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Article first published online:
21 Jul 2021 | DOI: 10.1111/ene.14347

ORIGINAL ARTICLE

Evaluation of the 2020 European Academy of Neurology virtual congress: transition from a face‐to‐face to a virtual meeting

Background and purpose

Due to the COVID‐19 pandemic, scientific congresses are increasingly being organized as virtual congresses (VCs). In May 2020, the European Academy of Neurology (EAN) held a VC, free of charge. In the absence of systematic studies on this topic, the aim of this study is to evaluate the attendance and perceived quality of the 2020 EAN VC compared to the 2019 EAN face‐to‐face congress (FFC).

Methods

An analysis of the demographic data of participants obtained from the online registration was done. A comparison of the two congresses based on a survey with questions on the perception of speakers’ performance, quality of networking and other aspects was made.

Results

Of 43,596 registered participants, 20,694 active participants attended the VC. Compared to 2019, the number of participants tripled (6916 in 2019) and the cumulated number of participants attending the sessions was five times higher (169,334 in 2020 vs. 33,024 in 2019). Out of active participants 55% were from outside Europe, 42% were board‐certified neurologists (FFC 80%) and 21% were students (FFC 0.6%). The content of the congress was evaluated as ‘above expectation’ by 56% of the attendees (FFC 41%). Of the respondents who had been exposed to earlier EAN congresses 73% preferred the FFC compared to the VC (17%).

Conclusion

The VC fulfilled the main mission of organizing high quality EAN congresses despite the restrictions of the impersonal format. The geographical distribution of the participants proves the expected higher inclusivity of a VC. The large participation of students and neurologists in training opens new educational potentials for the EAN.

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Authors:
Maria Stamelou, Walter Struhal, Olle ten Cate, Magdalena Matczak, Süleyman Ayhan Çalışkan, Riccardo Soffietti, Anthony Marson, Panagiotis Zis, Francesco di Lorenzo, Anja Sander, Günther Deuschl, Marianne de Visser and Claudio L. A. Bassetti

Article first published online:
11 Jun 2021 | DOI: 10.1111/ene.14702

POSITION PAPER

Ozone‐induced encephalopathy: A novel iatrogenic entity

Background and purpose

Ozone‐based treatments can be associated with central nervous system toxicity, which we have termed ozone‐induced encephalopathy (OIE). A detailed description of its phenotype is lacking.

Methods

Three cases with findings suggestive of OIE are presented, and the literature is reviewed.

Results

Case 1 is a healthy 59‐year‐old man presenting with loss of consciousness, cortical blindness, restlessness, and anterograde amnesia immediately following a cervical ozone‐therapy (OT) session for chronic neck pain. Brain magnetic resonance imaging (MRI) on admission was normal. A follow‐up scan demonstrated a subtle increased T2 fluid‐attenuated inversion recovery signal within the left cerebellum; an echocardiography showed a patent foramen ovale (PFO). Case 2 is a 56‐year‐old woman with history of migraine, PFO, and lumbar pain who presented with headache, bilateral visual impairment, motor dysphasia, and agitation. All her symptoms began immediately after lumbar OT. Her brain MRI was negative. Case 3 is a healthy 27‐year‐old man who complained of vertigo and mild blurred vision 5 min following a cervical ozone injection. His neurological examination and brain MRI were normal. All three patients had full recovery within 48 h. We found eight additional cases of OIE in the literature.

Conclusions

OIE should be considered in patients presenting with neurological symptoms in close relation to OT. OIE is likely a novel iatrogenic entity with a complex pathogenesis; it is probably underreported because it mimics other neurological conditions.

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Authors:
Shalom Haggiag, Luca Prosperini, Alessandro Stasolla, Carmela Gerace, Carla Tortorella and Claudio Gasperini

Article first published online:
19 Mar 2021 | DOI: 10.1111/ene.14793

CORRIGENDUM

Corrigendum

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Article first published online:
21 Jul 2021 | DOI: 10.1111/ene.14825

ORIGINAL ARTICLE

Scoring the 10‐year risk of ambulatory disability in multiple sclerosis: the RoAD score

Background and purpose

Both baseline prognostic factors and short‐term predictors of treatment response can influence the long‐term risk of disability accumulation in patients with relapsing–remitting multiple sclerosis (RRMS). The objective was to develop and validate a scoring system combining baseline prognostic factors and 1‐year variables of treatment response into a single numeric score predicting the long‐term risk of disability.

Methods

We analysed two independent datasets of patients with RRMS who started interferon beta or glatiramer acetate, had an Expanded Disability Status Scale (EDSS) score <4.0 at treatment start and were followed for at least 10 years. The first dataset (‘training set’) included patients attending three MS centres in Italy and served as a framework to create the so‐called RoAD score (Risk of Ambulatory Disability). The second (‘validation set’) included a cohort of patients followed in Barcelona, Spain, to explore the performance of the RoAD score in predicting the risk of reaching an EDSS score ≥6.0.

Results

The RoAD score (ranging from 0 to 8) derived from the training set ( = 1225), was based on demographic (age), clinical baseline prognostic factors (disease duration, EDSS) and 1‐year predictors of treatment response (number of relapses, presence of gadolinium enhancement and new T2 lesions). The best cut‐off score for discriminating patients at higher risk of reaching the disability milestone was ≥4. When applied to the validation set ( = 296), patients with a RoAD score ≥4 had an approximately 4‐fold increased risk for reaching the disability milestone ( < 0.001).

Discussion

The RoAD score is proposed as an useful tool to predict individual prognosis and optimize treatment strategy of patients with RRMS.

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Authors:
Claudio Gasperini, Luca Prosperini, Àlex Rovira, Mar Tintoré, Jaume Sastre‐Garriga, Carla Tortorella, Shalom Haggiag, Simonetta Galgani, Ruggero Capra, Carlo Pozzilli, Xavier Montalban and Jordi Río

Article first published online:
19 Apr 2021 | DOI: 10.1111/ene.14845

ORIGINAL ARTICLE

Cervical spinal degenerative disease in multiple sclerosis

Background and purpose

Root and cord irritation from cervical spinal degenerative disease (SDD) may share clinical features with progressive multiple sclerosis (MS), so diagnostic overshadowing may occur. We hypothesized that cervical stenotic SDD is commoner in people with progressive MS, compared to controls.

Methods

A retrospective case–control study of 111 cases (56 with progressive MS and 55 age‐ and sex‐matched controls) was conducted. Five types of cervical SDD (disc degeneration, posterior disc protrusion, endplate changes, canal stenosis and foraminal stenosis) were assessed objectively on magnetic resonance imaging using published scales. Multivariable regression analysis was performed.

Results

Moderate‐to‐severe cervical spinal degeneration occurred more frequently in progressive MS, compared to controls. In multivariable regression, foraminal stenosis was three times more likely in progressive MS (odds ratio 3.20, 95% confidence interval 1.27, 8.09; = 0.014), and was more severe ( = 0.009). This finding was confirmed on retrospective evaluation of clinical radiology reports in the same population. Foraminal stenosis was twice as likely in progressive MS, compared to relapsing‐remitting MS.

Conclusions

People with progressive MS are susceptible to foraminal stenosis. A higher index of suspicion for cervical SDD is required when appropriate neurological symptoms occur in the setting of progressive MS, to guide appropriate treatment or monitoring.

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Authors:
Simran Chhugani, Nivedita Agarwal, Faraz Sheikh, Florina Borca, Aginor Spanoulis and Ian Galea

Article first published online:
05 May 2021 | DOI: 10.1111/ene.14855

ORIGINAL ARTICLE

A novel prognostic score to assess the risk of progression in relapsing−remitting multiple sclerosis patients

Background

At the patient level, the prognostic value of several features that are known to be associated with an increased risk of converting from relapsing−remitting (RR) to secondary phase (SP) multiple sclerosis (MS) remains limited.

Methods

Among 262 RRMS patients followed up for 10 years, we assessed the probability of developing the SP course based on clinical and conventional and non‐conventional magnetic resonance imaging (MRI) parameters at diagnosis and after 2 years. We used a machine learning method, the random survival forests, to identify, according to their minimal depth (MD), the most predictive factors associated with the risk of SP conversion, which were then combined to compute the secondary progressive risk score (SP‐RiSc).

Results

During the observation period, 69 (26%) patients converted to SPMS. The number of cortical lesions (MD = 2.47) and age (MD = 3.30) at diagnosis, the global cortical thinning (MD = 1.65), the cerebellar cortical volume loss (MD = 2.15) and the cortical lesion load increase (MD = 3.15) over the first 2 years exerted the greatest predictive effect. Three patients’ risk groups were identified; in the high‐risk group, 85% (46/55) of patients entered the SP phase in 7 median years. The SP‐RiSc optimal cut‐off estimated was 17.7 showing specificity and sensitivity of 87% and 92%, respectively, and overall accuracy of 88%.

Conclusions

The SP‐RiSc yielded a high performance in identifying MS patients with high probability to develop SPMS, which can help improve management strategies. These findings are the premise of further larger prospective studies to assess its use in clinical settings.

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Authors:
Anna Isabella Pisani, Antonio Scalfari, Francesco Crescenzo, Chiara Romualdi and Massimiliano Calabrese

Article first published online:
05 May 2021 | DOI: 10.1111/ene.14859

SHORT COMMUNICATION

A next generation sequencing‐based analysis of a large cohort of ataxic patients refines the clinical spectrum associated with spinocerebellar ataxia 21

Background and purpose

Spinocerebellar ataxia 21 (SCA21) is a rare autosomal dominant neurodegenerative disorder caused by gene mutations. To date, SCA21 has been reported only in a limited number of families worldwide. Here, we describe clinical and molecular findings in five additional SCA21 patients from four unrelated families, diagnosed through a multicentre next generation sequencing‐based molecular screening project on a large cohort of patients with degenerative and congenital ataxias.

