cover image European Journal of Neurology

European Journal of Neurology

2024 - Volume 31
Issue 5 | May 2024
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ISSUE INFORMATION

Issue Information

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Article first published online:
07 Apr 2024 | DOI: 10.1111/ene.15881

CASE REPORT

Background and purpose

Charcot−Marie−Tooth disease type 1A (CMT1A) is the most prevalent hereditary neuropathy worldwide and classically has slow nerve conduction velocity (NCV), in most cases below 38 m/s. Two unrelated patients with motor NCVs in the upper limbs above 38 m/s are reported.

Method

Case report.

Results

Two genetically confirmed CMT1A patients are presented, from two unrelated families (one of British origin and the other of Brazilian origin). Both individuals had upper limb motor NCVs above 38 m/s, with values ranging from 41.9 to 45 m/s in the median nerve and from 42 to 42.3 m/s in the ulnar nerve. They presented with a very mild phenotype, with CMT Neuropathy Score version 2 (CMTNSv2) of 6 and 5, respectively. In contrast, affected family members within both kinships exhibited a classical phenotype with more severe disease manifestation (CMTNSv2 ranging from 12 to 20) and motor NCVs below 30 m/s.

Conclusion

These cases, although very rare, highlight the importance of testing duplication in patients with intermediate conduction velocities.

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Authors:
Pedro José Tomaselli, Julian Blake, James M. Polke, Osvaldo José Moreira do Nascimento, Mary M. Reilly, Wilson Marques Júnior and Matilde Laurá
Article first published online:
26 Feb 2024 | DOI: 10.1111/ene.16199

ORIGINAL ARTICLE

Background

Nonreversible hearing loss (HL) is the main sequelae of Susac syndrome (SuS). We aimed to identify risk factors for HL in SuS.

Methods

The CARESS study is a prospective national cohort study that started in December 2011, including all consecutive patients with SuS referred to the French reference center. The CARESS study was designed with a follow‐up including fundoscopy, audiometry, and brain magnetic resonance imaging at 1, 3, 6, and 12 months after diagnosis and then annually for 5 years. The primary outcome was the occurrence at last follow‐up of severe HL defined as the loss of 70 dB in at least one ear on audiometry or the need for hearing aids.

Results

Thirty‐six patients (female 66.7%, median age 37.5 [range 24.5–42.5] years) included in the clinical study were analyzed for the primary outcome. Thirty‐three patients (91.7%) had cochleovestibular involvement at SuS diagnosis including HL >20 dB in at least one ear in 25 cases. At diagnosis, 32 (88.9%), 11 (30.6%), and 7 (19.4%) patients had received steroids, intravenous immunoglobulin, and/or immunosuppressive (IS) drugs, respectively. After a median follow‐up of 51.8 [range 29.2–77.6] months, 19 patients (52.8%) experienced severe HL that occurred a median of 13 [range 1.5–29.5] months after diagnosis. Multivariable analysis showed that the odds of severe HL were lower in patients who received IS drugs at diagnosis (OR 0.15, 95% CI 0.01–1.07,  = 0.058).

Conclusions

Severe HL in SuS is associated with the absence of IS drugs given at diagnosis. Our findings support the systematic use of IS drugs in SuS.

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Authors:
Marion Peyre, Arthur Mageau, Marie‐Cécile Henry Feugeas, Serge Doan, Caroline Halimi, Isabelle Klein, Tiphaine Goulenok, Chrystelle François, Marie‐Paule Chauveheid, Thomas Papo, Karim Sacré, Jean‐Francois Alexandra, Olivier Aumaitre, Naima Beldjoudi, Marie Bodenant, Jean Capron, Thomas de Brouker, Elisabeth Medeiros de Bustos, Sabrina Debruxelles, Nicole Delory, Antoine Dossier, Emmanuel Ellie, Fatima Farhi, Sophie Godard‐Ducceschi, Bertrand Godeau, Deborah Grosset‐Janin, Mohamed Hamidou, Cedric Laouenan, Bertand Lapergue, Alain Le Quellec, Shirine Mohamed, Luc Mouthon, Jean‐Baptiste Noury, Diane Rouzaud, Guillaume Turc and Anne Wacongne
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16211

ORIGINAL ARTICLE

Background and purpose

Myopathies are associated with classic signs and symptoms, but also with possible life‐threatening complications that may require assistance in an emergency setting. This phenomenon is understudied in the literature. We aimed to assess the presentation, management, and outcomes of clinical manifestations potentially related to a muscle disorder requiring referral to the adult emergency department (ED) and hospitalization.

Methods

Anonymized patient data retrieved using the International Classification of Diseases, Ninth Revision codes related to muscle disorders over 4 years were retrospectively analyzed. Medical reports were evaluated to extract demographic and clinical variables, along with outcomes. Two groups were defined based on the presence (known diagnosis [KD] group) or absence (unknown diagnosis [UD] group) of a diagnosed muscle disorder at arrival.

Results

A total of 244 patients were included, 51% of whom were affected by a known myopathy, predominantly limb‐girdle muscular dystrophies and myotonic dystrophies. The main reasons for ED visits in the KD group were respiratory issues, worsening of muscle weakness, and gastrointestinal problems. Heart complications were less prevalent. In the UD group, 27 patients received a new diagnosis of a specific primary muscle disorder after the ED access, mostly an inflammatory myopathy. Death during hospitalization was recorded in 26 patients, with a higher rate in the KD group and in patients affected by mitochondrial and inflammatory myopathies. Sepsis and dyspnea were associated with increased death risk.

Conclusions

Respiratory complications are the most common reason for myopathic patients accessing the ED, followed by gastrointestinal issues. Infections are severe threats and, once hospitalized, these patients have relatively high mortality.

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Authors:
Mauro Monforte, Eleonora Torchia, Sara Bortolani, Beatrice Ravera, Enzo Ricci, Gabriella Silvestri, Serenella Servidei, Guido Primiano, Massimiliano Mirabella, Mario Sabatelli, Eugenio Mercuri, Francesco Franceschi, Paolo Calabresi, Marcello Covino and Giorgio Tasca
Article first published online:
16 Jan 2024 | DOI: 10.1111/ene.16214

ORIGINAL ARTICLE

Background and purpose

Anti‐calcitonin gene‐related peptide (CGRP) therapies are recent preventive therapies approved for both episodic and chronic migraine. One of the measures of effectiveness is the withdrawal of other preventive treatments. The objective of this study is to quantify the impact of anti‐CGRP drugs in concomitant preventive treatment in patients with migraine.

