cover image European Journal of Neurology

European Journal of Neurology

2024 - Volume 31
Issue 4 | April 2024
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ISSUE INFORMATION

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Article first published online:
13 Mar 2024 | DOI: 10.1111/ene.15880

LETTER TO THE EDITOR

Central European Group on Genetics of Movement Disorders

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Authors:
Miriam Ostrozovicova, Petr Dusek, Milan Grofik, Vladimir Han, Petr Holly, Robert Jech, Peter Klivenyi, Norbert Kovacs, Kristina Kulcsarova, Egon Kurca, Alexandra Lackova, Veronika Magocova, Jan Necpal, David Pinter, Evzen Ruzicka, Tereza Serranova, Katarzyna Smilowska, Igor Straka, Tatiana Svorenova, Gertrud Tamas, Peter Valkovic, Katerina Zarubova, Henry Houlden, Mie Rizig and Matej Skorvanek
Article first published online:
07 Dec 2023 | DOI: 10.1111/ene.16165

SHORT COMMUNICATION

Background and purpose

Influenza vaccination is associated with a longer‐term protective effect against stroke; however, it has a short‐term inflammatory response which may increase short‐term risk of stroke. The aim was to investigate the association between influenza vaccination and short‐term risk of stroke in adults.

Methods

Administrative data were obtained from the Alberta Health Care Insurance Plan for all adults in Alberta, Canada, from September 2009 to December 2018. The hazard of any stroke (acute ischaemic stroke, intracerebral haemorrhage, subarachnoid haemorrhage and transient ischaemic attack) within 3, 7, 14, 21 and 30 days of influenza vaccination compared to unexposed time was analysed using Andersen−Gill Cox models, with adjustment for age, sex, anticoagulant use, atrial fibrillation, chronic obstructive pulmonary disease, diabetes, hypertension, income quintile, and rural or urban home location.

Results

In the entire cohort consisting of 4,141,209 adults (29,687,899 person‐years), 1,769,565 (42.7%) individuals received at least one vaccination. In total 38,126 stroke events were recorded with 1309 occurring within 30 days of a vaccination event. Influenza vaccination was associated with a significantly reduced hazard of stroke within 3 days (hazard ratio [HR] 0.83, 95% confidence interval [CI] 0.73–0.93), 7 days (HR 0.87, 95% CI 0.80–0.95), 14 days (HR 0.87, 95% CI 0.81–0.93), 21 days (HR 0.85, 95% CI 0.80–0.91) and 30 days (HR 0.66, 95% CI 0.65–0.68).

Conclusions

An increased early risk associated with vaccination was not observed. The risk of stroke was reduced at all time points within 30 days after influenza vaccination.

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Authors:
Koji Tanaka, Andrew M. Demchuk, Shaun Malo, Michael D. Hill and Jessalyn K. Holodinsky
Article first published online:
20 Dec 2023 | DOI: 10.1111/ene.16172

SHORT COMMUNICATION

Background and purpose

Orolingual angioedema (OA) represents a rare but life‐threatening complication among patients with acute ischemic stroke treated with intravenous thrombolysis with alteplase. Novel agents, including icatibant, are recommended in resistant patients with alteplase‐induced OA who have failed to respond to first‐line therapies including corticosteroids, antihistamines, and/or adrenaline.

Methods

We present a patient with alteplase‐induced OA who showed substantial clinical improvement following the administration of icatibant.

Results

We describe a 71‐year‐old woman with known arterial hypertension under treatment with angiotensin‐converting enzyme inhibitor, who presented with acute ischemic stroke in the territory of the right middle cerebral artery and received intravenous alteplase. During intravenous thrombolysis, the case was complicated with OA without any response to standard anaphylactic treatment including corticosteroids, dimetindene, and adrenaline. Thirty minutes after symptom onset, icatibant, a synthetic selective bradykinin B2‐receptor antagonist, was administered subcutaneously. Substantial symptomatic resolution was observed only following the icatibant administration.

Conclusions

This case highlights the effectiveness of icatibant in the acute management of alteplase‐induced OA. In particular, icatibant administration, following first‐line therapies including corticosteroids, antihistamines, and/or adrenaline, may avert tracheostomy and intubation in resistant and refractory cases with OA following intravenous thrombolysis for acute ischemic stroke.

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Authors:
Aikaterini Theodorou, Evangelia‐Makrina Dimitriadou, Dimitrios Tzanetakos, Eleni Bakola, Maria Chondrogianni, Lina Palaiodimou, Anna Keramida, Sofia Vassilopoulou, Michael Makris, Georgios P. Paraskevas and Georgios Tsivgoulis
Article first published online:
28 Dec 2023 | DOI: 10.1111/ene.16173

SHORT COMMUNICATION

Background and purpose

Pulmonary arteriovenous malformations (PAVMs) may cause recurrent brain abscess. The primary aim was to determine the prevalence of PAVM amongst survivors of brain abscess. The proportion with cardiac right‐to‐left shunts was also assessed post hoc.

Methods

This was a cross‐sectional population‐based study of adult (≥18 years) survivors of cryptogenic bacterial brain abscess in Denmark from 2007 through 2016. Patients were invited for bubble‐echocardiography to detect vascular right‐to‐left shunting and, if abnormal, subsequent computed tomography thorax for diagnosis of PAVM. Data are presented as / (%) or median with interquartile range (IQR).