Methods

A cohort of 393 patients with ataxia of unknown aetiology was selected. Following the identification of heterozygous pathogenic variants using a target resequencing panel, we carried out an in‐depth phenotyping of the novel SCA21 patients.

Results

Five patients from four unrelated families, three of Italian and one of Libyan origin, were identified. These patients were carriers of previously reported mutations. Clinically, our SCA21 cohort includes both adult onset, slowly progressive cerebellar ataxias associated with cognitive impairment resembling cerebellar cognitive affective syndrome and early onset forms associated with cognitive delay, neuropsychiatric features, or evidence of hypomyelination on brain magnetic resonance imaging. None of our patients exhibited signs of extrapyramidal involvement. The so‐called “recurrent” c.509C>T (p.Pro170Leu) mutation was detected in two of four families, corroborating its role as a hot spot.

Conclusions

Our results confirm that SCA21 is present also in Italy, suggesting that it might not be as rare as previously thought. The phenotype of these novel SCA21 patients indicates that slowly progressive cerebellar ataxia, and cognitive and psychiatric symptoms are the most typical clinical features associated with mutations in the gene.

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Authors:
Vittorio Riso, Daniele Galatolo, Melissa Barghigiani, Serena Galosi, Alessandra Tessa, Ivana Ricca, Salvatore Rossi, Caterina Caputi, Ettore Cioffi, Vincenzo Leuzzi, Carlo Casali, Filippo M. Santorelli and Gabriella Silvestri

Article first published online:
27 May 2021 | DOI: 10.1111/ene.14868

ORIGINAL ARTICLE

Assessment of the genetic contribution to brain magnetic resonance imaging lesion load and atrophy measures in multiple sclerosis patients

Background and purpose

Multiple sclerosis (MS) susceptibility is influenced by genetics; however, little is known about genetic determinants of disease expression. We aimed at assessing genetic factors influencing quantitative neuroimaging measures in two cohorts of progressive MS (PMS) and relapsing–remitting MS (RRMS) patients.

Methods

Ninety‐nine PMS and 214 RRMS patients underwent a 3‐T brain magnetic resonance imaging (MRI) scan, with the measurement of five MRI metrics including T2 lesion volumes and measures of white matter, grey matter, deep grey matter, and hippocampal volumes. A candidate pathway strategy was adopted; gene set analysis was carried out to estimate cumulative contribution of genes to MRI phenotypes, adjusting for relevant confounders, followed by single nucleotide polymorphism (SNP) regression analysis.

Results

Seventeen Kyoto Encyclopedia of Genes and Genomes pathways and 42 Gene Ontology (GO) terms were tested. We additionally included in the analysis genes with enriched expression in brain cells. Gene set analysis revealed a differential pattern of association across the two cohorts, with processes related to sodium homeostasis being associated with grey matter volume in PMS ( = 0.002), whereas inflammatory‐related GO terms such as adaptive immune response and regulation of inflammatory response appeared to be associated with T2 lesion volume in RRMS ( = 0.004 and = 0.008, respectively). As for SNPs, the rs7104613 mapping to gene was associated with reduced deep grey matter volume (β = −0.731, = 3.2*10) in PMS, whereas we found evidence of association between white matter volume and rs740948 mapping to gene (β = 22.04, = 5.5*10) in RRMS.

Conclusions

Our data suggest a different pattern of associations between MRI metrics and functional processes across MS disease courses, suggesting different phenomena implicated in MS.

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Authors:
Ferdinando Clarelli, Maria Assunta Rocca, Silvia Santoro, Ermelinda De Meo, Laura Ferrè, Melissa Sorosina, Filippo Martinelli Boneschi, Federica Esposito and Massimo Filippi

Article first published online:
08 Jun 2021 | DOI: 10.1111/ene.14872

ORIGINAL ARTICLE

Ultraearly thrombolysis by an anesthesiologist in a mobile stroke unit: A prospective, controlled intervention study

Background

Acute stroke treatment in mobile stroke units (MSU) is feasible and reduces time‐to‐treatment, but the optimal staffing model is unknown. We wanted to explore if integrating thrombolysis of acute ischemic stroke (AIS) in an anesthesiologist‐based emergency medical services (EMS) reduces time‐to‐treatment and is safe.

Methods

A nonrandomized, prospective, controlled intervention study. Inclusion criteria: age ≥18 years, nonpregnant, stroke symptoms with onset ≤4 h. The MSU staffing is inspired by the Norwegian Helicopter Emergency Medical Services crew with an anesthesiologist, a paramedic‐nurse and a paramedic. Controls were included by conventional ambulances in the same catchment area. Primary outcome was onset‐to‐treatment time. Secondary outcomes were alarm‐to‐treatment time, thrombolytic rate and functional outcome. Safety outcomes were symptomatic intracranial hemorrhage and mortality.

Results

We included 440 patients. MSU median (IQR) onset‐to‐treatment time was 101 (71–155) minutes versus 118 (90–176) minutes in controls, = 0.007. MSU median (IQR) alarm‐to‐treatment time was 53 (44–65) minutes versus 74 (63–95) minutes in controls, < 0.001. Golden hour treatment was achieved in 15.2% of the MSU patients versus 3.7% in the controls, = 0.005. The thrombolytic rate was higher in the MSU (81% vs 59%, = 0.001). MSU patients were more often discharged home (adjusted OR [95% CI]: 2.36 [1.11–5.03]). There were no other significant differences in outcomes.

Conclusions

Integrating thrombolysis of AIS in the anesthesiologist‐based EMS reduces time‐to‐treatment without negatively affecting outcomes. An MSU based on the EMS enables prehospital assessment of acute stroke in addition to other medical and traumatic emergencies and may facilitate future implementation.

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Authors:
Karianne Larsen, Henriette S. Jæger, Lars H. Tveit, Maren R. Hov, Kjetil Thorsen, Jo Røislien, Volker Solyga, Christian G. Lund and Kristi G. Bache

Article first published online:
24 May 2021 | DOI: 10.1111/ene.14877

ORIGINAL ARTICLE

Causal associations of serum matrix metalloproteinase‐8 level with ischaemic stroke and ischaemic stroke subtypes: a Mendelian randomization study

Background and purpose

Elevated serum matrix metalloproteinase‐8 (MMP‐8) concentrations are associated with high risk of vascular disease, but the causality remains unclear. A two‐sample Mendelian randomization (MR) study was performed to examine the causal effect of serum MMP‐8 concentrations on the risk of ischaemic stroke, ischaemic stroke subtypes and coronary artery disease.

Methods

Ten independent single‐nucleotide polymorphisms related to serum MMP‐8 concentrations were identified as instrumental variables from a genome‐wide association study of 6049 European subjects. Genetic association estimates for ischaemic stroke were obtained from the Multiancestry Genome‐wide Association Study of Stroke consortium with 446,696 European individuals. The inverse‐variance weighted method was applied to assess the causal associations of serum MMP‐8 with ischaemic stroke and its subtypes in the main analysis.

Results

No significant causal association was observed for MMP‐8 levels with total ischaemic stroke, large artery stroke or cardioembolic stroke. Genetically determined 1 – unit higher log‐transformed serum MMP‐8 concentration was associated with an increased risk of small vessel stroke (odds ratio 1.25; 95% confidence interval 1.12–1.39; < 0.001). In secondary analysis, a similar adverse impact was reported for MMP‐8 on coronary artery disease (odds ratio 1.05; 95% confidence interval 1.01–1.10; = 0.017). Sensitivity analyses further confirmed the relationship between serum MMP‐8 level and small vessel stroke and coronary artery disease. Mendelian randomization Egger regression showed no evidence of pleiotropic bias.

Conclusions

High serum MMP‐8 concentrations were causally associated with increased risks of small vessel stroke and coronary artery disease. The mechanism underlying the effect of serum MMP‐8 on the vascular system requires further investigation.

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Authors:
Yiming Jia, Daoxia Guo, Kaixin Zhang, Pinni Yang, Yuhan Zang, Lulu Sun, Yu Wang, Fanghua Liu, Mengyao Shi, Yonghong Zhang and Zhengbao Zhu

Article first published online:
14 May 2021 | DOI: 10.1111/ene.14878

ORIGINAL ARTICLE

Association between abnormal body weight and stroke outcome: A meta‐analysis and systematic review

Background and purpose

To test the hypothesis that “obesity paradox” exists in stroke patients, we conducted a meta‐analysis and systematic review on the association between abnormal body weight (obesity, overweight, or underweight) and the outcome of different types of stroke.

Methods

This meta‐analysis and systematic review was performed in conformity to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta‐Analysis) guidelines in Appendix S2. Studies investigating the association between abnormal body weight and the outcome of different types of stroke were searched for in the PubMed and Embase databases from their inception to 20 March 2021.

Results

Thirty‐three articles including 84,660 patients were included in this study. Obesity and overweight were associated with longer survival in mixed‐stroke patients (acute ischemic stroke [AIS] combined with one or more other stroke subtypes) than was normal weight, whereas underweight was related to shorter survival; the pooled hazard ratios (HRs) of mortality were 0.77 (95% confidence interval [CI] = 0.71–0.83) for obesity, 0.76 (95% CI = 0.72–0.80) for overweight, and 1.71 (95% CI = 1.56–1.87) for underweight. However, only obesity was associated with longer survival in AIS patients compared with normal weight, and underweight was related to shorter survival; the pooled HR of mortality was 0.75 (95% CI = 0.64–0.88) for obesity and 1.53 (95% CI = 1.27–1.85) for underweight. After merging mixed‐stroke and AIS patients, we obtained similar results as in mixed‐stroke patients.

Conclusions

Our results suggested that in patients with mixed stroke or AIS, obesity was associated with a longer survival time than normal weight, whereas underweight was associated with a shorter survival time.

