Kana Lin, Guangyun Zhang, Yiran Zhou, Cuijin Wang, Feng Han, Li Liu, Jie Liu, Changhua Mou, Yunqing Zhou, Jiwen Wang, Dawei Yuan, Yingyan Wang
{"title":"Treatment history as early indicators of short-term response to ACTH therapy in infantile epileptic spasms syndrome: a propensity score matching analysis.","authors":"Kana Lin, Guangyun Zhang, Yiran Zhou, Cuijin Wang, Feng Han, Li Liu, Jie Liu, Changhua Mou, Yunqing Zhou, Jiwen Wang, Dawei Yuan, Yingyan Wang","doi":"10.1186/s42466-026-00513-4","DOIUrl":"10.1186/s42466-026-00513-4","url":null,"abstract":"<p><strong>Background: </strong>Adrenocorticotropic hormone (ACTH) is a first-line therapy for infantile epileptic spasms syndrome (IESS), along with oral steroids and vigabatrin. the UKISS/ICISS trials have established the superior short-term efficacy of hormonal therapy over vigabatrin and the potential benefit of combination therapy. However, early identification of short-term responders to ACTH remains a clinical challenge. This study investigated whether treatment history, including pre-treatment exposure and on-treatment events, can serve as early indicators of short-term response using propensity score matching (PSM) analysis.</p><p><strong>Methods: </strong>In this retrospective cohort study, we analyzed IESS patients who received ACTH therapy at Shanghai Children's Medical Center from March 2015 to February 2024. Responders were defined as those achieving ≥ 50% spasm reduction. PSM was applied using covariates with P < 0.20 in pre-matching analysis to balance baseline characteristics between responders and non-responders. Treatment history variables were compared using Chi-square or Fisher's exact tests. Variables with P < 0.20 in univariate analysis were included in a penalized logistic regression with L1 penalty (LASSO) to identify independent indicators.</p><p><strong>Results: </strong>Among 162 enrolled patients, 110 (55 responders, 55 non-responders) were matched with well-balanced baselines. Multivariate analysis using LASSO regression identified the addition of new antiseizure medications (ASMs) during ACTH therapy (OR = 0.055, 95% CI: 0.012-0.250, P < 0.001) and frequent ACTH dose adjustments (one adjustment: OR = 0.153, 95% CI: 0.030-0.780, P = 0.024; ≥2 adjustments: OR = 0.251, 95% CI: 0.086-0.731, P = 0.012) as having strong associations with lower odds of good short-term response, despite wide confidence intervals. Importantly, these factors likely represent markers of refractory disease rather than independent early predictors. Prior steroid/ACTH use showed a non-significant trend toward higher odds of good response (OR = 3.203, 95% CI: 0.794-12.923, P = 0.102).</p><p><strong>Conclusions: </strong>The addition of new ASMs and frequent dose adjustments during ACTH therapy might reflect early clinical concern for poor response and should prompt reassessment of treatment strategy. Recognizing these signals may facilitate timely clinical intervention.</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13366787/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148451037","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reply to the letter to the editor regarding \"diagnosis, treatment, and outcome prediction of non-convulsive status epilepticus in unconscious patients in intensive care units\".","authors":"Laurent M Willems, Adam Strzelczyk","doi":"10.1186/s42466-026-00511-6","DOIUrl":"10.1186/s42466-026-00511-6","url":null,"abstract":"","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13371009/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148451077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Moisés León-Ruiz, Carlos Castañeda-Cabrero, Julián Benito-León
{"title":"Letter to the Editor regarding \"Diagnosis, treatment, and outcome prediction of non-convulsive status epilepticus in unconscious patients in intensive care units\" by Willems et al.","authors":"Moisés León-Ruiz, Carlos Castañeda-Cabrero, Julián Benito-León","doi":"10.1186/s42466-026-00512-5","DOIUrl":"10.1186/s42466-026-00512-5","url":null,"abstract":"","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13371294/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148451057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carla Palleis, Endy Weidinger, Sylvia Maaß, Melanie Linke, Matthias Brendel, Sophia Stöcklein, Amani Al Tawil, Ulrich Mansmann, Daniel Teupser, Tobias Borst, Ralph Heimke-Brinck, Bernhard Hemmer, Günter U Höglinger, Helen Bidner, Mikael Simons, Johannes Levin