Results

Study participation was accepted by 47/157 (30%) eligible patients amongst whom two did not appear for scheduled bubble‐echocardiography. The median age of participants was 54 years (IQR 45–62) and 19/57 (33%) were females compared with 59 years (IQR 48–68,  = 0.05) and 41/85 females (48%,  = 0.22) in non‐participants. Bubble‐echocardiography was suggestive of shunt in 10/45 (22%) participants and PAVM was subsequently confirmed by computed tomography in one patient with grade 1 shunting. The corresponding prevalence of PAVM was 2% (95% confidence interval 0.06–11.8) amongst all examined participants. Another 9/45 (20%) were diagnosed with patent in persistent foramen ovale ( = 8) or atrial septum defect ( = 1), which is comparable with the overall prevalence of 25% amongst adults in the Danish background population.

Conclusions

Undiagnosed PAVM amongst adult survivors of cryptogenic bacterial brain abscess is rare but may be considered in select patients. The prevalence of cardiac right‐to‐left shunts amongst brain abscess patients corresponds to the prevalence in the general population.

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Authors:
Jacob Bodilsen, Trine Madsen, Christian Thomas Brandt, Katrine Müllertz, Lothar Wiese, Semra Turan Demirci, Hannah Elena Suhrs, Lykke Larsen, Sabine Ute Alice Gill, Birgitte Rønde Hansen, Brian Nilsson, Lars Haukali Omland, Emil Fosbøl, Anette Drøhse Kjeldsen and Henrik Nielsen
Article first published online:
08 Dec 2023 | DOI: 10.1111/ene.16176

ORIGINAL ARTICLE

Background and purpose

Long‐term consequences after status epilepticus (SE) represent an unsettled issue. We investigated the incidence of remote unprovoked seizures (RS) and drug‐resistant epilepsy (DRE) in a cohort of first‐ever SE survivors.

Methods

A retrospective, observational, and monocentric study was conducted on adult patients (age ≥ 14 years) with first SE who were consecutively admitted to the Modena Academic Hospital, Italy (September 2013–March 2022). Kaplan–Meier survival analyses were used to calculate the probability of seizure freedom following the index event, whereas Cox proportional hazard regression models were used to identify outcome predictors.

Results

A total of 279 patients were included, 57 of whom (20.4%) developed RS (mean follow‐up = 32.4 months). Cumulative probability of seizure freedom was 85%, 78%, and 68% respectively at 12 months, 2 years, and 5 years. In 45 of 57 patients (81%), the first relapse occurred within 2 years after SE. The risk of RS was higher in the case of structural brain damage (hazard ratio [HR] = 2.1, 95% confidence interval [CI] = 1.06–4.01), progressive symptomatic etiology (HR = 2.7, 95% CI = 1.44–5.16), and occurrence of nonconvulsive evolution in the semiological sequence of SE (HR = 2.9, 95% CI = 1.37–6.37). Eighteen of 57 patients (32%) developed DRE; the risk was higher in the case of super‐refractory ( = 0.006) and non‐convulsive SE evolution ( = 0.008).

Conclusions

The overall risk of RS was moderate, temporally confined within 2 years after the index event, and driven by specific etiologies and SE semiology. Treatment super‐refractoriness and non‐convulsive SE evolution were associated with DRE development.

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Authors:
Niccolò Orlandi, Giada Giovannini, Maria Cristina Cioclu, Niccolò Biagioli, Laura Madrassi, Anna Elisabetta Vaudano, Matteo Pugnaghi, Simona Lattanzi and Stefano Meletti
Article first published online:
23 Jan 2024 | DOI: 10.1111/ene.16177

ORIGINAL ARTICLE

Background and purpose

White matter lesions (WMLs) are frequent in sickle cell disease (SCD), with a prevalence described to be as high as 53% by age 30. Cerebrovascular regulation and cardiovascular autonomic regulation, more specifically the sympatho‐vagal balance, can be altered in SCD. In this study the association between WMLs, cerebrovascular regulation and sympatho‐vagal balance was assessed in SCD patients.

Methods and results

Sickle cell disease patients with no history of stroke were prospectively evaluated for cerebrovascular reactivity using the breath‐holding test (BHT), the sympatho‐vagal balance (ratio low frequency/high frequency [HF]) using heart rate variability parameters and cerebral autoregulation in the time domain using correlation index Mx, and arterial cerebral compliance based on continuous assessment of cerebral blood flow velocities using transcranial Doppler ultrasound and arterial blood pressure with photo‐plethysmography. WMLs were assessed with magnetic resonance imaging using Fazekas score grading and the presence of lacunes. Forty‐one patients (F/M 25/16) were included. Median age was 37.5 years (19–65). Twenty‐nine (70.7%) patients had SS genotype. Eleven patients had WMLs (26.8%). Patients with WMLs were significantly older ( < 0.001), had a lower HF ( < 0.005) and an impaired cerebral arterial compliance ( < 0.014). The receiver operating curve for the regression model including age and HF showed a higher area under the curve compared to age alone (0.946 vs. 0.876). BHT and Mx did not significantly differ between the two groups.

Conclusions

Lower parasympathetic activity and impaired cerebral arterial compliance were associated with WMLs in adults with SCD. This could potentially yield to a better understanding of pathophysiological parameters leading to premature cerebrovascular ageing in SCD.

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Authors:
Christophe Ferreira De Matos, Pierre Cougoul, Oana Maria Zaharie, Marc Kermorgant, Anne Pavy‐Le Traon, Celine Gales, Jean‐Michel Senard, Mathilde Strumia, Fabrice Bonneville and Nathalie Nasr
Article first published online:
02 Jan 2024 | DOI: 10.1111/ene.16183

ORIGINAL ARTICLE

Background and purpose

Post‐stroke aphasia is associated with a reduced quality of life (QoL) and higher risk of depression. Few studies have addressed the effect of coping with aphasia. Our aim is to evaluate the impact of post‐stroke aphasia on self‐reported QoL and symptoms of depression.