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Authors:
Peng Zhang, Xiu‐Li Yan, Yang Qu, Zhen‐Ni Guo and Yi Yang

Article first published online:
19 Jun 2021 | DOI: 10.1111/ene.14881

ORIGINAL ARTICLE

Physical and cognitive profiles in motoric cognitive risk syndrome in an older population from Southern Italy

Background and purpose

In older age, physical and cognitive declines have been shown to occur simultaneously or consequent to one another, and several operational definitions have been proposed to consider the co‐presence of the two declines; for example, “Motoric cognitive risk syndrome” (MCR) has been proposed as a definition for the coexistence of slow gait plus subjective cognitive complaints. Given the increasing interest in MCR and its potential role as both biomarker and therapeutic target, we aimed to estimate its prevalence in a large cohort of non‐demented older subjects, and to examine the associations between physical status, global cognitive dysfunction, and impairment in various cognitive domains in MCR.

Methods

A population‐based sample of 1041 older people in Southern Italy (mean age 75.15 years) was enrolled. We defined MCR using slowness and a single question for subjective cognitive complaints. We also administered a comprehensive neuropsychological test battery, together with tests assessing physical function.

Results

The prevalence of MCR was 9.9% (95% confidence interval 8.2–11.9). MCR was associated with decreased processing speed and executive function after adjusting for all relevant confounders. However, we found no significant association of MCR with decreased global cognition and immediate/delayed free recall of verbal memory. MCR was also associated with increased exhaustion, low muscle strength, and low physical activity, and increased levels of C‐reactive protein and interleukin‐6.

Conclusions

The present findings on MCR prevalence and associated cognitive and physical domains and inflammatory biomarkers may help to uncover altered pathways and therapeutic targets for intervention during the long preclinical phase of neurodegenerative dementia.

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Authors:
Ilaria Bortone, Chiara Griseta, Petronilla Battista, Fabio Castellana, Luisa Lampignano, Roberta Zupo, Giancarlo Sborgia, Madia Lozupone, Biagio Moretti, Gianluigi Giannelli, Rodolfo Sardone and Francesco Panza

Article first published online:
14 May 2021 | DOI: 10.1111/ene.14882

ORIGINAL ARTICLE

Prevalence/potential risk factors for motoric cognitive risk and its relationship to falls in elderly Chinese people: a cross‐sectional study

Background and purpose

Motoric cognitive risk syndrome (MCR) is characterized by slow walking speed and subjective memory complaints (SMCs). This study investigated the prevalence and potential risk factors of MCR and its association with falls in Chinese community‐dwelling older adults.

Methods

The analysis was based on data from the Rugao Longevity and Aging Study (RuLAS). MCR was defined as the presence of both SMCs and slow walking speed in participants free of major neurocognitive disorders. SMCs were determined according to a positive answer to the question ‘Do you feel you have more problems with memory than most?’ in the 15‐item Geriatric Depression Scale. Slow walking speed was defined as one standard deviation or more below the mean value for patients’ age and sex. Data on falls were derived from a standardized questionnaire.

Results

The prevalence of SMCs, slow walking speed and MCR in the RuLAS cohort ( = 1592) was 51.9%, 15.6% and 8.3%, respectively. After adjusting for other covariates, an occupation of farming (odds ratio [OR] 2.358, 95% confidence interval [CI] 1.007–5.521, = 0.048), history of cerebrovascular disease (OR 2.215, 95% CI 1.032–4.752, = 0.041) and hospitalization (OR 2.008, 95% CI 1.120–3.602, = 0.019) were risk factors for MCR. Binary logistic regression analysis indicated that the risk of falls was increased by MCR (OR 1.547, 95% CI 1.009–2.371), SMC (OR 1.308, 95% CI 1.003–1.707) and slow walking speed (OR 1.442, 95% CI 1.030–2.017).

Conclusions

Early identification of potential risk factors of MCR can prevent the occurrence of adverse health events such as falls in the elderly.

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Authors:
Jing‐lin Yuan, Rui‐xue Zhao, Ya‐jun Ma, Xiao‐dong Li, Xiao‐mei Zhou, Xiao‐feng Wang, Xiao‐yan Jiang and Shu‐juan Li

Article first published online:
14 May 2021 | DOI: 10.1111/ene.14884

LETTERS TO THE EDITOR

Two patients with dermatoneuro syndrome after influenza A infection

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Authors:
Galia Valentinova Anguelova, Korné Jellema, Sander Wagemakers and Ron Peters

Article first published online:
20 May 2021 | DOI: 10.1111/ene.14886

ORIGINAL ARTICLE

Restless legs syndrome and perceived olfactory and taste dysfunction: A community‐based study

Background and purpose

Restless legs syndrome (RLS) has been suggested as a prodromal symptom of Parkinson disease (PD). Olfactory or taste dysfunction can also occur preceding PD diagnosis. However, whether RLS is associated with chemosensory dysfunction remains unknown. We thus aim to investigate the association between RLS and perceived olfactory and taste dysfunction.

Methods

We performed a cross‐sectional analysis including 90,337 Chinese adults free of neurodegenerative diseases in the Kailuan study in 2016. Presence of RLS was defined using revised RLS diagnostic criteria or the Cambridge‐Hopkins questionnaire for RLS. Perceived olfactory and taste dysfunction was collected via a questionnaire. The association between RLS and perceived olfactory and taste dysfunction was assessed using logistic regression model, adjusting for potential cofounders such as age, sex, and medical history.

Results

RLS was associated with high odds of having perceived olfactory and/or taste dysfunction (adjusted odds ratio = 5.92, 95% confidence interval = 3.11–11.3). The significant association persisted when using the Cambridge‐Hopkins questionnaire (adjusted odds ratio = 5.55, 95% confidence interval = 2.37–13.0) or when excluding participants with major chronic diseases.

Conclusions

RLS was associated with increased odds of perceived olfactory and taste dysfunction.

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Authors:
Sheng Zhuang, Xiaodong Yuan, Chaoran Ma, Na Yang, Chun‐Feng Liu, Muzi Na, John W. Winkelman, Shouling Wu and Xiang Gao

Article first published online:
24 May 2021 | DOI: 10.1111/ene.14890

ORIGINAL ARTICLE

Status epilepticus with prominent motor symptoms clusters into distinct electroclinical phenotypes

Background and purpose

Status epilepticus (SE) is a heterogeneous condition and considerable variability exists in its etiology, semiology, electroencephalographic correlates, and response to treatment. The aim of the present study was to explore whether distinct phenotypes may be identified within SE with prominent motor symptoms.

Methods

Consecutive episodes of SE with prominent motor symptoms in patients aged ≥14 years were included. Etiology of SE was defined as symptomatic (acute, remote, progressive) or unknown. Electroencephalogram (EEG) recordings were searched for lateralized periodic discharges (LPDs), generalized sharply and/or triphasic periodic potentials (GPDs), and spontaneous burst suppression (BS). According to treatment response, SE was classified into responsive, refractory and super‐refractory. Average linkage hierarchical cluster analysis was performed with Pearson's correlation as a similarity measure.

Results

A total of 240 episodes of SE were identified. Three major clusters were found. The first cluster linked focal motor SE evolving into non‐convulsive SE (NCSE), presence of LPDs/GPDs on EEG, unknown etiology and treatment refractoriness. The second cluster linked convulsive and myoclonic SE evolving into NCSE, presence of spontaneous BS on EEG, progressive symptomatic etiology and super‐refractoriness. The third cluster linked convulsive and myoclonic SE not evolving into other semiologies, absence of LPDs/GPDs/spontaneous BS on EEG, acute symptomatic etiology and treatment responsiveness.

Conclusions

Distinct electroclinical phenotypes characterized by different response to pharmacological intervention can be identified within the heterogeneity of SE with prominent motor phenomena.

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Authors:
Simona Lattanzi, Giada Giovannini, Francesco Brigo, Niccolò Orlandi, Eugen Trinka and Stefano Meletti

Article first published online:
20 May 2021 | DOI: 10.1111/ene.14891

ORIGINAL ARTICLE

Patent foramen ovale closure in stroke patients with migraine in the CLOSE trial. The CLOSE‐MIG study

Background and purpose

The efficacy of patent foramen ovale (PFO) closure to reduce the frequency of migraine attacks remains controversial.

Methods

This was a planned sub‐study in migraine patients enrolled in a randomized, clinical trial designed to assess the superiority of PFO closure plus antiplatelet therapy over antiplatelet therapy alone to prevent stroke recurrence in patients younger than 60 years with a PFO‐associated cryptogenic ischaemic stroke. The main outcome was the mean annual number of migraine attacks in migraine patients with aura and in those without aura, as recorded at each follow‐up visit by study neurologists.

Results

Of 473 patients randomized to PFO closure or antiplatelet therapy, 145 (mean age 41.9 years; women 58.6%) had migraine (75 with aura and 70 without aura). Sixty‐seven patients were randomized to PFO closure and 78 to antiplatelet therapy. During a mean follow‐up of about 5 years, there were no differences between antiplatelet‐only and PFO closure groups in the mean annual number of migraine attacks, both in migraine patients with aura (9.2 [11.9] vs. 12.0 [19.1], = 0.81) and in those without aura (12.1 [16.1] vs. 11.8 [18.4], > 0.999). There were no differences between treatment groups regarding cessation of migraine attacks, migraine‐related disability at 2 years and use of migraine‐preventive drugs during follow‐up.

Conclusions

In young and middle‐aged adults with PFO‐associated cryptogenic stroke and migraine, PFO closure plus antiplatelet therapy did not reduce the mean annual number of migraine attacks compared to antiplatelet therapy alone, in migraine patients both with and without aura.