{"title":"Study protocol: double-blind, randomized, prospective, placebo controlled parallel group phase II study to investigate the effect of glycerol phenylbutyrate (GPB) on neurofilament light chain (NfL) levels in patients with corticobasal syndrome (CBS).","authors":"Carla Palleis, Endy Weidinger, Sylvia Maaß, Melanie Linke, Matthias Brendel, Sophia Stöcklein, Amani Al Tawil, Ulrich Mansmann, Daniel Teupser, Tobias Borst, Ralph Heimke-Brinck, Bernhard Hemmer, Günter U Höglinger, Helen Bidner, Mikael Simons, Johannes Levin","doi":"10.1186/s42466-026-00504-5","DOIUrl":"10.1186/s42466-026-00504-5","url":null,"abstract":"<p><strong>Introduction: </strong>Corticobasal syndrome (CBS) is a rare progressive neurodegenerative disorder, with no disease-modifying treatments currently available. The most common underlying pathology is a 4-repeat tauopathy. Neurofilament light chain (NfL) is a biomarker of neuronal damage and has shown potential as a measure of disease progression. Glycerol phenylbutyrate (GPB), a prodrug of phenylbutyric acid, has demonstrated potential neuroprotective properties in preclinical studies on tauopathies. This phase II clinical trial will investigate the effects of GPB on NfL levels in CBS patients. The primary objective is to assess the efficacy of GPB in reducing NfL levels over 26 weeks compared to placebo as well as safety and tolerability of GPB. Secondary objectives include evaluating changes in clinical scales.</p><p><strong>Methods and analysis: </strong>This is an investigator-initiated double-blind, randomized, placebo-controlled, parallel-group phase II clinical trial, performed in two German university hospitals. A total of 32 patients with CBS will be enrolled and randomized to receive either GPB or placebo. The primary outcome is the change in NfL levels between baseline and 26 weeks as well as safety and tolerability of GPB. Secondary outcomes are changes in clinical scores. Exploratory analyses involve pharmacokinetics, changes in the metabolomic, proteomic and lipidomic profiles and imaging outcomes, such as MRI and microglia-PET.</p><p><strong>Ethics and dissemination: </strong>The study protocol has been approved by the lead ethics committee at LMU Munich and conforms to the ethical principles outlined in the Declaration of Helsinki and Good Clinical Practice (GCP) guidelines.</p><p><strong>Perspectives: </strong>If successful, this clinical trial could identify a novel therapeutic approach for slowing disease progression in CBS, contributing to a broader understanding of GPB's therapeutic potential.</p><p><strong>Clinical trial registration: </strong>The clinical trial has been registered in ClinicalTrials.gov (NCT05983588) and due to Transition in the Clinical Trials Information System (CTIS; EUCT No. 2024-516897-31-00, date of transition: 2024-09-26).</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13332586/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148383410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Charlotte Schedlich-Teufer, Gregor A Brandt, Christina van der Linden, Hannah Jergas, Juan Carlos Baldermann, Vasilija Stopic, Gereon R Fink, Veerle Visser-Vandewalle, Till A Dembek, Michael T Barbe, Jan Niklas Petry-Schmelzer
{"title":"Stimulation settings in subthalamic nucleus deep brain stimulation for parkinson's disease - a retrospective single-center observational study.","authors":"Charlotte Schedlich-Teufer, Gregor A Brandt, Christina van der Linden, Hannah Jergas, Juan Carlos Baldermann, Vasilija Stopic, Gereon R Fink, Veerle Visser-Vandewalle, Till A Dembek, Michael T Barbe, Jan Niklas Petry-Schmelzer","doi":"10.1186/s42466-026-00477-5","DOIUrl":"10.1186/s42466-026-00477-5","url":null,"abstract":"<p><strong>Background: </strong>Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is a well-established treatment for Parkinson's disease (PD). Beyond basic omnidirectional, monopolar stimulation, advanced stimulation settings (AS), such as directional or vertical current steering, variation of pulse width and frequency, bipolar and interleaving stimulation are increasingly available. Our aim was to summarize current evidence on AS in STN-DBS in PD and systemically report their application and potential benefits in clinical routine.</p><p><strong>Methods: </strong>In this retrospective single-center observational study, we analyzed stimulation settings of 145 patients with bilateral STN-DBS 3, 6, and 12 months postoperatively. Secondary outcomes included preoperative levodopa response, lead positions, and postoperative reduction of levodopa-equivalent daily dose (LEDD) in patients staying with basic stimulation settings (BS) compared to those initially with AS at 3-months follow-up and those with a change from BS to AS.