Methods

This was a cross‐sectional prospective case–control study. Cases involved patients with post‐stroke aphasia included in the DULCINEA trial (NCT04289493). Healthy controls were recruited using snowball sampling. All subjects completed the following questionnaires: General Health Questionnaire (GHQ‐12), Stroke Aphasia Quality of Life Scale (SAQOL‐39), Communicative Activity Log (CAL) and Stroke Aphasic Depression Questionnaire (SADQ‐10).

Results

Twenty‐three patients (eight women; mean age 62.9 years) and 73 controls (42 women; mean age 53.7 years) were included. Cases scored lower than controls in perception of health (GHQ‐12: median 3 [IQR 1; 6] vs. 0 [IQR 0; 2]) and perception of QoL (SAQOL‐39: median 3.6 [IQR 3.3; 40] vs. 4.6 [IQR 4.2; 4.8]). Functional communication (CAL: median 135 [IQR 122; 148] vs. 94 [IQR 74; 103]) and SAQOL‐39 communication subscale (median 2.7 [IQR 2.1; 3.2] vs. 4.8 [IQR 4.6; 5.0]) were also significantly lower in the case group. Notably, cases reported fewer depressive symptoms than controls (SADQ‐10: median 11 [IQR 9; 15] vs. 13 [IQR 11; 16];  = 0.016). A mediational analysis revealed that the relationship between post‐stroke aphasia and depression was not mediated by functional communication.

Conclusions

Although communication difficulties impact the QoL of patients with post‐stroke aphasia, such patients report fewer depressive symptoms on the SADQ‐10 scale than healthy people, with no differences in scores related to social participation.

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Authors:
Nereida Bueno‐Guerra, Marta Provencio, Aida Tarifa‐Rodríguez, Ana Navarro, Cristian Sempere‐Iborra, Pablo Jordi, Elena de Celis‐Ruiz, María Alonso de Leciñana, Marta Martín‐Alonso, Ricardo Rigual, Gerardo Ruiz‐Ares, Jorge Rodríguez‐Pardo, Javier Virués‐Ortega and Blanca Fuentes
Article first published online:
14 Dec 2023 | DOI: 10.1111/ene.16184

LETTER TO THE EDITOR

Acute truncal ataxia without nystagmus

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Authors:
Sergio Carmona, Carlos Martínez, Guillermo Zalazar, Nehzat Koohi and Diego Kaski
Article first published online:
21 Dec 2023 | DOI: 10.1111/ene.16185

ORIGINAL ARTICLE

Background and purpose

Susac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long‐term neuropsychological outcomes of patients with SuS.

Methods

Thirteen patients with SuS (mean [SD] age 39.5 [11.1] years) were enrolled at the Ghent University Hospital by their treating neurologist. The cognitive functioning and emotional well‐being of each patient was evaluated by means of a thorough neuropsychological test battery at baseline and after 2 years. Follow‐up testing after 2 years was performed in 11 patients (mean [SD] age 42.2 [11.5] years).

Results

Patients showed normal neuropsychological test results at a group level, both at baseline and follow‐up testing. Significant improvements over time were found for information processing speed, verbal recognition, and semantic and phonological fluency. Individual test results showed interindividual variability at baseline, with most impairments being in attention, executive functioning and language, which improved after a 2‐year period. In addition, patients reported significantly lower mental and physical well‐being, both at baseline and follow‐up testing.

Conclusions

Our results suggest that neuropsychological dysfunction in SuS is limited at a group level and improves over time. Nonetheless, individual test results reveal interindividual variability, making cognitive screening essential. Furthermore, a high psycho‐emotional burden of the disease was reported, for which screening and follow‐up are necessary.

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Authors:
Tineke Van Vrekhem, Marijke Miatton, Dimitri Hemelsoet, Liesbeth Van Hijfte, Cathérine Dekeyser, Julie De Zaeytijd, Veroniek Van Driessche, Helen Van Hoecke, Leen Maes and Guy Laureys
Article first published online:
02 Feb 2024 | DOI: 10.1111/ene.16186

ORIGINAL ARTICLE

Background and purpose

Inhibition of the neonatal Fc receptor (FcRn) for IgG is a promising new therapeutic strategy for antibody‐mediated disorders. We report our real‐life experience with efgartigimod (EFG) in 19 patients with generalized myasthenia gravis (gMG) along a clinical follow‐up of 14 months.

Methods

EFG was administered according to the GENERATIVE protocol (consisting of a Fixed period of two treatment cycles [given 1 month apart] of four infusions at weekly intervals, followed by a Flexible period of re‐cycling in case of worsening). Eight patients were positive for acetylcholine receptor antibody, four for muscle‐specific tyrosine kinase antibody, and two for lipoprotein‐related protein 4 antibody, and five were classified as triple negative. Efficacy of EFG was assessed by the Myasthenia Gravis Activities of Daily Living, Myasthenia Gravis Composite, and Quantitative Myasthenia Gravis scales.

Results

Fifty‐three percent of patients needed three treatment cycles, 26% needed four, and 21% needed five along the 14‐month clinical follow‐up. Meaningful improvement was observed at the end of each cycle with the clinical scores adopted. EFG had a dramatic effect on disease course, as during the year before treatment eight of 19 patients (42%) were hospitalized, and 15 of 19 (79%) needed treatment with plasma exchange or immunoglobulins; three of 19 (16%) were admitted to the intensive care unit. During EFG, none of the patients was hospitalized and only one patient required plasma exchange and intravenous immunoglobulins. No major side effects or infusion‐related reactions occurred.

Conclusions

We observed that EFG was safe and modified significantly the course of the disease along a 14‐month follow‐up. Our experience strengthens the role of FcRn inhibition as an effective new tool for long‐term treatment of gMG.