Methods

This was an observational, retrospective, multicenter cohort study with patients from nine national headache units. Patients with migraine undergoing treatment for at least 6 months with anti‐CGRP antibodies, who were initially associated with some preventive treatment (oral and/or onabotulinumtoxinA) were included. Demographic and clinical variables were collected, as well as variables related to headache. Differences according to withdrawal or nonwithdrawal were evaluated.

Results

A total of 408 patients were included, 86.52% women, 48.79 (SD = 1.46) years old. Preventive treatment was withdrawn in 43.87% (179/408), 20.83% partially and 23.04% totally. In 27.45% (112/408), it was maintained exclusively due to comorbidity and in 28.6% (117/408) due to partial efficacy. The most frequent time of withdrawal was between 3 and 5 months after the start of treatment. The baseline characteristics associated with nonwithdrawal were comorbidities: insomnia, hypertension and obesity, chronic migraine, and medication overuse. In the multivariate analysis, the absence of high blood pressure, a greater number of preventive treatments at the start, and a lower number of migraine days/month after anti‐CGRP treatment were independently associated with withdrawal of the treatment ( < 0.05).

Conclusions

Anti‐CGRP antibodies allow the withdrawal of associated preventive treatment in a significant percentage of patients, which supports its effectiveness in real‐life conditions.

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Authors:
Ana Beatriz Gago‐Veiga, Noelia Lopez‐Alcaide, Sonia Quintas, Iris Fernández Lázaro, Javier Casas‐Limón, Carlos Calle, Germán Latorre, Nuria González‐García, Jesús Porta‐Etessam, Jaime Rodriguez‐Vico, Alex Jaimes, Andrea Gómez García, David García‐Azorín, Ángel Luis Guerrero‐Peral, Álvaro Sierra, Alberto Lozano Ros, Antonio Sánchez‐Soblechero, Javier Díaz‐de‐Teran, Javier A. Membrilla, Cristina Treviño and Alicia Gonzalez‐Martinez
Article first published online:
07 Feb 2024 | DOI: 10.1111/ene.16215

SHORT COMMUNICATION

Background and purpose

Identifying vestibular causes of dizziness and unsteadiness in multi‐sensory neurological disease can be challenging, with problems typically attributed to central or peripheral nerve involvement. Acknowledging vestibular dysfunction as part of the presentation provides an opportunity to access targeted vestibular rehabilitation, for which extensive evidence exists. A diagnostic framework was developed and validated to detect vestibular dysfunction, benign paroxysmal positional vertigo or vestibular migraine. The specificity and sensitivity of the diagnostic framework was tested in patients with primary mitochondrial disease.

Methods

Adults with a confirmed diagnosis of primary mitochondrial disease were consented, between September 2020 and February 2022. Participants with and without dizziness or unsteadiness underwent remote physiotherapy assessment and had in‐person detailed neuro‐otological assessment. The six framework question responses were compared against objective neuro‐otological assessment or medical notes. The output was binary, with sensitivity and specificity calculated.

Results

Seventy‐four adults completed the study: age range 20–81 years (mean 48 years, ±SD 15.05 years); ratio 2:1 female to male. The framework identified a vestibular diagnosis in 35 participants, with seven having two diagnoses. The framework was able to identify vestibular diagnoses in adults with primary mitochondrial disease, with a moderate (40–59) to very high (90–100) sensitivity and positive predictive value, and moderate to high (60–74) to very high (90–100) specificity and negative predictive value.

Conclusions

Overall, the clinical framework identified common vestibular diagnoses with a moderate to very high specificity and sensitivity. This presents an opportunity for patients to access effective treatment in a timely manner, to reduce falls and improve quality of life.

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Authors:
Amanda J. Male, Sarah L. Holmes, Nehzat Koohi, Magdalena Dudziec, Michael G. Hanna, Gita M. Ramdharry, Chiara Pizzamiglio, Robert D. S. Pitceathly and Diego Kaski
Article first published online:
21 Jan 2024 | DOI: 10.1111/ene.16216

ORIGINAL ARTICLE

Background and purpose

Post‐stroke movement disorders (PMDs) following ischemic lesions of the basal ganglia (BG) are a known entity, but data regarding their incidence are lacking. Ischemic strokes secondary to proximal middle cerebral artery (MCA) occlusion treated with thrombectomy represent a model of selective damage to the BG. The aim of this study was to assess the prevalence and features of movement disorders after selective BG ischemia in patients with successfully reperfused acute ischemic stroke (AIS).

Methods

We enrolled 64 consecutive subjects with AIS due to proximal MCA occlusion treated with thrombectomy. Patients were clinically evaluated by a movement disorders specialist for PMDs onset at baseline, and after 6 and 12 months.

Results

None of the patients showed an identifiable movement disorder in the subacute phase of the stroke. At 6 and 12 months, respectively, 7/25 (28%) and 7/13 (53.8%) evaluated patients developed PMDs. The clinical spectrum of PMDs encompassed parkinsonism, dystonia and chorea, either isolated or combined. In most patients, symptoms were contralateral to the lesion, although a subset of patients presented with bilateral involvement and prominent axial signs.

Conclusion

Post‐stroke movement disorders are not uncommon in long‐term follow‐up of successfully reperfused AIS. Follow‐up conducted by a multidisciplinary team is strongly advisable in patients with selective lesions of the BG after AIS, even if asymptomatic at discharge.

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Authors:
Leonardo Rigon, Danilo Genovese, Carla Piano, Valerio Brunetti, Valeria Guglielmi, Angelo Tiziano Cimmino, Irene Scala, Salvatore Citro, Anna Rita Bentivoglio, Eleonora Rollo, Riccardo Di Iorio, Aldobrando Broccolini, Roberta Morosetti, Mauro Monforte, Giovanni Frisullo, Pietro Caliandro, Alessandro Pedicelli, Anselmo Caricato, Giovanna Masone, Paolo Calabresi and Giacomo Della Marca
Article first published online:
01 Feb 2024 | DOI: 10.1111/ene.16219

LETTER TO THE EDITOR

Melatonin's role in managing nocturia in Parkinson disease: A critical examination of recent studies

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Authors:
Yu‐Hsiang Lin and Horng‐Heng Juang
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16220

ORIGINAL ARTICLE

Background and purpose

Biological sex is known to have an impact on quality metrics of acute stroke. We aimed to determine whether COVID positivity accentuates this effect and constitutes worse outcome.

Methods

The present analysis was based on the Global COVID‐19 Stroke Registry, a retrospective, international, cohort study of consecutive ischemic stroke patients receiving intravenous thrombolysis and/or endovascular thrombectomy between 1 March 2020 and 30 June 2021. We investigated differences between the sexes in patient characteristics, acute stroke metrics as well as post‐stroke outcome in COVID‐positive and COVID‐negative stroke patients undergoing acute revascularization procedures.