</p><p><strong>Results: </strong>AS were applied in 40.7%, 55.9%, and 73.8% of patients at 3, 6, and 12 months respectively. LEDD reduction after three months was higher in patients remaining with BS or initially AS than in patients with a switch to AS after three months, while there was no difference at 12 months. Median distance of leads to the center of gravity of the motor-STN was slightly larger when AS were applied.</p><p><strong>Conclusions: </strong>AS are frequently employed in clinical routine at a specialized DBS center. They may compensate for deviant lead placement in terms of stimulation efficacy measured by postoperative LEDD reduction. Prospective studies are warranted, focusing on specific AS indications in chronic DBS to optimize individual patient outcomes.</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13330291/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148378879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amelie Bohn, Fiona S Teubner, Simone C Tauber, Anne Waschbisch
{"title":"Longitudinal changes of serum immunoglobulins under ocrelizumab: factors associated with hypogammaglobulinemia and infection risk in a real-world multiple sclerosis cohort.","authors":"Amelie Bohn, Fiona S Teubner, Simone C Tauber, Anne Waschbisch","doi":"10.1186/s42466-026-00509-0","DOIUrl":"10.1186/s42466-026-00509-0","url":null,"abstract":"<p><strong>Background and objectives: </strong>Ocrelizumab (OCR) is an effective anti-CD20 therapy for relapsing-remitting and primary progressive multiple sclerosis (MS), but long-term effects on humoral immunity in real-world settings remain incompletely characterized. We investigated longitudinal changes in serum immunoglobulins, explored factors associated with hypogammaglobulinemia, and assessed associations with infection risk.</p><p><strong>Methods: </strong>In this retrospective, single-center observational study, adult patients with MS treated with OCR between 2018 and 2023 were included after ≥ 2 treatment cycles. Patients underwent standardized clinical, radiological, and laboratory follow-up, including serial measurements of serum immunoglobulins and varicella-zoster virus (VZV) serology. Infections were graded according to CTCAE criteria.</p><p><strong>Results: </strong>Of 116 patients (81% relapsing-remitting MS; median follow-up 2.3 years), approximately one-third developed lower limit of normal (LLN) IgM and one-sixth LLN IgG. Declines in immunoglobulins were class-specific and treatment-duration-dependent, most pronounced for IgM. Low baseline immunoglobulin levels were the strongest factors associated with LLN values, whereas demographic and disease-related factors were not. Prior immunotherapy, particularly fingolimod or interferon, was associated with hypogammaglobulinemia. Severe infections were rare (1.1 per 100 patient-years) and not consistently associated with LLN immunoglobulins.</p><p><strong>Discussion: </strong>OCR induced predictable, class-specific declines in immunoglobulins. Despite frequent hypogammaglobulinemia - particularly affecting IgM - no association with an increased short- to mid-term risk of clinically relevant infections was observed, although limited event numbers restrict conclusions. These findings support individualized immunoglobulin monitoring focused on patients with low baseline levels or prior immunotherapy exposure and underscore the need for prospective studies to define clinically meaningful thresholds for intervention during long-term anti-CD20 therapy.</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13317294/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148355107","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sarah Mai Viebahn, Emily Nothnik, Susan Raths, Markus Krohn, Finn Brüggemann, Thorsten Herr, Marie Süße, Agnes Flöel, Tjalf Ziemssen, Steffen Fleßa, Matthias Grothe