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Authors:
Rita Frangiamore, Elena Rinaldi, Fiammetta Vanoli, Francesca Andreetta, Emilio Ciusani, Silvia Bonanno, Lorenzo Maggi, Annamaria Gallone, Anna Colasuonno, Irene Tramacere, Marta Cheli, Alessandro Pinna, Renato Mantegazza and Carlo Antozzi
Article first published online:
02 Jan 2024 | DOI: 10.1111/ene.16189

ORIGINAL ARTICLE

Background and purpose

There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated.

Methods

We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP.

Results

According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory‐predominant CIDP (7%), 10 motor CIDP (3%), and eight motor‐predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F‐wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor‐predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory‐predominant CIDP did.

Conclusions

The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.

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Authors:
Alberto De Lorenzo, Giuseppe Liberatore, Pietro Emiliano Doneddu, Fiore Manganelli, Dario Cocito, Chiara Briani, Raffaella Fazio, Anna Mazzeo, Angelo Schenone, Vincenzo Di Stefano, Giuseppe Cosentino, Girolama Alessandra Marfia, Luana Benedetti, Marinella Carpo, Massimiliano Filosto, Giovanni Antonini, Angelo Maurizio Clerici, Marco Luigetti, Sabrina Matà, Tiziana Rosso, Marta Lucchetta, Gabriele Siciliano, Giuseppe Lauria Pinter, Guido Cavaletti, Maurizio Inghilleri, Teresa Cantisani, Francesca Notturno, Dario Ricciardi, Francesco Habetswallner, Emanuele Spina, Erdita Peci, Alessandro Salvalaggio, Yuri Falzone, Camilla Strano, Luca Gentile, Elisa Vegezzi, Giorgia Mataluni, Stefano Cotti Piccinelli, Luca Leonardi, Angela Romano, Eduardo Nobile‐Orazio, Pietro Emiliano Doneddu, Alberto De Lorenzo, Giuseppe Liberatore, Eduardo Nobile ‐Orazio, Dario Cocito, Fiore Manganelli, Emanuele Spina, Enrica Pisano, Lucio Santoro, Daniele Velardo, Camilla Strano, Raffaella Fazio, Marta Ruiz, Mario Cacciavillani, Francesca Castellani, Chiara Briani, Stefano Cotti Piccinelli, Filomena Caria, Massimiliano Filosto, Elisa Bianchi, Ettore Beghi, Elena Pinuccia Verrengia, Stefano Jann, Antonio Toscano, Luca Gentile, Massimo Russo, Anna Mazzeo, Luca Leonardi, Giovanni Antonini, Giuseppe Cosentino, Ilaria Callegari, Andrea Cortese, Giorgia Mataluni, Girolama Alessandra Marfia, Angelo Maurizio Clerici, Federica Scrascia, Marinella Carpo, Angelo Schenone, Luana Benedetti, Corrado Cabona, Alessandro Beronio, Erika Schirinzi, Gabriele Siciliano, Marco Luigetti, Patrizia Dacci, Giuseppe Lauria, Tiziana Rosso, Claudia Balducci, Guido Cavaletti, Mario Sabatelli and Erdita Peci
Article first published online:
02 Jan 2024 | DOI: 10.1111/ene.16190

SHORT COMMUNICATION

Background and purpose

The purpose of this study was to evaluate the possible correlation between SARS‐CoV‐2 vaccines and the onset of neurological syndromes. The aim was to challenge the association between SARS‐CoV‐2 vaccinations and the onset of acute functional neurological disorders (FNDs) compared to other neurological syndromes in hospitalized patients.

Methods

In this prospective cohort study, all adult inpatients consecutively admitted to a tertiary neurological centre were included. The prevalence and characteristics of neurological syndromes were compared between unvaccinated and vaccinated cases stratified according to the onset from vaccination. The study involved 843 subjects, namely 411 unvaccinated (UVC) and 432 vaccinated cases; these groups were comparable for demographics and clinical diagnosis distribution.

Results

Compared to UVC, subjects hospitalized within the first 30 days from vaccine exhibited higher prevalence of FNDs (12.3% vs. 3.6%; odds ratio 4.2, 95% confidence interval 1.6–11.1) and headache (10.8% vs. 5%; odds ratio 4.1, 95% confidence interval 1.9–8.8) but no other neurological syndromes. The FND cases following vaccinations showed similar premorbid conditions and severity but a higher percentage of sensory symptoms and pain compared to UVC FND cases.

Conclusions

SARS‐CoV‐2 vaccination is associated with a significant short‐term increased risk of FND and headache requiring hospitalization in an acute neurological setting.

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Authors:
Andrea Pilotto, Marcello Catania, Irene Mattioli, Nicola Zoppi, Giulia Ceccardi, Renata Rao, Stefano Gipponi, Mauro Magoni, Massimo Gamba and Alessandro Padovani
Article first published online:
02 Jan 2024 | DOI: 10.1111/ene.16191

ORIGINAL ARTICLE

Background and purpose

Diagnosing small fiber neuropathies can be challenging. To address this issue, whether serum neurofilament light chain (sNfL) could serve as a potential biomarker of damage to epidermal Aδ‐ and C‐fibers was tested.

Methods

Serum NfL levels were assessed in 30 patients diagnosed with small fiber neuropathy and were compared to a control group of 19 healthy individuals. Electrophysiological studies, quantitative sensory testing and quantification of intraepidermal nerve fiber density after skin biopsy were performed in both the proximal and distal leg.

Results

Serum NfL levels were not increased in patients with small fiber neuropathy compared to healthy controls (9.1 ± 3.9 and 9.4 ± 3.8,  = 0.83) and did not correlate with intraepidermal nerve fiber density at the lateral calf or lateral thigh or with other parameters of small fiber impairment.