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Authors:
Jean‐Louis Mas, Benoît Guillon, Anaïs Charles‐Nelson, Valérie Domigo, Laurent Derex, Evelyne Massardier, Caroline Arquizan, Fabrice Vuillier, Serge Timsit, Yannick Béjot, Olivier Detante, Denis Sablot, Céline Guidoux, Igor Sibon, Nelly Dequatre‐Ponchelle, Emmanuel Touzé, Sandrine Canaple, Sonia Alamowitch, Pierre Aubry, Emmanuel Teiger, Geneviève Derumeaux and Gilles Chatellier

Article first published online:
16 Jun 2021 | DOI: 10.1111/ene.14892

ORIGINAL ARTICLE

Seizures after decompressive hemicraniectomy for large middle cerebral artery territory infarcts: Incidence, associated factors, and impact on long‐term outcomes

Background and purpose

Decompressive hemicraniectomy (DH) reduces mortality of large middle cerebral artery (MCA) territory infarcts. Survivors are at high risk of poststroke seizures (PSSs). This study aims to describe the incidence of PSSs, to identify associated factors, and to assess their impact on long‐term outcomes.

Methods

We included consecutive patients who underwent DH for large MCA infarcts from May 2005 to December 2019 at Lille University Hospital. Patients were followed up at 3 months, 1 year, and 3 years. We analysed (i) the incidence and associated factors of early onset PSSs (EPSSs) with logistic regression models; (ii) the incidence and associated factors of late onset PSSs (LPSSs) in survivors at 7 days with a univariate Cox proportional hazard regression model for interval‐censored data; and (iii) the impact of PSSs (EPSSs and LPSSs) on mortality with univariate and multivariate Cox proportional hazard regression models and modified Rankin Scale at 1 and 3 years, with univariate and adjusted multivariate ordinal logistic regression analyses.

Results

Of 248 patients (150 men, 60.5%; mean age = 50.4 ± 9.6 years), 106 (42.7%) presented PSSs (six inaugural seizures, 22 EPSSs, 78 LPSSs) during follow‐up. The PSS cumulative incidence was 12.3% at 7 days, 24.9% at 3 months, 49.8% at 1 years, and 54.8% at 3 years. No predictor was significantly associated with either EPSSs or LPSSs. PSSs did not significantly impact mortality and long‐term functional outcome.

Conclusions

The incidence of PSSs after DH is high, reaching more than 50% 3 years after stroke, but PSSs did not influence long‐term mortality or functional outcome.

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Authors:
Olivier Masheka‐Cishesa, Maéva Kyheng, Charlotte Cordonnier, Grégory Kuchcinski, Maxime Chochoi, Jean Paul Lejeune, Hilde Hénon and Barbara Casolla

Article first published online:
24 May 2021 | DOI: 10.1111/ene.14893

ORIGINAL ARTICLE

A functional variant of the long noncoding RNA AL110200 is associated with the risk of ischaemic stroke recurrence

Background and purpose

This study aimed to test the hypothesis that long noncoding RNA (lncRNA) AL110200 exerts a proinflammatory effect on atherosclerosis and that the variant rs901681 contributes to ischaemic stroke incidence and recurrence.

Methods

The expression of AL110200 was analyzed in THP‐1 cells treated with oxidized low‐density lipoprotein and in human peripheral blood in a coronary heart disease and control population to determine the role of AL110200 in atherosclerosis. The effect of AL110200 on cell adhesion and invasion was tested. The plasma level of leukotriene B4 and rs901681 genotype distribution were assessed in 220 participants. In 1004 ischaemic stroke patients and 1434 controls, the association between rs901681 and stroke incidence was analyzed by logistic regression, and the association of rs901681 and stroke prognosis was analyzed using Kaplan–Meier analysis and the Cox proportional hazards model.

Results

Increased expression of AL110200 was observed in THP‐1 cells under oxidized low‐density lipoprotein treatment. Knockdown of AL110200 reduced the adhesive and invasive ability of THP‐1 cells. AL110200 expression in peripheral blood was significantly higher in the coronary heart disease group than in the controls. The GG genotype of rs901681 is associated with reduced plasma leukotriene B4. In the ischaemic stroke population, rs901681 was not associated with ischaemic stroke incidence (= 0.686). Patients carrying rs901681 GG had a lower risk for stroke recurrence at age ≥60 years (= 0.001), cardiovascular stroke death (= 0.022) and all‐cause mortality (= 0.034) in the all‐age group.

Conclusions

AL110200 might exert a proinflammatory effect on atherosclerosis, and the variant rs901681 might be a strong predictor of stroke prognosis in ischaemic stroke patients.

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Authors:
Li Song, Hao Li, Miaomiao Suo, Yingying Sun, Ming Su, Yan Song, Ning Xiao, Rutai Hui, Chunchang Qin and Jingzhou Chen

Article first published online:
01 Jun 2021 | DOI: 10.1111/ene.14895

ORIGINAL ARTICLE

Quantitative magnetic resonance imaging of the brachial plexus shows specific changes in nerve architecture in chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy and motor neuron disease

Background

The immunological pathophysiologies of chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) differ considerably, but neither has been elucidated completely. Quantitative magnetic resonance imaging (MRI) techniques such as diffusion tensor imaging, T2 mapping, and fat fraction analysis may indicate in vivo pathophysiological changes in nerve architecture. Our study aimed to systematically study nerve architecture of the brachial plexus in patients with CIDP, MMN, motor neuron disease (MND) and healthy controls using these quantitative MRI techniques.

Methods

We enrolled patients with CIDP ( = 47), MMN ( = 29), MND ( = 40) and healthy controls ( = 10). All patients underwent MRI of the brachial plexus and we obtained diffusion parameters, T2 relaxation times and fat fraction using an automated processing pipeline. We compared these parameters between groups using a univariate general linear model.

Results

Fractional anisotropy was lower in patients with CIDP compared to healthy controls ( < 0.001), patients with MND ( = 0.010) and MMN ( < 0.001). Radial diffusivity was higher in patients with CIDP compared to healthy controls ( = 0.015) and patients with MND ( = 0.001) and MMN ( < 0.001). T2 relaxation time was elevated in patients with CIDP compared to patients with MND ( = 0.023). Fat fraction was lower in patients with CIDP and MMN compared to patients with MND (both < 0.001).

Conclusion

Our results show that quantitative MRI parameters differ between CIDP, MMN and MND, which may reflect differences in underlying pathophysiological mechanisms.

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Authors:
Marieke H. J. van Rosmalen, H. Stephan Goedee, Rosina Derks, Fay‐Lynn Asselman, Camiel Verhamme, Alberto de Luca, J. Hendrikse, W. Ludo van der Pol and Martijn Froeling

Article first published online:
27 May 2021 | DOI: 10.1111/ene.14896

REVIEW ARTICLE

Uncrossed corticospinal tract in health and genetic disorders: Review, case report, and clinical implications

Background and purpose

Crossing pathologies of the corticospinal tract (CST) are rare and often associated with genetic disorders. However, they can be present in healthy humans and lead to ipsilateral motor deficits when a lesion to motor areas occurs. Here, we review historical and current literature of CST crossing pathologies and present a rare case of asymmetric crossing of the CST.

Methods

Description of the case and systematic review of the literature were based on the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta‐Analyses) guidelines. The PubMed database was searched for peer‐reviewed articles in English since 1950. All articles on ipsilateral stroke, uncrossed CST, and associated neurologic disorders were screened. Furthermore, a literature review between the years 1850 and 1980 including articles in other languages, books, opinions, and case studies was conducted.

Results

Only a few descriptions of CST crossing pathologies exist in healthy humans, whereas they seem to be more common in genetic disorders such as horizontal gaze palsy with progressive scoliosis or congenital mirror movements. Our patient presented with aphasia and left‐sided hemiparesis. Computed tomographic (CT) scan revealed a perfusion deficit in the left middle cerebral artery territory, which was confirmed by diffusion‐weighted magnetic resonance imaging (MRI), so that thrombolysis was administered. Diffusion tensor imaging with fibre tracking revealed an asymmetric CST crossing.

Conclusions

The knowledge of CST crossing pathologies is essential if a motor deficit occurs ipsilateral to the lesion side. An ipsilateral deficit should not lead to exclusion or delay of therapeutic options in patients with suspected stroke. Here, a combined evaluation of CT perfusion imaging and MRI diffusion imaging may be of advantage.

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Authors:
Filipp M. Filippopulos, Christian Brem, Klaus Seelos, Thomas Köglsperger, Stefan Sonnenfeld, Lars Kellert and Christian Vollmar

Article first published online:
24 May 2021 | DOI: 10.1111/ene.14897

ORIGINAL ARTICLE

Guillain–Barré syndrome in Eastern China: A study of 595 patients

Background and purpose

This study aimed to investigate geographical differences in the clinical features of Guillain–Barré syndrome (GBS) between patients from our region in Eastern China and patients from other areas.

Methods

A total of 595 patients fulfilling the diagnostic criteria for GBS or its variants were included from two large hospitals located in Eastern China. Data collection included demographics, antecedent events, clinical presentation and signs, electrophysiological subtypes, treatment, complications during hospitalization, clinical severity at nadir, and outcome at 12 months, and these data were compared to data from a study conducted in Southern China and the Europe/Americas section of the International GBS Outcome Study.

Results

The median (interquartile range) age of patients was 50 (36–61) years, the ratio of men to women was 1.2, and 49% of patients had antecedent events. Patients in our region of Eastern China had pure motor predominant GBS (158/340, 46%) and 30% (103/340) had complications during hospitalization. Patients aged over 60 years had a lower frequency of antecedent infections and single, axonal subtypes, but higher disability scores at entry, nadir, and 12 months. When compared with the Europe/Americas data, our patients had a lower frequency of antecedent infection (46% vs. 63%), cranial nerve involvement (43% vs. 49%), sensory deficits (45% vs. 69%), pain (19% vs. 57%) and mechanical ventilation (11% vs. 17%), but a higher frequency of axonal subtype (35% vs. 6%). There was a higher frequency of patients with antecedent gastroenteritis (16% vs. 8%), mechanical ventilation (11% vs. 8%) and axonal subtypes (35% vs. 19%) in our region in Eastern China than in Southern China.

