Wolfgang Wick,&nbsp;Jennifer C. Hayes","doi":"10.1111/ene.70121","DOIUrl":"https://doi.org/10.1111/ene.70121","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Friedreich's ataxia (FRDA) affects both the central and peripheral nervous system. Peripheral nerve involvement manifests predominantly as a progressive sensory neuropathy caused by dorsal root ganglionopathy. An additional direct involvement of peripheral nerves leading to abnormal myelination is increasingly discussed. Here, we characterize lower extremity peripheral nerve involvement in FRDA by quantitative magnetic resonance neurography (MRN).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Sixteen genetically confirmed FRDA patients and 16 age-/sex-matched controls were prospectively enrolled. Patients underwent neurologic examinations and nerve conduction studies (NCS). Large-coverage MRN of sciatic and tibial nerves was conducted utilizing dual-echo turbo-spin-echo sequences with spectral fat saturation for T2-relaxometry, and two gradient-echo sequences with and without off-resonance saturation rapid frequency pulses for magnetization transfer contrast imaging. Microstructural and morphometric MRN markers including T2-relaxation time (T2_app), proton spin density (<i>ρ</i>), magnetization transfer ratio (MTR), and cross-sectional area (CSA) were calculated to characterize nerve lesions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Tibial nerve <i>ρ</i> and T2_app were markedly decreased in FRDA at the thigh (<i>ρ</i>: 368.4 ± 11.0 a.u.; T2_app: 59.5 ± 1.8 ms) and lower leg (<i>ρ</i>: 337.3 ± 12.6 a.u.; T2_app: 53.9 ± 1.4 ms) versus controls (thigh, <i>ρ</i>: 458.9 ± 9.5 a.u., <i>p</i> &lt; 0.0001; T2_app: 66.3 ± 0.8 ms, <i>p</i> = 0.0019; lower leg, <i>ρ</i>: 449.9 ± 12.1 a.u., <i>p</i> &lt; 0.0001; T2_app: 62.4 ± 1.2 ms, <i>p</i> &lt; 0.0001) and correlated well with clinical scores, disease duration, and NCS. MTR and CSA did not differentiate between FRDA and controls.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Our study results provide a profound characterization of peripheral nerve involvement in FRDA. The identified good correlation between <i>ρ</i> and T2_app with clinical symptom scores and NCS suggests that parameters of T2 relaxometry may become relevant biomarkers to monitor disease progression and therapeutic responses in potential future clinical trials.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70121","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143690201","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Testing for Acetylcholine Receptor and Muscle-Specific Tyrosine Kinase Antibodies by Fixed Cell-Based Assay in Clinical Practice: Positive Predictive Value for Myasthenia Gravis","authors":"Cathy Meng Fei Li,&nbsp;Michael W. Nicolle,&nbsp;Kamala Sangam,&nbsp;Liju Yang,&nbsp;Adrian Budhram","doi":"10.1111/ene.70123","DOIUrl":"https://doi.org/10.1111/ene.70123","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Fixed cell-based assays (CBAs) to detect acetylcholine receptor and muscle-specific tyrosine kinase antibodies (anti-AChR/MuSK) are now available, but evaluations of their diagnostic performance in clinical practice are lacking. We examined the positive predictive value (PPV) of anti-AChR/MuSK fixed CBA for myasthenia gravis (MG), following the implementation of this assay as first-line testing at our centre.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We identified all patients at our centre with positive anti-AChR/MuSK fixed CBA results between November 2021 and July 2024. Clinical information was reviewed to classify patients as having true positive or false positive antibody results. Patients with a clinical presentation compatible with MG and no more likely alternative diagnosis were classified as true positives, whereas all others were classified as false positives. Test PPV was calculated as the proportion of positives that were classified as true positives.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 770 patients who underwent anti-AChR/MuSK fixed CBA testing, 109 (14%) had positive antibody results (Anti-AChR, 105; Anti-MuSK, 4). Among them, one patient with anti-AChR positivity was classified as a false positive (suspected thymic hyperplasia without neurologic symptoms). The remaining 108 patients were classified as true positives, resulting in a calculated PPV of 99%.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>We found that anti-AChR/MuSK fixed CBA had excellent PPV for MG. Anti-AChR positivity in one asymptomatic patient with suspected thymic hyperplasia was classified as a false positive result, although the possibility that it represents a true marker of thymic pathology in a patient who may later develop MG is challenging to exclude. The high PPV reported herein supports the use of anti-AChR/MuSK fixed CBA as first-line testing for suspected MG.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70123","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143690205","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurological long COVID in the outpatient clinic: Is it so long?","authors":"Stefano Giuseppe Grisanti,&nbsp;Sara Garbarino,&nbsp;Margherita Bellucci,&nbsp;Cristina Schenone,&nbsp;Valentina Candiani,&nbsp;Simmaco Di Lillo,&nbsp;Cristina Campi,&nbsp;Emanuela Barisione,&nbsp;Teresita Aloè,&nbsp;Elena Tagliabue,&nbsp;Alberto Serventi,&nbsp;Giampaola Pesce,&nbsp;Sara Massucco,&nbsp;Corrado Cabona,&nbsp;Anastasia Lechiara,&nbsp;Antonio Uccelli,&nbsp;Angelo Schenone,&nbsp;Michele Piana,&nbsp;Luana Benedetti","doi":"10.1111/ene.16510","DOIUrl":"10.1111/ene.16510","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Neurological involvement in long COVID (coronavirus disease 2019) is well known. In a previous study we identified two subtypes of neurological long COVID, one characterized by memory disturbances, psychological impairment, headache, anosmia and ageusia, and the other characterized by peripheral nervous system involvement, each of which present a different risk factor profile. In this study, we aimed to clarify the persistence of neurological long COVID symptoms with a significantly longer term follow-up.