Conclusion

Serum NfL levels cannot serve as a biomarker for small fiber damage.

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Authors:
Panoraia Baka, Livia Steenken, Fabiola Escolano‐Lozano, Falk Steffen, Aikaterini Papagianni, Claudia Sommer, Esther Pogatzki‐Zahn, Silke Hirsch, Maria Protopapa, Stefan Bittner and Frank Birklein
Article first published online:
08 Jan 2024 | DOI: 10.1111/ene.16192

ORIGINAL ARTICLE

Background and purpose

Previous studies have examined the risk of stroke in patients with Parkinson disease (PD), but the incidence of PD onset among stroke patients and its risk according to severity of poststroke disabilities have scarcely been investigated. This study aims to determine whether the risk of PD is increased among stroke patients using a retrospective cohort with a large population‐based database.

Methods

We used data collected by the Korean National Health Insurance Service from 2010 to 2018 and examined 307,361 stroke patients and 380,917 sex‐ and age‐matched individuals without stroke to uncover the incidence of PD. Cox proportional hazards regression was used to calculate the hazard ratio (HR) and 95% confidence interval (CI), and the risk of PD was compared according to presence and severity of disability.

Results

During 4.31 years of follow‐up, stroke patients had a 1.67 times higher risk of PD compared to individuals without stroke (adjusted HR = 1.67, 95% CI = 1.57–1.78). The risk of PD was greater among stroke patients with disabilities than among those without disabilities, even after adjustment for multiple covariates (adjusted HR = 1.72, 95% CI = 1.55–1.91; and adjusted HR = 1.66, 95% CI = 1.56–1.77, respectively).

Conclusions

Our study demonstrated an increased risk of PD among stroke patients. Health professionals need to pay careful attention to detecting movement disorders as clues for diagnosing PD.

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Authors:
Hea Lim Choi, Jong Hyeon Ahn, Won Hyuk Chang, Wonyoung Jung, Bong Sung Kim, Kyungdo Han, Jinyoung Youn and Dong Wook Shin
Article first published online:
02 Jan 2024 | DOI: 10.1111/ene.16194

ORIGINAL ARTICLE

Background and purpose

The integration of artificial intelligence (AI) in healthcare has the potential to revolutionize patient care and clinical decision‐making. This study aimed to explore the reliability of large language models in neurology by comparing the performance of an AI chatbot with neurologists in diagnostic accuracy and decision‐making.

Methods

A cross‐sectional observational study was conducted. A pool of clinical cases from the American Academy of Neurology's Question of the Day application was used as the basis for the study. The AI chatbot used was ChatGPT, based on GPT‐3.5. The results were then compared to neurology peers who also answered the questions—a mean of 1500 neurologists/neurology residents.

Results

The study included 188 questions across 22 different categories. The AI chatbot demonstrated a mean success rate of 71.3% in providing correct answers, with varying levels of proficiency across different neurology categories. Compared to neurology peers, the AI chatbot performed at a similar level, with a mean success rate of 69.2% amongst peers. Additionally, the AI chatbot achieved a correct diagnosis in 85.0% of cases and it provided an adequate justification for its correct responses in 96.1%.

Conclusions

The study highlights the potential of AI, particularly large language models, in assisting with clinical reasoning and decision‐making in neurology and emphasizes the importance of AI as a complementary tool to human expertise. Future advancements and refinements are needed to enhance the AI chatbot's performance and broaden its application across various medical specialties.

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Authors:
Ângelo Fonseca, Axel Ferreira, Luís Ribeiro, Sandra Moreira and Cristina Duque
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16195

ORIGINAL ARTICLE

Background and purpose

In acute spinal cord injury (SCI), magnetic resonance imaging (MRI) reveals tissue bridges and neurodegeneration for 2 years. This 5‐year study aims to track initial lesion changes, subsequent neurodegeneration, and their impact on recovery.

Methods

This prospective longitudinal study enrolled acute SCI patients and healthy controls who were assessed clinically—and by MRI—regularly from 3 days postinjury up to 60 months. We employed histologically cross‐validated quantitative MRI sequences sensitive to volume, myelin, and iron changes, thereby reflecting indirectly processes of neurodegeneration and neuroinflammation. General linear models tracked lesion and remote changes in volume, myelin‐ and iron‐sensitive magnetic resonance indices over 5 years. Associations between lesion, degeneration, and recovery (using the Spinal Cord Independence Measure [SCIM] questionnaire and the International Standards for Neurological Classification of Spinal Cord Injury total motor score) were assessed.

Results

Patients' motor scores improved by an average of 12.86 (95% confidence interval [CI] = 6.70–19.00) points, and SCIM by 26.08 (95% CI = 17.00–35.20) points. Within 3–28 days post‐SCI, lesion size decreased by more than two‐thirds (3 days: 302.52 ± 185.80 mm, 28 days: 76.77 ± 88.62 mm), revealing tissue bridges. Cervical cord and corticospinal tract volumes transiently increased in SCI patients by 5% and 3%, respectively, accompanied by cervical myelin decreases and iron increases. Over time, progressive atrophy was observed in both regions, which was linked to early lesion dynamics. Tissue bridges, reduced swelling, and myelin content decreases were predictive of long‐term motor score recovery and improved SCIM score.

Conclusions

Studying acute changes and their impact on longer follow‐up provides insights into SCI trajectory, highlighting the importance of acute intervention while indicating the potential to influence outcomes in the later stages.

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Authors:
Tim M. Emmenegger, Dario Pfyffer, Armin Curt, Simon Schading‐Sassenhausen, Markus Hupp, John Ashburner, Karl Friston, Nikolaus Weiskopf, Alan Thompson and Patrick Freund
Article first published online:
23 Jan 2024 | DOI: 10.1111/ene.16196

SHORT COMMUNICATION

Background and purpose

Despite it being an immunotherapy‐responsive neurological syndrome, patients with autoimmune encephalitis (AE) frequently exhibit residual neurobehavioural features. Here, we report criminal behaviours as a serious and novel postencephalitic association.