Conclusions

Patients with GBS in Eastern China showed significant clinical heterogeneity and differences when compared to other geographic areas.

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Authors:
Yan Song, Yong Zhang, Nobuhiro Yuki, Benjamin R. Wakerley, Chen Liu, Jin Song, Min Wang, Xungang Feng, Yanlei Hao and Yuzhong Wang

Article first published online:
24 May 2021 | DOI: 10.1111/ene.14898

ORIGINAL ARTICLE

Outcomes in young adults with acute ischemic stroke undergoing endovascular thrombectomy: A real‐world multicenter experience

Abstract

Endovascular thrombectomy (EVT) is the standard of care for anterior circulation acute ischemic stroke (AIS) with large vessel occlusion (LVO). Young patients with AIS‐LVO have distinctly different underlying stroke mechanisms and etiologies. Much is unknown about the safety and efficacy of EVT in this population of young AIS‐LVO patients.

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Authors:
Leonard Leong‐Litt Yeo, Vanessa Hui En Chen, Aloysius Sheng‐Ting Leow, Lukas Meyer, Jens Fiehler, Tian‐Ming Tu, Carol Huilian Tham, Ching‐Hui Sia, Ala Jamous, Daniel Behme, Andreas Kastrup, Panagiotis Papanagiotou, Hanna Styczen, Michael Forsting, Tsong‐Hai Lee, Chan‐Lin Chu, Sebastian Fischer, Volker Maus, Nuran Abdullayev, Christoph Kabbasch, Sebastian Mönch, Christian Maegerlein, Fabian Arnberg, Tommy Andersson, Staffan Holmin, Hock‐Luen Teoh, Prakash Paliwal, Aftab Ahmad, Anil Gopinathan, Cunli Yang, Raymond Chee‐Seong Seet, Bernard Poon‐Lap Chan, Vijay K. Sharma and Benjamin Yong‐Qiang Tan

Article first published online:
25 May 2021 | DOI: 10.1111/ene.14899

ORIGINAL ARTICLE

Diagnostic accuracy of the Dysphagia Trained Nurse Assessment tool in acute stroke

Background and purpose

Comprehensive swallow screening assessments to identify dysphagia and make early eating and drinking recommendations can be used by trained nurses. This study aimed to validate the Dysphagia Trained Nurse Assessment (DTNAx) tool in acute stroke patients.

Methods

Participants with diagnosed stroke were prospectively and consecutively recruited from an acute stroke unit. Following a baseline DTNAx on admission, participants underwent a speech and language therapist (SLT) bedside assessment of swallowing (speech and language therapist assessment [SLTAx]), videofluoroscopy (VFS) and a further DTNAx by the same or a different nurse.

Results

Forty‐seven participants were recruited, of whom 22 had dysphagia. Compared to SLTAx in the identification of dysphagia, DTNAx had a sensitivity of 96.9% (95% confidence interval [CI] 83.8–99.9) and specificity of 89.5% (95% CI 75.2–97.1). Compared to VFS in the identification of aspiration, DTNAx had a sensitivity of 77.8% (95% CI 40.0–97.2) and a specificity of 81.6% (95% CI 65.7–92.3). Over 81% of the diet and fluid recommendations made by the dysphagia trained nurses were in absolute agreement compared to SLTAx. Both DTNAx and SLTAx had low diagnostic accuracy compared to the VFS‐based definition of dysphagia.

Conclusions

Nurses trained in DTNAx showed good diagnostic accuracy in identifying dysphagia compared to SLTAx and in identifying aspiration compared to VFS. They made appropriate diet and fluid recommendations in line with SLTs in the early management of dysphagia.

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Authors:
Jacqueline K. Benfield, Gwenllian Wilkinson, Lisa F. Everton, Philip M. Bath and Timothy J. England

Article first published online:
29 May 2021 | DOI: 10.1111/ene.14900

ORIGINAL ARTICLE

Very late onset neuromyelitis optica spectrum disorders

Background and purpose

Neuromyelitis optica spectrum disorder (NMOSD) often presents in the elderly with an insidious onset of symptoms and aggressive progression. There have been anecdotal cases of very late onset (VLO)‐NMOSD, but case series reports are rare. The aim of this retrospective study was to clarify the clinical features of VLO‐NMOSD.

Methods

According to the age at onset, we classified patients with NMOSD into three subgroups: ≤49 years, early onset NMOSD (EO‐NMOSD); 50–69 years, late onset NMOSD (LO‐NMOSD); and ≥70 years, VLO‐NMOSD. We evaluated the clinical characteristics, magnetic resonance imaging (MRI) findings, laboratory data, and immunotherapies of the groups.

Results

Overall, 12 men and 64 women with a median (interquartile range) age at onset and duration of disease of 42.0 (29.0–55.8) years and 70.0 (16.3–143.0) months, respectively, were included. Eight (11%) patients had VLO‐NMOSD, 22 (29%) had LO‐NMOSD, and 46 (61%) had EO‐NMOSD. Patients with EO‐NMOSD had a significantly longer interval between episodes as well as time between the first symptom and diagnosis of NMOSD than did those with VLO‐NMOSD and LO‐NMOSD ( = 0.046). Optic neuritis and nerve lesions on MRI were significantly less frequent in patients with VLO‐NMOSD than in those with LO‐NMOSD and EO‐NMOSD ( = 0.002 and = 0.028, respectively). In contrast, patients with VLO‐NMOSD had higher nadir Expanded Disability Status Scale and Nurick scale scores and a significantly longer spinal lesion length than did those with LO‐NMOSD and EO‐NMOSD ( = 0.029, = 0.049, and = 0.032, respectively).

Conclusions

Patients with VLO‐NMOSD tend to develop severe myelitis with long cord lesions but not optic neuritis.

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Authors:
Keiichi Nakahara, Shunya Nakane, Akiko Nagaishi, Tomoko Narita, Hidenori Matsuo and Yukio Ando

Article first published online:
25 May 2021 | DOI: 10.1111/ene.14901

ORIGINAL ARTICLE

Treatment expectations and perception of therapy in adult patients with spinal muscular atrophy receiving nusinersen

Background and purpose

This was an investigation of treatment expectations and of the perception of therapy in adult patients with 5q‐associated spinal muscular atrophy (5q‐SMA) receiving nusinersen.

Methods

A prospective, non‐interventional observational study of nusinersen treatment in adult 5q‐SMA patients was conducted at nine SMA centers in Germany. The functional status, treatment expectations and perceived outcomes were assessed using the Amyotrophic Lateral Sclerosis Functional Rating Scale—extended (ALS‐FRS‐ex), the Measure Yourself Medical Outcome Profile (MYMOP2), the Treatment Satisfaction Questionnaire for Medication (TSQM‐9) and the Net Promoter Score (NPS).

Results

In all, 151 patients were included with a median age of 36 years (15–69 years). SMA type 3 ( = 90, 59.6%) prevailed, followed by type 2 (33.8%) and type 1 (6.6%). In SMA types 1–3, median ALS‐FRS‐ex scores were 25, 33 and 46 (of 60 scale points), respectively. MYMOP2 identified distinct treatment expectations: head verticalization ( = 13), bulbar function ( = 16), arm function ( = 65), respiration ( = 15), trunk function ( = 34), leg function ( = 76) and generalized symptoms ( = 77). Median symptom severity decreased during nusinersen treatment (median observational period 6.1 months, 0.5–16 months) from 3.7 to 3.3 MYMOP2 score points (< 0.001). The convenience of drug administration was critical (49.7 of 100 TSQM‐9 points, SD 22); however, the overall treatment satisfaction was high (74.3, SD 18) and the recommendation rating very positive (NPS +66).

Conclusions

Nusinersen was administered across a broad range of ages, disease durations and motor function deficits. Treatment expectations were highly differentiated and related to SMA type and functional status. Patient‐reported outcomes demonstrated a positive perception of nusinersen therapy in adult patients with 5q‐SMA.

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Authors:
Thomas Meyer, André Maier, Zeljko Uzelac, Tim Hagenacker, René Günther, Olivia Schreiber‐Katz, Markus Weiler, Robert Steinbach, Ute Weyen, Jan Christoph Koch, Dagmar Kettemann, Jenny Norden, Johannes Dorst, Claudia Wurster, Albert C. Ludolph, Benjamin Stolte, Maren Freigang, Alma Osmanovic, Susanne Petri, Julian Grosskreutz, Annekathrin Rödiger, Ramona Griep, Marcel Gaudlitz, Bertram Walter, Christoph Münch and Susanne Spittel

Article first published online:
16 Jun 2021 | DOI: 10.1111/ene.14902

SHORT COMMUNICATION

White matter abnormalities in a patient with visual snow syndrome: New evidence from a diffusion tensor imaging study

Background

Visual snow syndrome (VSS) is a neurological condition characterized by flickering dots throughout the entire visual field. Both the pathophysiology and possible location of VSS are still under debate. White matter abnormalities were investigated using diffusion tensor imaging (DTI) in a patient with VSS.

Methods

A 28‐year‐old patient with VSS and 10 healthy controls were investigated with DTI. Diffusion parametric maps were calculated and reconstructed using q‐space diffeomorphic reconstruction. White matter pathways of the dorsal, ventral, integrative visual streams and thalamic connectivity were tracked. Then, they were applied to each subject's parameter map, stretched to the same length, and sampled along the tracts for regional analyses of DTI parameters.

Results

Compared with healthy controls, our patient displayed higher axial diffusivity (AD) and radial diffusivity (RD) in the dorsal visual stream (cingulum, arcuate fasciculus, horizontal indirect anterior segment of the superior longitudinal fasciculus), in the ventral visual stream (fronto‐occipital fasciculus, inferior longitudinal fasciculus) and in the integrative visual stream (indirect posterior component of the superior longitudinal fasciculus, vertical occipital fasciculus). Higher AD and RD were also detected in acoustic and optic radiations, and in thalamic radiations distal to the thalamus.