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We prospectively collected data from patients with prior COVID-19 infection who showed symptoms of neurological long COVID. We conducted a descriptive analysis to investigate the progression of neurological symptoms over time at 3-, 6-, 12-, and 18-month follow-ups. We performed a k-means clustering analysis on the temporal evolution of the symptoms at 6, 12, and 18 months. Finally, we assessed the difference between the recovery course of vaccinated and non-vaccinated patients by computing the cumulative recovery rate of symptoms in the two groups.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The study confirmed the presence of two subtypes of neurological long COVID. Further, 50% of patients presented a complete resolution of symptoms at 18 months of follow-up, regardless of which subtype of neurological long COVID they had. Vaccination against SARS-Cov-2 appeared to imply a higher overall recovery rate for all neurological symptoms, although the statistical reliability of this finding is hampered by the limited sample size of the unvaccinated patients included in this study.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Neurological long COVID can undergo complete resolution after 18 months of follow-up in 50% of patients and vaccination can accelerate the recovery.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11926444/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143669139","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Practice in evaluating solid organ transplant candidates and recipients with neurological impairment: The European Academy of Neurology neurocritical care panel survey","authors":"Vincenzina Lo Re,&nbsp;Giulia Fiume,&nbsp;Monica Rizzo,&nbsp;Federica Avorio,&nbsp;Maria Lolich,&nbsp;Emanuele Lo Gerfo,&nbsp;Massimo Pinzani,&nbsp;Antonio Toscano","doi":"10.1111/ene.70039","DOIUrl":"10.1111/ene.70039","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Brain disorders can occur in the context of peripheral organ diseases as well as solid organ transplants. The aim of this study was to explore the involvement of neurologists in the evaluation and management of solid organ transplant candidates and recipients when a nervous system impairment co-exists.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We invited all European Academy of Neurology members to answer a web-based survey. Descriptive statistics were used to summarize the results.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In total, 176 respondents completed the survey; neurologists are more involved in the evaluation of neurological complications after (31.8%) than before an organ transplant (21%), when they see a small volume of patients, and mainly for neurological comorbidities. A minority (9%) of neurologists received a specific training on the topic of neurological manifestations of extracranial conditions as well as only 6.2% of them are involved in research activities in the brain–body interactions field.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This survey highlights a mismatch between the impressive statistics on brain dysfunction in peripheral organ diseases as well as transplant and the small volume of solid organ transplant candidates/recipients seen by neurologists. According to recent recommendations every organ-eligible candidates should undergo a cognitive screening, however, it is not common that neurologists are involved in the multidisciplinary committee to determine the transplant eligibility. Delirium is the most frequent reason for a neurological consultation after a transplant, but also other critical brain conditions. Educational programs in the field of neurology of systemic diseases are worth being implemented as well as including neurologists in research programs on brain–body interactions.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11926398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143669186","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retrograde Amnesia in LGI1 and CASPR2 Limbic Encephalitis: Two Case Reports and a Systematic Literature Review","authors":"Malvaso Antonio,&nbsp;Denise Cerne,&nbsp;Bernini Sara,&nbsp;Bottiroli Sara,&nbsp;Marchioni Enrico,&nbsp;Businaro Pietro,&nbsp;Masciocchi Stefano,&nbsp;Morandi Chiara,&nbsp;Scaranzin Silvia,&nbsp;Emanuela Maria Mobilia,&nbsp;Stefano F. Cappa,&nbsp;Benedetti Luana,&nbsp;Franciotta Diego,&nbsp;Matteo Gastaldi","doi":"10.1111/ene.70113","DOIUrl":"10.1111/ene.70113","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Both anterograde and retrograde amnesia can typically co-occur in limbic autoimmune encephalitis (LAE), including the forms associated with antibodies to CASPR2/LGI1, two protein complexed with the voltage-gated potassium channel (VGKC). However, isolated retrograde amnesia is very rare, and it has never been described in LAE.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We report two patients with CASPR2 LAE who showed isolated retrograde amnesia, without other significant cognitive impairments. A systematic literature review was performed in accordance with the PRISMA guidelines on patients with LAE, antibodies to the VGKC complex (including LGI1, CASPR2, or the VGKC), and memory impairment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We identified 467 patients from 29 studies. Fourteen/467 had retrograde amnesia (2.9%), which co-occurred with anterograde amnesia in 12 with VGKC antibodies (7 with LGI1 LAE-like clinical phenotypes). Our two cases with CASPR2 LAE (2/469, 0.4%) were the only ones with isolated retrograde amnesia, which was actively investigated in only 56/467 patients. Thirteen/14 patients, including the two with isolated retrograde amnesia, had partial or poor cognitive improvement.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Retrograde amnesia is rare but likely under-recognized in VGKC-complex antibodies LAE and associates with poor recovery. When isolated, it adds to the spectrum of CASPR2 LAE. These findings promote insights into retrograde amnesia pathophysiology, deserving investigation across the whole spectrum of AE.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11927019/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143673629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}