Methods

This retrospective cohort study included 301 AE patients. Five of who committed crimes underwent direct assessments and records review alongside autoantibody studies.

Results

Five of 301 patients (1.7%) with AE exhibited criminal behaviours, which included viewing child pornography ( = 3), repeated shoplifting, and conspiracy to commit murder. All five were adult males, with LGI1 autoantibodies ( = 3), CASPR2 autoantibodies, or seronegative AE. None had evidence of premorbid antisocial personality traits or psychiatric disorders. Criminal behaviours began a median of 18 months (range = 15 months–12 years) after encephalitis onset. At the time of crimes, two patients were immunotherapy‐naïve, three had been administered late immunotherapies (at 5 weeks–4 months), many neurobehavioural features persisted, and new obsessive behaviours had appeared. However, cognition, seizure, and disability measures had improved, alongside reduced autoantibody levels.

Conclusions

Criminal behaviours are a rare, novel, and stigmatizing residual neurobehavioural phenotype in AE, with significant social and legal implications. With caution towards overattribution, we suggest they occur as part of a postencephalitis limbic neurobehavioural syndrome.

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Authors:
Sophia Michael, James Varley, Robyn Williams, Tomasz Bajorek, Ava Easton and Sarosh R. Irani
Article first published online:
08 Jan 2024 | DOI: 10.1111/ene.16197

ORIGINAL ARTICLE

Background and purpose

It is unknown whether changes to the peripheral nervous system following spinal cord injury (SCI) are relevant for functional recovery or the development of neuropathic pain below the level of injury. Magnetic resonance neurography (MRN) at 3 T allows detection and localization of structural and functional nerve damage. This study aimed to combine MRN and clinical assessments in individuals with chronic SCI and nondisabled controls.

Methods

Twenty participants with chronic SCI and 20 controls matched for gender, age, and body mass index underwent MRN of the L5 dorsal root ganglia (DRG) and the sciatic nerve. DRG volume, sciatic nerve mean cross‐sectional area (CSA), fascicular lesion load, and fractional anisotropy (FA), a marker for functional nerve integrity, were calculated. Results were correlated with clinical assessments and nerve conduction studies.

Results

Sciatic nerve CSA and lesion load were higher (21.29 ± 5.82 mm vs. 14.08 ± 4.62 mm,  < 0.001; and 8.70 ± 7.47% vs. 3.60 ± 2.45%,  < 0.001) in individuals with SCI compared to controls, whereas FA was lower (0.55 ± 0.11 vs. 0.63 ± 0.08,  = 0.022). DRG volumes were larger in individuals with SCI who suffered from neuropathic pain compared to those without neuropathic pain (223.7 ± 53.08 mm vs. 159.7 ± 55.66 mm,  = 0.043). Sciatic MRN parameters correlated with electrophysiological results but did not correlate with the extent of myelopathy or clinical severity of SCI.

Conclusions

Individuals with chronic SCI are subject to a decline of structural peripheral nerve integrity that may occur independently from the clinical severity of SCI. Larger volumes of DRG in SCI with neuropathic pain support existing evidence from animal studies on SCI‐related neuropathic pain.

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Authors:
Johann M. E. Jende, Laura Heutehaus, Fabian Preisner, Christina M. Verez Sola, Christoph M. Mooshage, Sabine Heiland, Rüdiger Rupp, Martin Bendszus, Norbert Weidner, Felix T. Kurz and Steffen Franz
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16198

ORIGINAL ARTICLE

Background and purpose

Demographics, clinical characteristics, stroke mechanisms and long‐term outcomes were compared between acute ischaemic stroke (AIS) patients with active cancer (AC) versus non‐cancer patients.

Methods

Using data from 2003 to 2021 in the Acute STroke Registry and Analysis of Lausanne, a retrospective cohort study was performed comparing patients with AC, including previously known and newly diagnosed cancers, with non‐cancer patients. Patients with inactive cancer were excluded. Outcomes were the modified Rankin Scale (mRS) score at 3 months, death and cerebrovascular recurrences at 12 months before and after propensity score matching.

Results

Amongst 6686 patients with AIS, 1065 (15.9%) had a history of cancer. After excluding 700 (10.4%) patients with inactive cancer, there were 365 (5.5%) patients with AC and 5621 (84%) non‐cancer AIS patients. Amongst AC patients, 154 (42.2%) strokes were classified as cancer related. In multivariable analysis, patients with AC were older (adjusted odds ratio [aOR] 1.02, 95% confidence interval [CI] 1.00–1.03), had fewer vascular risk factors and were 48% less likely to receive reperfusion therapies (aOR 0.52, 95% CI 0.35–0.76). Three‐month mRS scores were not different in AC patients (aOR 2.18, 95% CI 0.96–5.00). At 12 months, death (adjusted hazard ratio 1.91, 95% CI 1.50–2.43) and risk of cerebrovascular recurrence (sub‐distribution hazard ratio 1.68, 95% CI 1.22–2.31) before and after propensity score matching were higher in AC patients.

Conclusions

In a large institutional registry spanning nearly two decades, AIS patients with AC had less past cerebrovascular disease but a higher 1‐year risk of subsequent death and cerebrovascular recurrence compared to non‐cancer patients. Antithrombotic medications at discharge may reduce this risk in AC patients.