Conclusion

This VSS patient displayed multiple, bilateral white matter changes in the temporo‐parieto‐occipital junction in white matter pathways related to vision. We encourage the study of white matter pathology using DTI in complex neurological syndromes including VSS.

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Authors:
Francesco Latini, Markus Fahlström, Niklas Marklund and Amalia Feresiadou

Article first published online:
28 May 2021 | DOI: 10.1111/ene.14903

ORIGINAL ARTICLE

Inadvertent hypothermia after endovascular therapy is not associated with improved outcome in stroke due to anterior circulation large vessel occlusion

Background and purpose

Hypothermia may be neuroprotective in acute ischemic stroke. Patients with anterior circulation large vessel occlusion (acLVO) are frequently hypothermic after endovascular therapy (EVT). We sought to determine whether this inadvertent hypothermia is associated with improved outcome.

Methods

We extracted data of consecutive patients (January 2016 to May 2019) who received EVT for acLVO from our prospective EVT register of all patients screened for EVT at our tertiary stroke center. We assessed functional outcome at 3 months and performed multivariate analysis to calculate adjusted risk ratios (aRRs) for favorable outcome (modified Rankin Scale scores = 0–2) and mortality across patients who were hypothermic (<36°C) and patients who were normothermic (≥36°C to <37.6°C) after EVT. Moreover, we compared the frequency of complications between these groups.

Results

Among 837 patients screened, 416 patients received EVT for acLVO and fulfilled inclusion criteria (200 [48.1%] male, mean age = 76 ± 16 years, median National Institutes of Health Stroke Scale score = 16, interquartile range [IQR] = 12–20). Of these, 209 patients (50.2%) were hypothermic (median temperature = 35.2°C, IQR = 34.7–35.7) and 207 patients were normothermic (median temperature = 36.4°C, IQR = 36.1–36.7) after EVT. In multivariate analysis, hypothermia was not associated with favorable outcome (aRR = 0.99, 95% confidence interval [CI] = 0.75–1.31) and mortality (aRR = 1.18, 95% CI = 0.84–1.66). More hypothermic patients suffered from pneumonia (36.4% vs. 25.6%, = 0.02) and bradyarrhythmia (52.6% vs. 16.4%, < 0.001), whereas thromboembolic events were distributed evenly (5.7% vs. 6.8%, not significant).

Conclusions

Inadvertent hypothermia after EVT for acLVO is not associated with improved functional outcome or reduced mortality but is associated with an increased rate of pneumonia and bradyarrhythmia in patients with acute ischemic stroke.

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Authors:
Christian Hartmann, Simon Winzer, Lars‐Peder Pallesen, Alexandra Prakapenia, Timo Siepmann, Haidar Moustafa, Hermann Theilen, Jessica Barlinn, Johannes C. Gerber, Jennifer Linn, Heinz Reichmann, Kristian Barlinn and Volker Puetz

Article first published online:
04 Jun 2021 | DOI: 10.1111/ene.14906

ORIGINAL ARTICLE

Patient perceptions of outcome measures in chronic inflammatory demyelinating polyneuropathy: A study of the Inflammatory Rasch‐built Overall Disability Scale

Background

Patients' perceptions of outcome measures used in chronic inflammatory demyelinating polyneuropathy (CIDP) are unknown.

Methods

We performed a cross‐sectional evaluation of patient perceptions of the Inflammatory Rasch‐built Overall Disability Scale (I‐RODS) from 41 subjects with CIDP through a structured questionnaire. We assessed perceived hesitation to provide a response, item importance and relevance, understanding of specific items and factors affecting responses.

Results

Hesitation to provide a categorical answer was reported by 20% of subjects or more, for 5/24 (20.8%) items. Uncertainty was most frequent for “travel by public transport” (22.4%) and “catch an object (e.g., ball)” (24%). Six of 24 (25%) items were perceived as unimportant to their disease by at least a third of participants. Items most commonly perceived as unimportant were “travel by public transport” in 53.7%, “catch an object (e.g., ball)” in 61% and “dance” in 65.9%. Several items were frequently perceived as irrelevant. These included “move a chair” (39%), “do the dishes” (46.3%), “catch an object (e.g., ball)” (61%), “travel by public transport” (68.3%) and “stand for hours” (82.9%). The understanding of multiple items such as “read a book”, “sit on a toilet” and “take a shower” was found to be highly variable. Fatigue was perceived more commonly than mood (53.7% vs. 17.1%, = 0.001), and more commonly in younger subjects ( = 0.037), as influencing responses to the I‐RODS.

Conclusions

Patient‐perceived uncertainty, unimportance, irrelevance and poor understanding of items, as well as fatigue and mood, impact on the value of the I‐RODS. Greater emphasis on individualized disability assessments requires consideration in future.

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Authors:
Daniel White, Christina Englezou and Yusuf A. Rajabally

Article first published online:
27 May 2021 | DOI: 10.1111/ene.14907

ORIGINAL ARTICLE

variants as a possible cause of frontotemporal dementia with distinct neuroimaging features

Background and purpose

Nasu–Hakola disease (NHD) is a rare, autosomal recessive disorder characterized by skeletal and neurological symptoms. Behavioral symptoms with cognitive impairment may mimic the behavioral variant of frontotemporal dementia (bvFTD) and other early‐onset dementias. Our patients were analyzed and the literature was reviewed to delineate neurological and neuroimaging findings suggestive of NHD.

Method

Fourteen patients carrying a pathogenic mutation in the gene were found in our database. Demographic, clinical, laboratory and radiological data were retrieved and analyzed.

Results

The presenting clinical picture was behavioral changes with cognitive decline resembling bvFTD in all patients. The mean age was 37.1 ± 4.97 years and the mean duration of the disease was 8.9 ± 3.51 years. Only two patients had typical bone cysts. Seven patients had bilateral calcification of the basal ganglia in computed tomography of the brain. Magnetic resonance imaging of the brain revealed severe atrophy of the corpus callosum, enlargement of the ventricles, atrophy of the caudate nuclei and periventricular white matter changes in all patients. Symmetrical global atrophy of the brain mainly affecting frontoparietal and lateral temporal regions were observed in all cases, and 13 patients had atrophy of the hippocampus. Cerebrospinal fluid examination of 10 patients showed elevated protein levels in six and the presence of oligoclonal bands in four patients.

Conclusion

A combination of white matter changes, enlarged ventricles, atrophy of the caudate nuclei and thinning of the corpus callosum in magnetic resonance imaging strongly suggests NHD in patients with FTD syndrome. Molecular genetic analysis should be performed in suspected cases, and families should receive genetic counseling.

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Authors:
Bedia Samanci, Başar Bilgiç, Özlem Gelişin, Fatih Tepgeç, Gamze Guven, Zeynep Tüfekçioğlu, Merve Alaylıoğlu, Hasmet A. Hanagasi, Hakan Gürvit, Rita Guerreiro, John Hardy and Murat Emre

Article first published online:
01 Jun 2021 | DOI: 10.1111/ene.14908

ORIGINAL ARTICLE

Impaired fixation suppression of horizontal vestibular nystagmus during smooth pursuit: pathophysiology and clinical implications

Background and purpose

A peripheral spontaneous nystagmus (SN) is typically enhanced or revealed by removing fixation. Conversely, failure of fixation suppression of SN is usually a sign of a central disorder. Based on Luebke and Robinson ( 1988, vol. 28 (8), pp. 941–946), who suggested that the normal fixation mechanism is disengaged during pursuit, it is hypothesized that vertical tracking in the light would bring out or enhance a horizontal SN.

Methods

Eighteen patients with acute vestibular neuritis were studied. Eye movements were recorded using video‐oculography at straight‐ahead gaze with and without visual fixation, and during smooth pursuit. The slow‐phase velocity and the fixation suppression indices of nystagmus (relative to SN in darkness) were compared in each condition.

Results

During vertical tracking, the slow‐phase velocity of horizontal SN with eyes near straight‐ahead gaze was significantly higher (median 2.7°/s) than under static visual fixation (median 1.2°/s). Likewise, the fixation index was significantly higher (worse suppression) during pursuit (median 48%) than during fixation (median 26%). A release of SN was also suggested during horizontal pursuit, if one assumes superposition of SN on a normal and symmetrical pursuit capability.

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Authors:
Athanasia Korda, David S. Zee, Thomas Wyss, Ewa Zamaro, Marco D. Caversaccio, Franca Wagner, Roger Kalla and Georgios Mantokoudis

Article first published online:
07 Jun 2021 | DOI: 10.1111/ene.14909

ORIGINAL ARTICLE

Associations of cognitive reserve and psychological resilience with cognitive functioning in subjects with cerebral white matter hyperintensities

Background and purpose

Cerebral small vessel disease is characterized by progressive white matter hyperintensities (WMH) and cognitive decline. However, variability exists in how individuals maintain cognitive capabilities despite significant neuropathology. The relationships between individual cognitive reserve, psychological resilience and cognitive functioning were examined in subjects with varying degrees of WMH.

Methods

In the Helsinki Small Vessel Disease Study, 152 subjects (aged 65–75 years) underwent a comprehensive neuropsychological assessment, evaluation of subjective cognitive complaints and brain magnetic resonance imaging with volumetric WMH evaluation. Cognitive reserve was determined by education (years) and the modified Cognitive Reserve Scale (mCRS). Psychological resilience was evaluated with the Resilience Scale 14.

Results

The mCRS total score correlated significantly with years of education ( = 0.23, < 0.01), but it was not related to age, sex or WMH volume. Together, mCRS score and education were associated with performance in a wide range of cognitive domains including processing speed, executive functions, working memory, verbal memory, visuospatial perception and verbal reasoning. Independently of education, the mCRS score had incremental predictive value on delayed verbal recall and subjective cognitive complaints. Psychological resilience was not significantly related to age, education, sex, WMH severity or cognitive test scores, but it was associated with subjective cognitive complaints.