{"title":"Inpatient care for people with multiple sclerosis - a secondary data analysis in Germany between 2019 and 2024.","authors":"Sarah Mai Viebahn, Emily Nothnik, Susan Raths, Markus Krohn, Finn Brüggemann, Thorsten Herr, Marie Süße, Agnes Flöel, Tjalf Ziemssen, Steffen Fleßa, Matthias Grothe","doi":"10.1186/s42466-026-00507-2","DOIUrl":"10.1186/s42466-026-00507-2","url":null,"abstract":"<p><strong>Background: </strong>Multiple sclerosis (MS) is a chronic autoimmune disease imposing a substantial burden on healthcare systems. In Germany, MS care has been transitioning from inpatient to outpatient settings, driven by advances in disease-modifying therapies and evolving care infrastructure. Comprehensive data on inpatient utilisation trends and their determinants remain limited.</p><p><strong>Objectives: </strong>To analyse inpatient and day care admission patterns for people with MS (pwMS) in Germany between 2019 and 2024, examining trends by disease subtype, patient demographics, performed procedures, and regional distribution.</p><p><strong>Methods: </strong>Administrative data from the Institute for Hospital Remuneration (InEK) were analysed for inpatient cases coded under ICD-10-GM G35 (MS) from 2019 to 2024. Cases were stratified by MS subdiagnosis (G35.X). Regional analysis incorporated population density data from Destatis and hospital listings from the Bundes-Klinik-Atlas.</p><p><strong>Results: </strong>Nationwide inpatient MS admissions declined by 28%, from 45,067 cases in 2019 to 32,670 in 2024, while day care cases rose from 4,127 to 5,627. Regional variation in inpatient case reduction was substantial, ranging from - 11 to -59%. Declines were greatest for secondary progressive MS (37%) and primary progressive MS (31%). The average inpatient stay duration did not increase for admissions coded for first manifestation but did increase for all other disease courses. Diagnostic procedures dominated cases coded for first manifestation. Between 2019 and 2024, inpatient procedure rates per admission increased across most categories, and day care utilisation shifted markedly towards anti-CD20 immunotherapy administration.</p><p><strong>Conclusions: </strong>Inpatient MS care in Germany is declining substantially, with increasing day care utilisation partially compensating for this shift. Marked regional disparities and rising case complexity highlight the need for standardised outpatient care expansion and unified reimbursement frameworks.</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13309942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148341698","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kim L Vockert, Natalie Winter, Friederike S Kirschmann, Jörg B Schulz, Christiane Kuhl, Sven Nebelung, Teresa Lemainque, Maike F Dohrn
{"title":"Longitudinal five-year follow-up by muscle MRI and high-resolution nerve ultrasound in hereditary neuropathies.","authors":"Kim L Vockert, Natalie Winter, Friederike S Kirschmann, Jörg B Schulz, Christiane Kuhl, Sven Nebelung, Teresa Lemainque, Maike F Dohrn","doi":"10.1186/s42466-026-00508-1","DOIUrl":"10.1186/s42466-026-00508-1","url":null,"abstract":"<p><strong>Background and aims: </strong>Hereditary polyneuropathies are rare heterogeneous neurological disorders that cause progressive muscle weakness and disability. As new therapies emerge, sensitive imaging biomarkers to monitor disease progression are essential. This exploratory longitudinal study evaluated MRI with focus on the lower limbs and high-resolution ultrasound (HRUS) at two points over the period of 4.75 years in genetically confirmed patients, and compared imaging with clinical and electrophysiological findings.</p><p><strong>Methods: </strong>Fourteen patients (CMT1A, CMT1X, CMT1B, CMT1C, CMT2, and hereditary myoneuropathy) underwent standardized clinical examinations, including Charcot-Marie-Tooth Examination Score version 2 (CMTESv2), Medical Research Counsil muscle strength, and sensory testing. Nerve conduction studies (NCS) were performed on upper limb nerves. MRI of lower limbs evaluated muscle volume, normalized T1w signal intensity (nSI), and proton density fat fraction (PDFF). HRUS was used to assess nerve cross-sectional areas, the ultrasound pattern sum score (UPSS), and muscle echogenicity.</p><p><strong>Results: </strong>Over the observation interval, patients showed a significant increase in CMTESv2 (p = 0.0031), especially in motor symptoms (p = 0.0012). MRI-derived muscle volumes did not change; however, nSI significantly increased in gastrocnemius lateralis muscles (p = 0.0023), indicating progressive fat infiltration. UPSS increased in demyelinating polyneuropathies (p = 0.0299) and was inversely correlated with nerve conduction velocities of the median (p = 0.0413) and ulnar nerves (p = 0.0064).</p><p><strong>Interpretation: </strong>Over this 5-year interval, our cohort showed clinical disease progression, which was not reflected by NCS and MRI volumetry. In contrast, MRI-derived nSI correlated with PDFF and captured progressive muscle fat replacement, correlating with declining muscle strength. UPSS showed longitudinal progression in demyelinating neuropathies. PDFF and UPSS are promising biomarkers and may support outcome evaluation in future clinical trials.</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13292321/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148311069","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jan Purrucker, Kirsten Haas, Marilen Sieber, Viktoria Rücker, Peter U Heuschmann, Roland Veltkamp