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Authors:
Gianluca Costamagna, Andreas F. Hottinger, Haralampos Milionis, Alexander Salerno, Davide Strambo, Francoise Livio, Babak B. Navi and Patrik Michel
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16200

SHORT COMMUNICATION

Background and purpose

Resting‐state electroencephalography (EEG) holds promise for assessing brain networks in amyotrophic lateral sclerosis (ALS). We investigated whether neural β‐band oscillations in the sensorimotor network could serve as an objective quantitative measure of progressive motor impairment and functional disability in ALS patients.

Methods

Resting‐state EEG was recorded in 18 people with ALS and 38 age‐ and gender‐matched healthy controls. We estimated source‐localized β‐band spectral power in the sensorimotor cortex. Clinical evaluation included lower (LMN) and upper motor neuron scores, Amyotrophic Lateral Sclerosis Functional Rating Scale–Revised score, fine motor function (FMF) subscore, and progression rate. Correlations between clinical scores and β‐band power were analysed and corrected using a false discovery rate of  = 0.05.

Results

β‐Band power was significantly lower in people with ALS than controls ( = 0.004), and correlated with LMN score ( = −0.65,  = 0.013), FMF subscore ( = −0.53,  = 0.036), and FMF progression rate ( = 0.52,  = 0.036).

Conclusions

β‐Band spectral power in the sensorimotor cortex reflects clinically evaluated motor impairment in ALS. This technology merits further investigation as a biomarker of progressive functional disability.

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Authors:
Stefan Dukic, Antonio Fasano, Amina Coffey, Teresa Buxó, Roisin McMackin, Rangariroyashe Chipika, Mark Heverin, Peter Bede, Muthuraman Muthuraman, Madeleine Lowery, Richard G. Carson, Orla Hardiman and Bahman Nasseroleslami
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16201

ORIGINAL ARTICLE

Background and purpose

Identifying patients with inflammatory motor neuropathies (IMNs) is warranted since effective treatments are available and the prognosis of these patients differs from that of amyotrophic lateral sclerosis patients.

Methods

Between January 2019 and May 2022, 102 consecutive treatment‐naïve lower motor neuron syndrome (LMNS) patients were recruited; these patients were suspected of having multifocal motor neuropathy, pure motor chronic inflammatory demyelinating polyneuropathy or amyotrophic lateral sclerosis with initial lower motor neuron presentation. Neuromuscular ultrasound (US) and nerve conduction studies (NCSs) were conducted at baseline. Relevant diagnostic investigations were performed if clinically warranted. The proposed US evidence of IMN was as follows: (i) nerve enlargement at ≥1 of the predetermined sites or (ii) absence of high intensity fasciculations in predefined muscle groups. Final diagnoses were made by experienced physicians after a prolonged follow‐up period (≥12 months). IMN patients were defined as LMNS patients who experienced convincing improvements in response to immunotherapies. IMN patients without electrodiagnostic demyelinating features were diagnosed with treatment‐responsive LMNS (TR‐LMNS).

Results

In total, 16 patients were classified as IMN, including nine chronic inflammatory demyelinating polyneuropathy/multifocal motor neuropathy patients and seven TR‐LMNS patients. Six TR‐LMNS patients were identified by neuromuscular US. The sensitivity and specificity of NCSs, nerve US and muscle US were 56.3% and 100%, 43.8% and 90.7% and 68.8% and 97.7%, respectively. When these three modalities were combined, the sensitivity and specificity were 93.8% and 88.4%, respectively.

Conclusion

Neuromuscular US studies are supplementary modalities to NCSs, and the combined use of these techniques might improve the identification of IMNs in LMNS patients.

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Authors:
Lei Zhang, Yi Li, Jingwen Niu, Nan Hu, Jianfeng Ding, Liying Cui and Mingsheng Liu
Article first published online:
18 Jan 2024 | DOI: 10.1111/ene.16202

ORIGINAL ARTICLE

Background and purpose

According to the latest European guidelines, discontinuation of monoclonal antibodies against calcitonin gene‐related peptide (anti‐CGRP MAb) may be considered after 12–18 months of treatment. However, some patients may worsen after discontinuation. In this study, we assessed the response following treatment resumption.

Methods

This was a prospective study conducted in 14 Headache Units in Spain. We included patients with response to anti‐CGRP MAb with clinical worsening after withdrawal and resumption of treatment. Numbers of monthly migraine days (MMD) and monthly headache days (MHD) were obtained at four time points: before starting anti‐CGRP MAb (T‐baseline); last month of first treatment period (T‐suspension); month of restart due to worsening (T‐worsening); and 3 months after resumption (T‐reintroduction). The response rate to resumption was calculated. Possible differences among periods were analysed according to MMD and MHD.

Results

A total of 360 patients, 82% women, with a median (interquartile range [IQR]) age at migraine onset of 18 (12) years. The median (IQR) MHD at T‐baseline was 20 (13) and MMD was 5 (6); at T‐suspension, the median (IQR) MHD was 5 (6) and MMD was 4 (5); at T‐worsening, the median (IQR) MHD was 16 (13) and MMD was 12 (6); and at T‐reintroduction, the median (IQR) MHD was 8 (8) and MHD was 5 (5). In the second period of treatment, a 50% response rate was achieved by 57.4% of patients in MHD and 65.8% in MMD. Multivariate models showed significant differences in MHD between the third month after reintroduction and last month before suspension of first treatment period ( < 0.001).

Conclusion

The results suggest that anti‐CGRP MAb therapy is effective after reintroduction. However, 3 months after resumption, one third of the sample reached the same improvement as after the first treatment period.