Conclusions

Cognitive reserve has strong and consistent associations with cognitive functioning in subjects with WMH. Education is widely associated with objective cognitive functioning, whereas lifetime engagement in cognitively stimulating leisure activities (mCRS) has independent predictive value on memory performance and subjective cognitive complaints. Psychological resilience is strongly associated with subjective, but not objective, cognitive functioning.

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Authors:
Anne Arola, Hanna M. Laakso, Johanna Pitkänen, Juha Koikkalainen, Jyrki Lötjönen, Antti Korvenoja, Timo Erkinjuntti, Susanna Melkas and Hanna Jokinen

Article first published online:
25 May 2021 | DOI: 10.1111/ene.14910

ORIGINAL ARTICLE

Migraine prevalence in visual snow with prior illicit drug use (hallucinogen persisting perception disorder) versus without

Background and purpose

This study was undertaken to investigate migraine prevalence in persons with hallucinogen persisting perception disorder (HPPD) presenting as visual snow syndrome (VSS).

Methods

Persons with visual snow as a persisting symptom after illicit drug use (HPPD) were recruited via a Dutch consulting clinic for recreational drug use. A structured interview on (visual) perceptual symptomatology, details of drugs use, and medical and headache history was taken. As a control group, persons with visual snow who had never used illicit drugs prior to onset were included. The primary outcome was lifetime prevalence of migraine. Symptom severity was evaluated by the Visual Snow Handicap Inventory (VHI), a 25‐item questionnaire.

Results

None of the 24 HPPD participants had migraine, whereas 20 of 37 (54.1%) controls had migraine ( < 0.001). VHI scores did not differ significantly between the two groups; in both groups, the median score was 38 of 100. In most HPPD cases (17/24, 70.9%), visual snow had started after intake of ecstasy; other psychedelic drugs reported included cannabis, psilocybin mushrooms, amphetamine, 4‐fluoroamphetamine, 3‐methylmethcathinone, 4‐Bromo‐2,5‐dimethoxypenethylamine, and nitrous oxide.

Conclusions

Whereas none of the HPPD participants had migraine, more than half of the visual snow controls without prior use of illicit drugs had migraine. This suggests that at least partly different pathophysiological factors play a role in these disorders. Users of ecstasy and other hallucinogens should be warned of the risk of visual snow. Further studies are needed to enhance understanding of the underlying neurobiology of HPPD and VSS to enable better management of these conditions.

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Authors:
Robin M. van Dongen, Gerard J. Alderliefste, Gerrit L. J. Onderwater, Michel D. Ferrari and Gisela M. Terwindt

Article first published online:
15 Jun 2021 | DOI: 10.1111/ene.14914

REVIEW ARTICLE

The core/penumbra model: implications for acute stroke treatment and patient selection in 2021

Abstract

Despite major advances in prevention, ischaemic stroke remains one of the leading causes of death and disability worldwide. After centuries of nihilism and decades of failed neuroprotection trials, the discovery, initially in non‐human primates and subsequently in man, that ischaemic brain tissue termed the ischaemic penumbra can be salvaged from infarction up to and perhaps beyond 24 h after stroke onset has underpinned the development of highly efficient reperfusion therapies, namely intravenous thrombolysis and endovascular thrombectomy, which have revolutionized the management of the acute stroke patient. Animal experiments have documented that how long the penumbra can survive depends not only on time elapsed since arterial occlusion (‘time is brain’), but also on how severely perfusion is reduced. Novel imaging techniques allowing the penumbra and the already irreversibly damaged core in the individual subject to be mapped have documented that the time course of core growth at the expense of the penumbra widely differs from patient to patient, and hence that individual physiology should be considered in addition to time since stroke onset for decision‐making. This concept has been implemented to optimize patient selection in pivotal trials of reperfusion therapies beyond 3 h after stroke onset and is now routinely applied in clinical practice, using computed tomography or magnetic resonance imaging. The notion that, in order to be both efficient and harmless, treatment should be tailored to each patient's physiological characteristics represents a radical move towards precision medicine.

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Authors:
Jean‐Claude Baron

Article first published online:
18 Jun 2021 | DOI: 10.1111/ene.14916

ORIGINAL ARTICLE

Cross‐sectional study on patients' understanding and views of the informed consent procedure of a secondary stroke prevention trial

Background and purpose

Improving understanding of study contents and procedures might enhance recruitment into studies and retention during follow‐up. However, data in stroke patients on understanding of the informed consent (IC) procedure are sparse.

Methods

We conducted a cross‐sectional study among ischemic stroke patients taking part in the IC procedure of an ongoing cluster‐randomized secondary prevention trial. All aspects of the IC procedure were assessed in an interview using a standardized 20‐item questionnaire. Responses were collected within 72 h after the IC procedure and analyzed quantitatively and qualitatively. Participants were also asked their main reasons for participation.

Results

A total of 146 stroke patients (65 ± 12 years old, 38% female) were enrolled. On average, patients recalled 66.4% (95% confidence interval = 65.2%–67.5%) of the content of the IC procedure. Most patients understood that participation was voluntary (99.3%) and that they had the right to withdraw consent (97.1%); 79.1% of the patients recalled the study duration and 56.1% the goal. Only 40.3% could clearly state a benefit of participation, and 28.8% knew their group allocation. Younger age, higher graduation, and allocation to the intervention group were associated with better understanding. Of all patients, 53% exclusively stated a personal and 22% an altruistic reason for participation.

Conclusions

Whereas understanding of patient rights was high, many patients were unable to recall other important aspects of study content and procedures. Increased attention to older and less educated patients may help to enhance understanding in this patient population. Actual recruitment and retention benefit of an improved IC procedure remains to be tested in a randomized trial.

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Authors:
Felizitas A. Eichner, Joschua M. Reis, Joaquim Dores, Vladimir Pavlovic, Luisa Kreß, Naeimeh Daneshkhah, Renate Weinhardt, Armin Grau, Johannes Mühler, Hassan Soda, Christopher J. Schwarzbach, Michael Schuler, Karl Georg Häusler and Peter U. Heuschmann

Article first published online:
16 Jun 2021 | DOI: 10.1111/ene.14917

ORIGINAL ARTICLE

P2X7 receptor/NLRP3 inflammasome complex and α‐synuclein in peripheral blood mononuclear cells: a prospective study in neo‐diagnosed, treatment‐naïve Parkinson’s disease

Background and purpose

Neuroinflammation and probably systemic inflammation, with abnormal α‐synuclein deposition, participate in the development of Parkinson's disease (PD). The P2X7 receptor/NLRP3 inflammasome complex is upregulated in the brain of PD patients. By a prospective approach, the degree of systemic activation of such complex, and its regulatory mechanisms, were explored in treatment‐naïve PD individuals.

Methods

The expression and functional activity of the inflammasome were measured in peripheral blood mononuclear cells of 25 newly diagnosed PD patients and 25 controls at baseline and after 12 months of pharmacological treatment, exploring the intracellular signalling involved and its epigenetic regulation.

Results

De novo PD patients were characterized by a systemic hyper‐expression of the P2X7R/NLRP3 inflammasome platform, probably able to modulate lymphomonocyte α‐synuclein, whose brain deposits represent the main pathogenetic factor of PD. A reduced c‐Jun N‐terminal kinase (JNK) phosphorylation might be the intracellular signalling mediating this effect. miR‐7 and miR‐30, implied in the pathogenesis of PD and in the post‐transcriptional control of α‐synuclein and NLRP3 expression, were also increased in PD. After 1 year of usual anti‐Parkinson treatments, such inflammatory platform was significantly reduced.

Conclusions

Mononuclear cells of newly diagnosed PD subjects display a hyper‐expression of the P2X7R/NLRP3 inflammasome platform that seems to modulate cellular α‐synuclein content and is reduced after PD treatment; an impaired JNK phosphorylation might be the intracellular signalling mediating this effect, undergoing an epigenetic regulation by miR‐7 and miR‐30.

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Authors:
Anna Solini, Chiara Rossi, Eleonora Santini, Martina Giuntini, Francesco Raggi, Federico Parolini, Edoardo Biancalana, Eleonora Del Prete, Ubaldo Bonuccelli and Roberto Ceravolo

Article first published online:
02 Jun 2021 | DOI: 10.1111/ene.14918

COMMENTARY

Endovascular treatment of large vessel occlusion acute ischemic stroke: Is there a place for hypothermia?

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Authors:
Kosmas Macha and Stefan Schwab

Article first published online:
13 Jun 2021 | DOI: 10.1111/ene.14920

EDITORIAL

Guidelines should be guidelines: Time to leave the terms “consensus” and “position” for other purposes

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Authors:
Katina Aleksovska, Claudio L. A. Bassetti, Thomas Berger, Vanessa Carvalho, João Costa, Günther Deuschl, Kristian Steen Frederiksen, Joke Jaarsma, Teia Kobulashvili, Maurizio A. Leone, Lucia Pavlakova, Michele Romoli and Luca Vignatelli

Article first published online:
17 Jun 2021 | DOI: 10.1111/ene.14933

ORIGINAL ARTICLE

Diagnostic and prognostic utility of computed tomography perfusion imaging in posterior circulation acute ischemic stroke: A systematic review and meta‐analysis

Background

Computed tomography perfusion (CTP) imaging could be useful in the diagnosis of posterior circulation stroke (PCS) and in identifying patients who are likely to experience favorable outcomes following reperfusion therapy. The current study sought to investigate the diagnostic and prognostic capability of CTP in acute ischemic PCS by performing a systematic review and meta‐analysis.