Results

A total of 15,128 patients from 106 centers were recorded in the Global COVID‐19 Stroke Registry, 853 (5.6%) of whom were COVID‐positive. Overall, COVID‐positive individuals were treated significantly slower according to every acute stroke metric compared to COVID‐negative patients. We were able to show that key quality indicators in acute stroke treatment were unfavorable for COVID‐negative women compared to men (last‐seen‐well‐to‐door time + 11 min in women). Furthermore, COVID‐negative women had worse 3‐month outcomes (3‐month modified Rankin Scale score [interquartile range] 3.0 [4.0] vs. 2.0 [3.0];  < 0.01), even after adjusting for confounders. In COVID‐positive individuals no such difference between the sexes, either in acute management metrics or in 3‐month outcome, was seen.

Conclusion

Known sex‐related differences in acute stroke management exist and extend to times of crisis. Nevertheless, if patients were COVID‐19‐positive at stroke onset, women and men were treated the same, which could be attributed to structured treatment pathways.

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Authors:
Lukas Mayer‐Suess, João Pedro Marto, Davide Strambo, George Ntaios, Thanh Nguyen, Stefan Kiechl, Raimund Pechlaner, Raul Nogueira, Patrik Michel and Michael Knoflach
Article first published online:
30 Jan 2024 | DOI: 10.1111/ene.16221

ORIGINAL ARTICLE

Background and purpose

Clinical symptoms and laboratory indices for acute inflammatory demyelinating polyneuropathy (AIDP), a variant of Guillain−Barré syndrome, and acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP) were analyzed to identify factors that could contribute to early differential diagnosis.

Methods

A retrospective chart review was performed on 44 AIDP and 44 A‐CIDP patients looking for any demographic characteristics, clinical manifestations or laboratory parameters that might differentiate AIDP from acutely presenting CIDP.

Results

In Guillain−Barré syndrome patients ( = 63), 69.84% ( = 44) were classified as having AIDP, 19.05% ( = 12) were found to have acute motor axonal neuropathy, 6.35% ( = 4) were found to have acute motor and sensory axonal neuropathy, and 4.76% ( = 3) were found to have Miller Fisher syndrome. Serum uric acid (UA) was higher in A‐CIDP patients (329.55 ± 72.23 μmol/L) than in AIDP patients (221.08 ± 71.32 μmol/L) ( = 0.000). Receiver operating characteristic analyses indicated that the optimal UA cutoff was 283.50 μmol/L. Above this level, patients were more likely to present A‐CIDP than AIDP (specificity 81.80%, sensitivity 81.80%). During the follow‐up process, serum samples were effectively collected from 19 AIDP patients during the rehabilitation phase and 28 A‐CIDP patients during the remission stage, and it was found that UA levels were significantly increased in A‐CIDP (remission) (298.9 ± 90.39 μmol/L) compared with AIDP (rehabilitation) (220.1 ± 108.2 μmol/L,  = 0.009).

Conclusion

These results suggest that serum UA level can help to differentiate AIDP from A‐CIDP with high specificity and sensitivity, which is helpful for early diagnosis and guidance of treatment.

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Authors:
Weiyun Zhang, Wen Tao, Jun Wang, Ping Nie, Lihui Duan and Lanyun Yan
Article first published online:
14 Feb 2024 | DOI: 10.1111/ene.16222

ORIGINAL ARTICLE

Background and purpose

Pompe disease is a rare, inheritable, progressive metabolic myopathy. This study aimed to estimate the minimal clinically important difference (MCID) for an improvement in forced vital capacity in the upright seated position (FVC) and the 6‐min walk test (6MWT) after a year of treatment with enzyme replacement therapy.

Methods

Data were obtained from two prospective follow‐up studies. Between‐group and within‐group MCIDs were estimated using anchor‐based methods. Additionally, a distribution‐based method was used to generate supportive evidence. As anchors, self‐reported change in health and in physical functioning, shortness of breath and a categorization of the Short‐Form 36 Physical Component Summary score were used. Anchor appropriateness was assessed using Spearman correlations (absolute values ≥0.29) and a sufficient number of observations in each category.

Results

In all, 102 patients had at least one FVC or 6MWT measurement during enzyme replacement therapy. Based on the anchors assessed as appropriate, the between‐group MCID for an improvement in FVC ranged from 2.47% to 4.83% points. For the 6MWT, it ranged from 0.35% to 7.47% points which is equivalent to a distance of 2.18–46.61 m and 1.97–42.13 m for, respectively, a man and a woman of age 50, height 1.75 m and weight 80 kg. The results of the distribution‐based method were within these ranges when applied to change in the outcome values.

Conclusion

The MCIDs for FVC and 6MWT derived in this study can be used to interpret differences between and within groups of patients with Pompe disease in clinical trials and cohort studies.

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Authors:
Aglina Lika, Eleni‐Rosalina Andrinopoulou, Nadine A. M. E. van der Beek, Dimitris Rizopoulos, Ans T. van der Ploeg and Michelle E. Kruijshaar
Article first published online:
20 Feb 2024 | DOI: 10.1111/ene.16223

ORIGINAL ARTICLE

Background and purpose

Dysphagia is associated with poor outcome, higher mortality, reduced quality of life, and social isolation. We investigate the relationship between swallowing impairment and symptoms of anxiety and depression after ischemic stroke.

Methods

Consecutive patients with ischemic stroke participating in the prospective STROKE‐CARD Registry study from 2020 to 2022 were assessed for dysphagia on hospital admission (clinical swallowing assessment) and for persistence until discharge and 3‐month follow‐up (SINGER Independency Index). Anxiety and depression symptoms were recorded using Beck Depression Inventory (BDI) and Hospital Anxiety and Depression Scale (HADS) at 3‐month follow‐up.

Results

Of 648 patients, 19.3% had dysphagia on admission, persisting in 14.8% at discharge and 6.8% at 3‐month follow‐up. With the presence or duration of dysphagia (no dysphagia, dysphagia at baseline, at discharge, at 3 months), score (mean ± SD) increased on the BDI (7.9 ± 6.7, 12.5 ± 8.7, 13.5 ± 9.0, 16.5 ± 10.2), HADS‐D (4.4 ± 3.7, 7.1 ± 4.2, 7.7 ± 4.4, 9.8 ± 4.3), and HADS‐A (4.4 ± 3.5, 5.4 ± 3.6, 6.0 ± 3.6, 7.0 ± 3.6). In linear regression analysis adjusting for age, sex, diabetes, dementia, and either functional disability or stroke severity, BDI and HADS‐D scores were significantly higher in patients with dysphagia across all points in time (admission, discharge, 3‐month follow‐up). An independent association with HADS‐A scores was only evident in patients with persisting dysphagia after 3 months. Patients with dysphagia were more likely to receive antidepressants, antipsychotics, or benzodiazepines at discharge and 3‐month follow‐up.