{"title":"Author response to Sinha, \"Comment on \"Intracranial hemorrhage after ischemic stroke in patients on direct oral anticoagulants: Results from a prospective observational study\"\".","authors":"Jan Purrucker, Kirsten Haas, Marilen Sieber, Viktoria Rücker, Peter U Heuschmann, Roland Veltkamp","doi":"10.1186/s42466-026-00495-3","DOIUrl":"10.1186/s42466-026-00495-3","url":null,"abstract":"","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13289500/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148304501","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Unmet needs in the care of patients with neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody associated disease: insights from Germany.","authors":"Katrin Giglhuber, Alix Bertrand, Clarissa Zappe, Ingo Kleiter, Ilya Ayzenberg, Achim Berthele","doi":"10.1186/s42466-026-00503-6","DOIUrl":"10.1186/s42466-026-00503-6","url":null,"abstract":"<p><strong>Background: </strong>Neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are rare autoimmune disorders. Their true prevalence in Germany is unknown and can only be estimated from heterogeneous international data. Assuming 1-3 cases per 100,000 people for each disease suggests several thousand affected individuals nationwide, yet the German Neuromyelitis Optica Study Group (NEMOS) registry currently holds records of only about 1,300 patients seen in specialised centres. Numbers and care structures outside such facilities remain largely unknown. This survey aimed to assess the current state of NMOSD and MOGAD care in Germany, identify gaps, and inform future care strategies.</p><p><strong>Methods: </strong>An online questionnaire aimed at neurologists and neuropaediatricians was distributed via NEMOS, the German Neurological Society (DGN), the Professional Association of German Neurologists (BVDN), and the German Network for Research on Autoimmune Encephalitis (GENERATE) from March to May 2025. Questions addressed care structures, diagnostics, coding, treatment, guideline use, and practitioners' needs.</p><p><strong>Results: </strong>A total of 104 physicians from all German federal states participated. Half worked in university hospitals, the remainder in other clinics and outpatient settings. Most were specialised in neuroimmunology (70.2%). Many reported an increase in patient numbers for NMOSD (55.8%) and MOGAD (77.4%). Diagnostic practices revealed significant inconsistencies: almost half of the respondents were unaware of their referral laboratory's antibody assays, and ELISA remained in use despite clear recommendations for cell-based assays. ICD-10 coding varied widely. Off-label rituximab was most frequently used for first-line therapy of AQP4-antibody-positive NMOSD (69.6%), compared to satralizumab (57.1%), ravulizumab (55.4%) and inebilizumab (50.0%). AQP4-antibody-negative NMOSD was mainly treated with rituximab (87.0%). Also in MOGAD, rituximab was frequently used (by 58.9%), yet paediatricians preferred glucocorticoids and intravenous immunoglobulins. 69.6% initiated treatment for MOGAD after the first attack. Notably, 41.8% of physicians reported untreated NMOSD and 64.6% untreated MOGAD patients. Most respondents relied on national guidelines; 43.2% expressed a need for further education and patient information.</p><p><strong>Conclusions: </strong>Our findings highlight substantial heterogeneity in the diagnosis and treatment of NMOSD and MOGAD in Germany with potential implications for patient outcomes. This underscores the need for harmonised procedures and targeted educational resources to improve diagnostic reliability, treatment equity, and overall quality of care.</p>","PeriodicalId":94156,"journal":{"name":"Neurological research and practice","volume":"8 1","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13282859/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148284993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}