Methods

Medline/PubMed and the Cochrane Library were searched using the terms: “posterior circulation”, “CT perfusion”, “acute stroke”, and “reperfusion therapy”. The following studies were included: (1) patients aged 18 years or above; (2) patients diagnosed with PCS; and (3) studies with good methodological design. Pooled sensitivity (SENS), specificity (SPEC), and area under the curve (AUC), computed using the summary receiver operating characteristic (SROC) curves, were used to determine diagnostic/prognostic capability.

Results

Out of 14 studies included, a meta‐analysis investigating diagnostic accuracy of CTP was performed on nine studies. Meta‐analysis demonstrated comparable diagnostic accuracy of CTP to non‐contrast computed tomography (NCCT) (AUC: 0.90 [95% CI 0.87–0.92] vs. AUC: 0.96 [95% CI 0.94–0.97]); however, with higher pooled sensitivity (SENS: 72% [95% CI 57%–83%] vs. SENS: 25% [95% CI 17%–35%]) and lower specificity (SPEC: 90% [95% CI 83%–94%] vs. SPEC: 96% [95% CI 95%–98%]) than NCCT. Meta‐analysis to determine prognostic capability of CTP could not be performed.

Conclusions

CTP has limited diagnostic utility in acute ischemic PCS, albeit with superior diagnostic sensitivity and inferior diagnostic specificity to NCCT. Further prospective trials are required to validate the prognostic capability of CTP‐derived parameters in PCS.

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Authors:
Anubhav Katyal, Zeljka Calic, Murray Killingsworth and Sonu Menachem Maimonides Bhaskar

Article first published online:
12 Jun 2021 | DOI: 10.1111/ene.14934

ORIGINAL ARTICLE

Cardiovascular and cerebrovascular risk markers in Parkinson’s disease: Results from a case−control study

Background

The relationship between Parkinson's disease (PD) and cardiovascular and cerebrovascular disease is not yet well established. Recent data suggest an increased risk of myocardial infarction and stroke in PD patients. Therefore, we designed a study to assess surrogate markers of cardiovascular and cerebrovascular risk in PD.

Methods

We conducted a case−control study comparing PD patients recruited from a Movement Disorders Unit with controls randomly invited from a primary healthcare center. All participants underwent a detailed clinical evaluation, including medical history, physical assessment, carotid ultrasound, blood and urine analysis, and 24‐h ambulatory blood pressure monitoring. The primary outcome was the carotid intima‐media thickness (CIMT).

Results

We included 102 participants in each study arm. No significant difference was found in the CIMT among groups (MD: 0.01, 95% CI: −0.02, 0.04). Carotid plaques were more frequent in PD patients (OR: 1.90, 95% CI: 1.02, 3.55), although the lipid profile was more favorable in this group (LDL MD: −18.75; 95% CI: −10.69, −26.81). Nocturnal systolic blood pressure was significantly higher in PD patients (MD: 4.37, 95% CI: 0.27, 8.47) and more than half of the PD patients were non‐dippers or reverse dippers (OR: 1.83, 95% CI: 1.04, 3.20).

Conclusion

We did not find a difference in CIMT between PD and controls. A higher frequency of carotid plaques and abnormal dipper profile supports the hypothesis that PD patients are not protected from cardiovascular and cerebrovascular disease.

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Authors:
Mariana Alves, Patrícia Pita Lobo, Linda Azevedo Kauppila, Leonor Rebordão, M. Manuela Cruz, Fátima Soares, João Cruz, Ana Tornada, Daniel Caldeira, Sofia Reimão, Victor Oliveira, José M. Ferro and Joaquim J. Ferreira

Article first published online:
16 Jun 2021 | DOI: 10.1111/ene.14938

ORIGINAL ARTICLE

Prevalence of multimorbidity and its impact on survival in people with motor neuron disease

Background and purpose

This study was undertaken to determine the prevalence of multimorbidity in people with motor neuron disease (MND) and to identify whether specific patterns of multimorbidity impact survival beyond age alone.

Methods

We performed a retrospective analysis of the Scottish national MND register from 1 January 2015 to 29 October 2019. People with amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy, or progressive bulbar palsy were included. We fitted latent class regression models incorporating comorbidities (class indicators), age, sex, and bulbar onset (covariates), and survival (distal outcome) with multimorbidity as a hypothesised latent variable. We also investigated the association between the Charlson Comorbidity Index and survival in Cox regression and compared its discrimination and calibration to age alone.

Results

A total of 937 people with MND were identified (median age = 67 years, 60.2% male); 64.8% ( = 515) had two or more comorbidities. We identified a subpopulation with high prevalence of cardiovascular disease, but when accounting for the relationship between age and individual comorbidities, there was no difference in survival. Both Charlson Comorbidity Index (hazard ratio [HR] per unit increase = 1.11, 95% confidence interval [CI] = 1.07–1.15, < 0.0001) and age (HR per year increase = 1.04, 95% CI = 1.03–1.05, < 0.0001) were significantly associated with survival, but discrimination was higher for age compared to Charlson Comorbidity Index (C‐index = 0.63 vs. 0.59).

Conclusions

Multimorbidity is common in MND, necessitating holistic interdisciplinary management, but age is the dominant predictor of prognosis in people with MND. Excluding people with MND and multimorbidity from trial participation may do little to homogenise the cohort in terms of survival potential and could harm generalisability.

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Authors:
Stella A. Glasmacher, Patrick K. A. Kearns, Juan Larraz, Lucy Stirland, Arpan R. Mehta, Judith Newton, Christopher J. Weir, Siddharthan Chandran, Suvankar Pal, Richard Davenport, David Thomson, Louise Murrie, Jennifer Preston, Govind Chavada, Robert J. Swingler, Danielle Leighton, Ian Morrison, George Gorrie, Callum Duncan, Myles Connor, David Simpson, Ondrej Dolezal, Katja Lassak, Antonella Benvenga, Javier Carod Artal, Andrew Bethell, Gillian Craig, Laura Cunningham, Moira Flett, Janice Hatrick, Helen Lennox, Laura Marshall, Alison McEleney, Kitty Millar, Suzanne Byrne, Susan Stewart, Dorothy Storey, Gowri Saravanan, Gill Stott and Carolyn Webber

Article first published online:
15 Jun 2021 | DOI: 10.1111/ene.14940

SHORT COMMUNICATION

Psychogenic non‐epileptic seizure‐status in patients admitted to the intensive care unit

Background

Psychogenic non‐epileptic seizure‐status (PNES‐status), defined by psychogenic non‐epileptic seizures (PNES) over 30 min, are often misdiagnosed as status epilepticus. We aimed to describe the features of patients who experienced PNES‐status, admitted to an intensive care unit (ICU).

Methods

We screened the patients hospitalized in our epilepsy unit during a 4‐year period, with a diagnosis of PNES‐status and ICU admission.

Results

Among 171 patients with PNES, we identified 25 patients (15%) who presented 39 episodes of PNES‐status leading to ICU admission. Some 76% of the patients were women. The median age at the time of the PNES‐status episode was 35 years. Half (48%) alleged a history of epilepsy, but epilepsy was confirmed in only 12%. A history of psychiatric disease was found in 68%. PNES were present in 85% of patients before PNES‐status, and semiology of PNES and PNES‐status was similar for 79% of the patients, including hyperkinetic movements in 95% of the episodes and suspected loss of consciousness in 87%. Benzodiazepines were administrated in 77% of the episodes, antiepileptic drugs in 87%, and antibiotherapy for a ICU‐related infection in 15% of the episodes. Oral intubation was performed in 41% of the episodes. Blood tests showed normal levels of creatine phosphokinase and leucocytes in 90% and 95% of the episodes, respectively. No epileptic activity was found during per‐event electroencephalography but interictal epileptic activity was found in 10% of the episodes.

Conclusion

Hyperkinetic PNES‐status should always be considered as a differential diagnosis of status epilepticus, with a high risk of iatrogenic consequences.

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Authors:
Nicolas Mezouar, Sophie Demeret, Jean Yves Rotge, Sophie Dupont and Vincent Navarro

Article first published online:
20 Jun 2021 | DOI: 10.1111/ene.14941

LETTERS TO THE EDITOR

Spinocerebellar ataxia type 21 () with tremor and dystonia

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Authors:
Carlos Henrique Ferreira Camargo, Aline K. C. Piva‐Silva, Renato Puppi Munhoz, Salmo Raskin and Hélio Afonso Ghizoni Teive

Article first published online:
23 Jun 2021 | DOI: 10.1111/ene.14944

COMMENTARY

Motoric cognitive risk syndrome: Next steps

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Authors:
Joe Verghese

Article first published online:
19 Jun 2021 | DOI: 10.1111/ene.14949

SHORT COMMUNICATION

Familial clustering of primary lateral sclerosis and amyotrophic lateral sclerosis: Supplementary evidence for a continuum

Background and purpose

Primary lateral sclerosis (PLS) is a motor neuron disorder characterized by a pure upper motor neuron degeneration in the bulbar and spinal regions. The key difference with amyotrophic lateral sclerosis (ALS) is the lower motor neuron system integrity. Despite important literature on this disease, the pathophysiology of PLS remains unknown, and the link with ALS still balances between a continuum and a separate entity from ALS.

Methods

We report nine families in which both PLS and ALS cases occurred, in general among first‐degree relatives.

Results

The patients with PLS and ALS had a typical disease presentation. Genetic studies revealed mutations in , and genes in two PLS patients and one ALS patient.

Conclusions

These results strongly support a phenotypic continuum between PLS and ALS.

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Authors:
Philippe Corcia, Christian Lunetta, Philippe Couratier, Patrick Vourc'h, Marta Gromicho, Claude Desnuelle, Marie‐Hélène Soriani, Susana Pinto and Mamede de Carvalho

Article first published online:
23 Jun 2021 | DOI: 10.1111/ene.14960

European Journal of Neurology

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LETTERS TO THE EDITOR

Two patients with dermatoneuro syndrome after influenza A infection

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ORIGINAL ARTICLE

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