Conclusions

Dysphagia after stroke is common and severely affects psychosocial functioning of individuals. Our results highlight swallowing impairment as an independent predictor for poststroke depressive and, to a lesser extent, anxiety symptoms.

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Authors:
Anel Karisik, Benjamin Dejakum, Kurt Moelgg, Silvia Komarek, Thomas Toell, Lukas Mayer‐Suess, Raimund Pechlaner, Stefanie Kostner, Simon Sollereder, Sophia Kiechl, Sonja Rossi, Gudrun Schoenherr, Wilfried Lang, Stefan Kiechl, Michael Knoflach, Christian Boehme, Gregor Broessner, Michael Eller, Julia Ferrari, Ton Hanel, Katharina Kaltseis, Theresa Köhler, Stefan Krebs, Florian Krismer, Christoph Mueller, Wolfgang Nachbauer, Anna Neuner, Anja Perfler, Theresa Schneider and Christine Span
Article first published online:
02 Feb 2024 | DOI: 10.1111/ene.16224

CASE REPORT

Background

Damage to the insula has been associated with various types of cardiovascular dysfunction, including arrhythmias and blood pressure imbalances. Acute neuroendocrine disturbances following insular damage have also been described.

Case Presentation

A 50‐year‐old right‐handed man with a left insular ischemic lesion exhibited aphasia and right central VII nerve palsy. Five days after the stroke, the patient exhibited severe bradycardia and hypotension. He had been treated for ocular trauma with prednisone for the preceding 3 weeks. Cortisol and adrenocorticotropic hormone levels indicated secondary adrenal insufficiency. Despite adequate fluid intake, the patient's blood pressure dropped, requiring norepinephrine administration. Midodrine was also initiated, leading to clinical improvement. The therapy was gradually discontinued as vital signs normalized. By Day 24, electrocardiogram monitoring was unremarkable, hormonal levels normalized, and the neurological examination revealed only mild residual speech fluency impairment. Computed tomography scans confirmed a recovering ischemic lesion of the left insula.

Conclusions

This case reveals the inhibitory effect exerted by a left‐sided insular stroke on the autonomic system. It also highlights the still largely unexplored neuroendocrine complications of damage to this brain region.

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Authors:
Mirella Russo, Fedele Dono, Marco Onofrj and Stefano L. Sensi
Article first published online:
01 Feb 2024 | DOI: 10.1111/ene.16225

ORIGINAL ARTICLE

Background and purpose

Ischemic stroke, a major contributor to global disability and mortality, is underpinned by intricate pathophysiological mechanisms, notably neuroinflammation and immune cell dynamics. Prior research has identified a nuanced and often paradoxical link between immune cell phenotypes and ischemic stroke susceptibility. The aim of this study was to elucidate the potential causal links between the median fluorescence intensity (MFI) and morphological parameters (MP) of 731 immune cell types and ischemic stroke risk.

Methods

By analyzing extensive genetic datasets, we conducted comprehensive Mendelian randomization (MR) analyses to discern the genetic correlations between diverse immune cell attributes (MFI and MP) and ischemic stroke risk.

Results

Our study identified key immune cell signatures linked to ischemic stroke risk. Both B cells and T cells, among other immune cell types, have a bidirectional influence on stroke risk. Notably, the regulatory T‐cell phenotype demonstrates significant neuroprotective properties, with all odds ratio (OR) values and confidence intervals (CIs) being less than 1. Furthermore, CD39 phenotype immune cells, particularly CD39+ CD8+ T cells (inverse variance weighting [IVW] OR 0.92, 95% CI 0.87–0.97;  = 0.002) and CD39+ activated CD4 regulatory T cells (IVW OR 0.93, 95% CI 0.90–0.97;  < 0.001), show notable neuroprotection against ischemic stroke.

Conclusion

This investigation provides new genetic insights into the interplay between various immune cells and ischemic stroke, underscoring the complex role of immune processes in stroke pathogenesis. These findings lay a foundation for future research, which may confirm and expand upon these links, potentially leading to innovative immune‐targeted therapies for stroke prevention and management.

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Authors:
Xia Deng, Shuai Hou, Yanqiang Wang, Haiyan Yang and Chunping Wang
Article first published online:
07 Feb 2024 | DOI: 10.1111/ene.16226

ORIGINAL ARTICLE

Background

The aim of this study was to analyze cold stimulation‐induced changes in cerebral and cardiac hemodynamics.

Methods

Upon ingestion of an ice cube, the changes in resistance index, mean flow velocity and flow index of the middle cerebral arteries (MCA) were assessed using transcranial Doppler sonography. Extracranial duplex sonography was used to measure the mean flow velocity and resistance index of the right internal carotid artery (ICA). The change in mean arterial pressure, heart rate, root mean square of successive differences (RMSSD) and end‐tidal carbon dioxide pressure were analyzed additionally. These changes were compared to sham stimulation.

Results

Compared with sham stimulation, cooling of the oral cavity resulted in significant changes in cerebral and cardiac hemodynamics. The cold stimulation decreased the resistance index in the MCA (−4.5% ± 5.4%,  < 0.0001) and right ICA (−6.3% ± 15.6%,  = 0.001). This was accompanied by an increase in mean flow velocity (4.1% ± 8.0%,  < 0.0001) and flow index (10.1% ± 43.6%,  = 0.008) in the MCA. The cardiac effects caused an increase in mean arterial pressure (1.8% ± 11.2%,  = 0.017) and RMSSD (55% ± 112%,  = 0.048), while simultaneously decreasing the heart rate (−4.3% ± 9.6%,  = 0.0001).

Conclusion

Cooling of the oral cavity resulted in substantial changes in cerebral and cardiac hemodynamics resulting in a blood flow diversion to the brain.

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Authors:
Ole Hensel
Article first published online:
02 Feb 2024 | DOI: 10.1111/ene.16227

ORIGINAL ARTICLE

Background and purpose

Rituximab (RTX) is frequently used off‐label in multiple sclerosis. However, studies on the risk–benefit profile of RTX in pediatric‐onset multiple sclerosis are scarce.

Methods

In this multicenter retrospective cohort study, patients with pediatric‐onset multiple sclerosis from Sweden, Austria and Germany, who received RTX treatment were identified by chart review. Annualized relapse rates, Expanded Disability Status Scale scores and magnetic resonance imaging parameters (new T2 lesions and contrast‐enhancing lesions) were assessed before and during RTX treatment. The proportion of patients who remained free from clinical and disease activity (NEDA‐3) during RTX treatment was calculated. Side effects such as infusion‐related reactions, infections and laboratory abnormalities were assessed.

Results

Sixty‐one patients received RTX during a median (interquartile range) follow‐up period of 20.9 (35.6) months. The annualized relapse rate decreased from 0.6 (95% confidence interval [CI] 0.38–0.92) to 0.03 (95% CI 0.02–0.14). The annual rate of new T2 lesions decreased from 1.25 (95% CI 0.70–2.48) to 0.08 (95% CI 0.03–0.25) and annual rates of new contrast‐enhancing lesions decreased from 0.86 (95% CI 0.30–3.96) to 0. Overall, 70% of patients displayed no evidence of disease activity (NEDA‐3). Adverse events were observed in 67% of patients. Six patients discontinued treatment due to ongoing disease activity or adverse events.

Conclusion

Our study provides class IV evidence that RTX reduces clinical and radiological activity in pediatric‐onset multiple sclerosis.

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Authors:
Markus Breu, Fredrik Sandesjö, Ruxandra‐Iulia Milos, Jan Svoboda, Jonatan Salzer, Lisa Schneider, Julian Benedikt Reichelt, Annikki Bertolini, Astrid Blaschek, Katharina Fink, Romana Höftberger, Jan Lycke, Kevin Rostásy, Rainer Seidl, Sandy Siegert, Ronny Wickström and Barbara Kornek
Article first published online:
20 Feb 2024 | DOI: 10.1111/ene.16228

POSITION PAPER

Background

Generalized myasthenia gravis (MG) with antibodies against the acetylcholine receptor is a chronic disease causing muscle weakness. Access to novel treatments warrants authoritative treatment recommendations. The Nordic countries have similar, comprehensive health systems, mandatory health registers, and extensive MG research.

Methods

MG experts and patient representatives from the five Nordic countries formed a working group to prepare treatment guidance for MG based on a systematic literature search and consensus meetings.

Results

Pyridostigmine represents the first‐line symptomatic treatment, while ambenonium and beta adrenergic agonists are second‐line options. Early thymectomy should be undertaken if a thymoma, and in non‐thymoma patients up to the age of 50–65 years if not obtaining remission on symptomatic treatment. Most patients need immunosuppressive drug treatment. Combining corticosteroids at the lowest possible dose with azathioprine is recommended, rituximab being an alternative first‐line option. Mycophenolate, methotrexate, and tacrolimus represent second‐line immunosuppression. Plasma exchange and intravenous immunoglobulin are used for myasthenic crises and acute exacerbations. Novel complement inhibitors and FcRn blockers are effective and fast‐acting treatments with promising safety profiles. Their use depends on local availability, refunding policies, and cost–benefit analyses. Adapted physical training is recommended. Planning of pregnancies with optimal treatment, information, and awareness of neonatal MG is necessary. Social support and adaptation of work and daily life activities are recommended.

Conclusions

Successful treatment of MG rests on timely combination of different interventions. Due to spontaneous disease fluctuations, comorbidities, and changes in life conditions, regular long‐term specialized follow‐up is needed. Most patients do reasonably well but there is room for further improvement. Novel treatments are promising, though subject to restricted access due to costs.

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Authors:
Nils Erik Gilhus, Henning Andersen, Linda Kahr Andersen, Marion Boldingh, Sini Laakso, Margret Oddny Leopoldsdottir, Sidsel Madsen, Fredrik Piehl, Trine Haug Popperud, Anna Rostedt Punga, Liselotte Schirakow and John Vissing
Article first published online:
06 Feb 2024 | DOI: 10.1111/ene.16229

ORIGINAL ARTICLE

Background and purpose

With the emergence of new treatment options for myasthenia gravis (MG), there is a need for information regarding epidemiology, healthcare utilization, and societal costs to support economic evaluation and identify eligible patients. We aimed to enhance the understanding of these factors using nationwide systematic registry data in Norway.

Methods

We received comprehensive national registry data from five Norwegian health‐ and work‐related registries. The annual incidence and prevalence were estimated for the period 2013–2021 using nationwide hospital and prescription data. The direct, indirect (productivity losses) and intangible costs (value of lost life‐years [LLY] and health‐related quality of life [HRQoL]) related to MG were estimated over a period of 1 year.

Results

In 2021, the incidence of MG ranged from 15 to 16 cases per year per million population depending on the registry used, while the prevalence varied between 208.9 and 210.3 per million population. The total annual societal costs of MG amounted to EUR 24,743 per patient, of which EUR 3592 (14.5%) were direct costs, EUR 8666 (35.0%) were productivity loss, and EUR 12,485 (50.5%) were lost value from LLY and reduced HRQoL.

Conclusion

The incidence and prevalence of MG are higher than previously estimated, and the total societal costs of MG are substantial. Our findings demonstrate that productivity losses, and the value of LLY and HRQoL constitute a considerable proportion of the total societal costs.

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Authors:
Ingrid Engebretsen, Nils Erik Gilhus, Ivar Sønbø Kristiansen, Erik Magnus Sæther, Ingrid Lindberg‐Schager, Fredrik Arneberg and Christoffer Bugge
Article first published online:
07 Feb 2024 | DOI: 10.1111/ene.16233

ORIGINAL ARTICLE

Background

A lack of consensus exists in linking demographic, behavioral, and cognitive characteristics to biological stages of dementia, defined by the ATN (amyloid, tau, neurodegeneration) classification incorporating amyloid, tau, and neuronal injury biomarkers.

Methods

Using a random forest classifier we investigated whether 27 demographic, behavioral, and cognitive characteristics allowed distinction between ATN‐defined groups with the same cognitive profile. This was done separately for three cognitively unimpaired (CU) (112 A‐T‐N‐; 46 A+T+N+/−; 65 A‐T+/‐N+/−) and three mild cognitive impairment (MCI) (128 A‐T‐N‐; 223 A+T+N+/−; 94 A‐T+/‐N+/−) subgroups.

Results

Classification‐balanced accuracy reached 39% for the CU and 52% for the MCI subgroups. Logical Delayed Recall (explaining 16% of the variance), followed by the Alzheimer's Disease Assessment Scale 13 (14%) and Everyday Cognition Informant (10%), were the most relevant characteristics for classification of the MCI subgroups. Race and ethnicity, marital status, and Everyday Cognition Patient were not relevant (0%).

Conclusions

The demographic, behavioral, and cognitive measures used in our model were not informative in differentiating ATN‐defined CU profiles. Measures of delayed memory, general cognition, and activities of daily living were the most informative in differentiating ATN‐defined MCI profiles; however, these measures alone were not sufficient to reach high classification performance.

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Authors:
Sofia Marcolini, Jaime D. Mondragón, Zeus T. Dominguez‐Vega, Peter P. De Deyn and Natasha M. Maurits
Article first published online:
27 Feb 2024 | DOI: 10.1111/ene.16235

ORIGINAL ARTICLE

Background and purpose

The complex aetiology of Alzheimer's disease suggests prevention potential. Risk scores have potential as risk stratification tools and surrogate outcomes in multimodal interventions targeting specific at‐risk populations. The Australian National University Alzheimer's Disease Risk Index (ANU‐ADRI) was tested in relation to cognition and its suitability as a surrogate outcome in a multidomain lifestyle randomized controlled trial, in older adults at risk of dementia.

Methods

In this post hoc analysis of the Finnish Intervention Study to Prevent Cognitive Impairment and Disability (FINGER), ANU‐ADRI was calculated at baseline, 12, and 24 months ( = 1174). The association between ANU‐ADRI and cognition (at baseline and over time), the intervention effect on changes in ANU‐ADRI, and the potential impact of baseline ANU‐ADRI on the intervention effect on changes in cognition were assessed using linear mixed models with maximum likelihood estimation.

Results

A higher ANU‐ADRI was significantly related to worse cognition, at baseline (e.g., estimate for global cognition [95% confidence interval] was −0.028 [−0.032 to −0.025]) and over the 2‐year study (e.g., estimate for 2‐year changes in ANU‐ADRI and per‐year changes in global cognition [95% confidence interval] was −0.068 [−0.026 to −0.108]). No significant beneficial intervention effect was reported for ANU‐ADRI, and baseline ANU‐ADRI did not significantly affect the response to the intervention on changes in cognition.

Conclusions

The ANU‐ADRI was effective for the risk prediction of cognitive decline. Risk scores may be crucial for the success of novel dementia prevention strategies, but their algorithm, the target population, and the intervention design should be carefully considered when choosing the appropriate tool for each context.

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Authors:
Anette Hall, Mariagnese Barbera, Jenni Lehtisalo, Riitta Antikainen, Hamidul Huque, Tiina Laatikainen, Tiia Ngandu, Hilkka Soininen, Ruth Stephen, Timo Strandberg, Miia Kivipelto, Kaarin J. Anstey and Alina Solomon
Article first published online:
07 Feb 2024 | DOI: 10.1111/ene.16238

ORIGINAL ARTICLE

Background and purpose

Hearing impairment is common following aneurysmal subarachnoid haemorrhage (aSAH). Previous studies have demonstrated that auditory processing disorder (APD) is the primary underlying pathology. Assistive listening devices (ALDs) can be used to manage APD but have not been explored in aSAH. The aim of this study was to assess the benefit of an ALD for patients reporting hearing difficulty after aSAH.

Methods

This was a prospective pilot single‐arm intervention study of an ALD for APD following aSAH. Patients who reported subjective hearing difficulty following aSAH were identified from the Wessex Neurological Centre aSAH database. Speech‐in‐noise was evaluated using the Bamford−Kowal−Bench (BKB) test under 60 and 65 dB noise conditions. BKB performance was compared with and without an ALD. Cognition was assessed using the Addenbrooke's Cognitive Examination‐III.

Results

Fourteen aSAH patients with self‐reported hearing loss were included in the analysis. Under both noise conditions the ALD significantly improved BKB performance (60 dB,  = −3.30,  < 0.001; 65 dB,  = −3.33,  < 0.001). There was no relationship between cognition and response to the ALD.

Conclusions

This study demonstrates the marked benefit of ALDs to manage APD following aSAH, regardless of cognitive status. This finding has implications for the management of this common yet disabling deficit which impacts quality of life and employment. A further trial of ALDs in this patient group is needed to test whether these large, short‐term benefits can be practically translated to the community for long‐term benefit when used at home.

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Authors:
Ben Gaastra, Stuart Whyte, Bethan Hankin, Diederik Bulters, Ian Galea and Nicole Campbell
Article first published online:
08 Feb 2024 | DOI: 10.1111/ene.16240

ORIGINAL ARTICLE

Background and purpose

Diagnosis of lymphoma involving the central nervous system (CNS) is challenging. This study aimed to explore the abnormal vestibular and ocular motor findings in CNS lymphoma.

Methods

A retrospective search of the medical records identified 30 patients with CNS lymphoma presenting ocular motor and vestibular abnormalities from four neurology clinics of university hospitals in South Korea (22 men, age range 14–81 years, mean 60.6 ± 15.2). The demographic and clinical features and the results of laboratory, radiological and pathological evaluation were analyzed.

Results

Patients presented with diplopia (13/30, 43%), vestibular symptoms (15/30, 50%) or both (2/30, 7%). In 15 patients with diplopia, abnormal ocular motor findings included ocular motor nerve palsy ( = 10, 67%), internuclear ophthalmoplegia ( = 2, 13%), external ophthalmoplegia ( = 2, 13%) and exophoria ( = 1, 7%). The vestibular abnormalities were isolated in 14 (82%) of 17 patients with vestibular symptoms and included combined unilateral peripheral and central vestibulopathy in three from lesions involving the vestibular nuclei. CNS lymphoma involved the brainstem (53%), cerebellum (33%), leptomeninges (30%), deep gray nuclei (23%) or cranial nerves (17%). Two patients showed the “double‐panda” sign by involving the midbrain.

Conclusions

This study expands the clinical and radiological spectra of CNS lymphoma. Neuro‐ophthalmological and neuro‐otological evaluation may guide the early diagnosis of CNS lymphoma.

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Authors:
Suk‐Min Lee, Seo Young Choi, Jae‐Hwan Choi, Eun Hye Oh, Dallah Yoo, Sun‐Uk Lee, Hyo Jung Kim, Jeong‐Yoon Choi, Ji‐Soo Kim and Kwang‐Dong Choi
Article first published online:
12 Feb 2024 | DOI: 10.1111/ene.16242

ORIGINAL ARTICLE

Background and purpose

The conceptualization of brain death (BD) was pivotal in the shaping of judicial and medical practices. Nonetheless, media reports of alleged recovery from BD reinforced the criticism that this construct is a self‐fulfilling prophecy (by treatment withdrawal or organ donation). We meta‐analyzed the natural history of BD when somatic support (SS) is maintained.

Methods

Publications on BD were eligible if the following were reported: aggregated data on its natural history with SS; and patient‐level data that allowed censoring at the time of treatment withdrawal or organ donation. Endpoints were as follows: rate of somatic expiration after BD with SS; BD misdiagnosis, including “functionally brain‐dead” patients (FBD; i.e. after the pronouncement of brain‐death, ≥1 findings were incongruent with guidelines for its diagnosis, albeit the lethal prognosis was not altered); and length and predictors of somatic survival.

Results

Forty‐seven articles were selected (1610 patients, years: 1969–2021). In BD patients with SS, median age was 32.9 years (range = newborn–85 years). Somatic expiration followed BD in 99.9% (95% confidence interval = 89.8–100). Mean somatic survival was 8.0 days (range = 1.6 h–19.5 years). Only age at BD diagnosis was an independent predictor of somatic survival length (coefficient = −11.8, SE = 4,  < 0.01). Nine BD misdiagnoses were detected; eight were FBD, and one newborn fully recovered. No patient ever recovered from chronic BD (≥1 week somatic survival).

Conclusions

BD diagnosis is reliable. Diagnostic criteria should be fine‐tuned to avoid the small incidence of misdiagnosis, which nonetheless does not alter the prognosis of FBD patients. Age at BD diagnosis is inversely proportional to somatic survival.

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Authors:
Ivancarmine Gambardella, Francesco Nappi, Berhane Worku, Robert F. Tranbaugh, Aminat M. Ibrahim, Sandhya K. Balaram and James L. Bernat
Article first published online:
20 Feb 2024 | DOI: 10.1111/ene.16243

LETTER TO THE EDITOR

Serum neurofilament light chain and small fiber neuropathy: Every cloud has a silver lining

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Authors:
Domenico Plantone and Guido Primiano
Article first published online:
12 Feb 2024 | DOI: 10.1111/ene.16244

LETTER TO THE EDITOR

Reply to “Serum neurofilament light chain and small fiber neuropathy: Every cloud has a silver lining” by D. Plantone and G. Primiano

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Authors:
Panoraia Baka, Frank Birklein, Stefan Bittner and Claudia Sommer
Article first published online:
20 Feb 2024 | DOI: 10.1111/ene.16245

ORIGINAL ARTICLE

Background

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) may cause ischaemic stroke and intracranial haemorrhage. The aim of our study was to assess the frequency of the afore‐mentioned outcomes.

Methods

We performed a PROSPERO‐registered (CRD42022355704) systematic review and meta‐analysis accessing PubMed until 7 November 2022. The inclusion criteria were: (1) original publication, (2) adult patients (≥18 years), (3) enrolling patients with PRES and/or RCVS, (4) English language and (5) outcome information. Outcomes were frequency of (1) ischaemic stroke and (2) intracranial haemorrhage, divided into subarachnoid haemorrhage (SAH) and intraparenchymal haemorrhage (IPH). The Cochrane Risk of Bias tool was used.

Results

We identified 848 studies and included 48 relevant studies after reviewing titles, abstracts and full text. We found 11 studies on RCVS (unselected patients), reporting on 2746 patients. Among the patients analysed, 15.9% (95% CI 9.6%–23.4%) had ischaemic stroke and 22.1% (95% CI 10%–39.6%) had intracranial haemorrhage. A further 20.3% (95% CI 11.2%–31.2%) had SAH and 6.7% (95% CI 3.6%–10.7%) had IPH. Furthermore, we found 28 studies on PRES (unselected patients), reporting on 1385 patients. Among the patients analysed, 11.2% (95% CI 7.9%–15%) had ischaemic stroke and 16.1% (95% CI 12.3%–20.3%) had intracranial haemorrhage. Further, 7% (95% CI 4.7%–9.9%) had SAH and 9.7% (95% CI 5.4%–15%) had IPH.

Conclusions

Intracranial haemorrhage and ischaemic stroke are common outcomes in PRES and RCVS. The frequency reported in the individual studies varied considerably.

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Authors:
Jana Kaufmann, Philipp Buecke, Thomas Meinel, Morin Beyeler, Adrian Scutelnic, Johannes Kaesmacher, Adnan Mujanović, Thomas Dobrocky, Hakim Arsany, Nils Peters, Werner Z'Graggen, Simon Jung and David Seiffge
Article first published online:
12 Mar 2024 | DOI: 10.1111/ene.16246

SHORT COMMUNICATION

Background

Though headache is commonly observed after stroke and may affect survivors' quality of life, it has rarely been studied after spontaneous intracerebral haemorrhage (ICH). In a cohort of ICH survivors, we assessed the long‐term prevalence and determinants of headache.

Methods

We screened consecutive ICH survivors enrolled in the prospective, single‐centre Prognosis of Intracerebral Haemorrhage study for headache 1, 3, and 6 years after ICH, according to the International Headache Society's criteria. Depressive and anxiety symptoms severity was measured at 1‐year follow‐up. Variables associated with the presence of headache 1 year after ICH were analyzed using univariate and multivariable models.

Results

Among the 146 patients included in this study, 31 (21%), 25 (19%), and 14 (20%) patients reported headache at 1‐, 3‐, and 6‐year follow‐up, respectively. In an age‐adjusted model, patients with headache at ICH onset (adjusted odds ratio [aOR] 2.75; 95% CI 1.02–7.42) and previous history of headache (aOR 4.60; 95% CI 1.74–12.1) were associated with headache at 1‐year follow‐up. Patients with headache were more likely to report depressive and anxiety symptoms at 1‐year follow‐up (both  < 0.02).

Conclusions

One in five ICH survivors suffered from headache and patients who reported headache at ICH onset were especially at risk.

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Authors:
Ugur Gurol, Giuseppe Scopelliti, Charlotte Cordonnier and Laurent Puy
Article first published online:
13 Feb 2024 | DOI: 10.1111/ene.16247

ORIGINAL ARTICLE

Background and purpose

This study aimed to assess the diagnostic criteria, ancillary investigations and treatment response using real‐life data in multifocal motor neuropathy (MMN) patients.

Methods

Clinical and laboratory data were collected from 110 patients enrolled in the Italian MMN database through a structured questionnaire. Twenty‐six patients were excluded due to the unavailability of nerve conduction studies or the presence of clinical signs and symptoms and electrodiagnostic abnormalities inconsistent with the MMN diagnosis. Analyses were conducted on 73 patients with a confirmed MMN diagnosis and 11 patients who did not meet the diagnostic criteria.

Results

The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria were variably applied. AUTHOR:When applying the American Association of Electrodiagnostic Medicine criteria, an additional 17% of patients fulfilled the criteria for probable/definite diagnosis whilst a further 9.5% missed the diagnosis. In 17% of the patients only compound muscle action potential amplitude, but not area, was measured and subsequently recorded in the database by the treating physician. Additional investigations, including anti‐GM1 immunoglobulin M antibodies, cerebrospinal fluid analysis, nerve ultrasound and magnetic resonance imaging, supported the diagnosis in 46%–83% of the patients. Anti‐GM1 immunoglobulin M antibodies and nerve ultrasound demonstrated the highest sensitivity. Additional tests were frequently performed outside the EFNS/PNS guideline recommendations.

Conclusions

This study provides insights into the real‐world diagnostic and management strategies for MMN, highlighting the challenges in applying diagnostic criteria.

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Authors:
Pietro Emiliano Doneddu, Luca Gentile, Dario Cocito, Raffaella Fazio, Marco Luigetti, Chiara Briani, Massimiliano Filosto, Gabriele Siciliano, Luana Benedetti, Giovanni Antonini, Sabrina Matà, Girolama Alessandra Marfia, Maurizio Inghilleri, Fiore Manganelli, Giuseppe Cosentino, Filippo Brighina, Marinella Carpo, Francesca Carta, Anna Mazzeo, Erdita Peci, Camilla Strano, Angela Romano, Marta Campagnolo, Stefano Cotti‐Piccinelli, Divina Valeria Viola, Francesco Germano, Luca Leonardi, Martina Sperti, Giorgia Mataluni, Marco Ceccanti, Emanuele Spina, Elisa Vegezzi, Vincenzo Di Stefano and Eduardo Nobile‐Orazio
Article first published online:
20 Feb 2024 | DOI: 10.1111/ene.16248

ORIGINAL ARTICLE

Background and purpose

The aim of this study was to determine the prevalence of anti‐myelin‐associated glycoprotein (MAG) neuropathy and the current status of such patients in Japan.

Methods

We conducted a nationwide survey in 2021 using established epidemiological methods. Questionnaires were sent to all neurology and pediatric neurology departments throughout Japan to identify patients with anti‐MAG neuropathy. An initial questionnaire was used to determine the number of patients, with a second one used to collect detailed clinical information.

Results

The estimated number of patients with anti‐MAG neuropathy was 353, with a prevalence of 0.28 per 100,000 and an incidence of 0.05 per 100,000. The detailed clinical profiles of 133 patients were available. The median (range) age of onset was 67 (30–87) years, with a prominent peak in the age range 66–70 years, and the male‐to‐female ratio was 3.6. Most patients had distal sensory‐predominant polyneuropathy, and neuropathic pain (50%), or sensory ataxia (42%), while 18% had Waldenström's macroglobulinemia or multiple myeloma. Intravenous immunoglobulin was the most frequently used treatment (65%), but the response rate was <50%, whereas rituximab was given in 32% of patients, and 64% of these showed improvement. At the last visit, 27% of patients could not walk independently.

Conclusions

This study on anti‐MAG neuropathy provides updated insights into the epidemiology of this disease, clinical profiles, and treatment approaches in Japan. Rituximab therapy, used for only one‐third of the patients, demonstrated efficacy. During the final visit, a quarter of the patients were unable to walk independently. Further studies are warranted to determine the optimal management of this rare and intractable disorder.

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Authors:
Yuya Aotsuka, Sonoko Misawa, Tomoki Suichi, Kazumoto Shibuya, Keigo Nakamura, Hiroki Kano, Ryo Otani, Marie Morooka, Moeko Ogushi, Kengo Nagashima, Yasunori Sato, Nagato Kuriyama and Satoshi Kuwabara
Article first published online:
20 Feb 2024 | DOI: 10.1111/ene.16249

ORIGINAL ARTICLE

Background and purpose

Timely prehospital stroke recognition was explored in the Paramedic Norwegian Acute Stroke Prehospital Project (ParaNASPP) by implementation of stroke education for paramedics and use of the National Institutes of Health Stroke Scale (NIHSS) through a mobile application. The study tested triage and facilitated communication between paramedics and stroke physicians. To complement the quantitative results of the clinical trial, a qualitative approach was used to identify factors that influence triage decisions and diagnostic accuracy in prehospital stroke recognition experienced by paramedics and stroke physicians.

Method

Semi‐structured qualitative individual interviews were performed following an interview guide. Informants were recruited from the enrolled paramedics and stroke physicians who participated in the ParaNASPP trial from Oslo University Hospital. Interviews were audio recorded, transcribed verbatim and approached inductively using the principles of thematic analysis.

Results

Fourteen interviews were conducted, with seven paramedics and seven stroke physicians. Across both groups two overarching themes were identified related to triage decisions and diagnostic accuracy in prehospital stroke recognition: prehospital NIHSS reliably improves clinical assessment and communication quality; overtriage is widely accepted whilst undertriage is not.

Conclusion

Paramedics and stroke physicians described how prehospital NIHSS improved communication quality and reliably improved prehospital clinical assessment. The qualitative results support a rationale of an application algorithm to decide which NIHSS items should prompt immediate prenotification rather than a complete NIHSS as default.

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Authors:
Mona Guterud, Camilla Hardeland, Helge Fagerheim Bugge, Else Charlotte Sandset, Edel Jannecke Svendsen and Maren Ranhoff Hov
Article first published online:
25 Feb 2024 | DOI: 10.1111/ene.16252

ORIGINAL ARTICLE

Background and purpose

Pediatric‐onset multiple sclerosis (PoMS) is associated with high health care use. To plan resource allocation for this patient group, knowledge of the incidence rate and prevalence is important. However, such studies are scarce, few are population‐based, and the methodology varies widely. We aimed to address this knowledge gap by performing a nationwide study of the incidence rate and prevalence of PoMS in Sweden, an area of high multiple sclerosis (MS) incidence and prevalence.

Methods

MS cases were identified by linking two nationwide registers, the National Patient Register and the Swedish MS Registry. MS cases having their first central nervous system demyelinating event or MS clinical onset before age 18 years were classified as pediatric onset. Incidence rate and prevalence were estimated annually over the study period (2006–2016) for the total population and stratified by sex and age group (<12, 12–15, and 16–17 years). Temporal trends and ratios between sexes and age groups were estimated.

Results

We identified 238 incident cases from 2006 to 2016, corresponding to an overall crude incidence rate of 1.12 per 100,000 person‐years and an overall crude prevalence of 2.82 per 100,000 population. There was a higher incidence rate among females and the highest age category. The overall incidence rate and prevalence estimates remained stable during the study period.

Conclusions

Sweden exhibits a consistently high incidence rate and prevalence of PoMS that has remained stable over time. This knowledge serves as a tool to aid in planning resource allocation and health services for this patient population.

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Authors:
Fredrik Sandesjö, Helen Tremlett, Katharina Fink, Ruth Ann Marrie, Feng Zhu, Ronny Wickström and Kyla A. McKay
Article first published online:
18 Feb 2024 | DOI: 10.1111/ene.16253