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Authors:
Celia Romero del Rincón, Alicia Gonzalez‐Martinez, Sonia Quintas, David García‐Azorín, Iris Fernández Lázaro, Angel Luis Guerrero‐Peral, Yesica Gonzalez Osorio, Sonia Santos‐Lasaosa, Carmen González Oria, Norberto Sánchez Rodríguez, Fernando Iglesias Díez, Ana Echavarría Íñiguez, Sendoa Gil Luque, Mariano Huerta‐Villanueva, Sergio Campoy Díaz, Albert Muñoz‐Vendrell, Alberto Lozano Ros, Antonio Sánchez‐Soblechero, Fernando Velasco Juanes, Izaro Kortazar‐Zubizarreta, Amaya Echeverría, Jaime Rodríguez‐Vico, Alex Jaimes Sánchez, Andrea Gómez García, Noemí Morollón Sánchez‐Mateos, Robert Belvis, María Pilar Navarro Pérez, Juan Carlos García‐Moncó, María Rocío Álvarez Escudero, Nuria Montes and Ana Beatriz Gago‐Veiga
Article first published online:
25 Jan 2024 | DOI: 10.1111/ene.16203

ORIGINAL ARTICLE

Background and purpose

In 2016, we concluded a randomized controlled trial testing 1 mg rasagiline per day add‐on to standard therapy in 252 amyotrophic lateral sclerosis (ALS) patients. This article aims at better characterizing ALS patients who could possibly benefit from rasagiline by reporting new subgroup analysis and genetic data.

Methods

We performed further exploratory in‐depth analyses of the study population and investigated the relevance of single nucleotide polymorphisms (SNPs) related to the dopaminergic system.

Results

Placebo‐treated patients with very slow disease progression (loss of Amyotrophic Lateral Sclerosis Functional Rating Scale–Revised [ALSFRS‐R] per month before randomization of ≤0.328 points) showed a per se survival probability after 24 months of 0.85 (95% confidence interval = 0.65–0.94). The large group of intermediate to fast progressing ALS patients showed a prolonged survival in the rasagiline group compared to placebo after 6 and 12 months ( = 0.02,  = 0.04), and a reduced decline of ALSFRS‐R after 18 months ( = 0.049). SNP genotypes in the gene and gene did not show clear associations with rasagiline treatment effects.

Conclusions

These results underline the need to consider individual disease progression at baseline in future ALS studies. Very slow disease progressors compromise the statistical power of studies with treatment durations of 12–18 months using clinical endpoints. Analysis of and SNPs revealed no clear relationship to any outcome parameter. More insights are expected from future studies elucidating whether patients with genotype (Rs2283265) show a pronounced benefit from treatment with rasagiline, pointing to the opportunities precision medicine could open up for ALS patients in the future.

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Authors:
Joachim Schuster, Jens Dreyhaupt, Karla Mönkemöller, Luc Dupuis, Stéphane Dieterlé, Jochen H. Weishaupt, Jan Kassubek, Susanne Petri, Thomas Meyer, Julian Grosskreutz, Berthold Schrank, Matthias Boentert, Alexander Emmer, Andreas Hermann, Daniel Zeller, Johannes Prudlo, Andrea S. Winkler, Torsten Grehl, Michael T. Heneka, Siw Johannesen, Bettina Göricke, Simon Witzel, Johannes Dorst and Albert C. Ludolph
Article first published online:
19 Jan 2024 | DOI: 10.1111/ene.16204

ORIGINAL ARTICLE

Background and purpose

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease with humoral and cellular autoimmunity causing demyelination of peripheral nerves, commonly treated with intravenous immunoglobulins (IVIg). The neonatal Fc receptor (FcRn), encoded by the gene, prevents the degradation of immunoglobulin G (IgG) by recycling circulating IgG. A variable number of tandem repeat (VNTR) polymorphism in the promoter region of the gene is associated with different expression levels of mRNA and protein. Thus, patients with genotypes associated with relatively low FcRn expression may show a poorer treatment response to IVIg due to increased IVIg degradation.

Methods

VNTR genotypes were analyzed in 144 patients with CIDP. Patients' clinical data, including neurological scores and treatment data, were collected as part of the Immune‐Mediated Neuropathies Biobank registry.

Results

Most patients ( = 124, 86%) were VNTR 3/3 homozygotes, and 20 patients (14%) were VNTR 2/3 heterozygotes. Both VNTR 3/3 and VNTR 2/3 genotype groups showed no difference in clinical disability and immunoglobulin dosage. However, patients with a VNTR 2 allele were more likely to receive subcutaneous immunoglobulins (SCIg) than patients homozygous for the VNTR 3 allele (25% vs. 9.7%,  = 0.02) and were more likely to receive second‐line therapy (75% vs. 54%,  = 0.05).

Conclusions

The VNTR 2/3 genotype is associated with the administration of SCIg, possibly reflecting a greater benefit from SCIg due to more constant immunoglobulin levels without lower IVIg levels between the treatment circles. Also, the greater need for second‐line treatment in VNTR 2/3 patients could be an indirect sign of a lower response to immunoglobulins.

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Authors:
Anna Lena Fisse, Emelie Schäfer, Alina Hieke, Maximilian Schröder, Rafael Klimas, Jil Brünger, Sophie Huckemann, Thomas Grüter, Melissa Sgodzai, Christiane Schneider‐Gold, Ralf Gold, Huu Phuc Nguyen, Kalliopi Pitarokoili, Jeremias Motte and Larissa Arning
Article first published online:
11 Jan 2024 | DOI: 10.1111/ene.16205

ORIGINAL ARTICLE

Background

Alpha‐synuclein (α‐Syn) oligomers and fibrils have been shown to augment the aggregation of TAR DNA‐binding Protein 43 (TDP‐43) monomers in vitro, supporting the idea that TDP‐43 proteinopathies such as ALS may be modulated by the presence of toxic forms of α‐Syn. Recently, parkinsonian features were reported in a study of European patients and Lewy bodies have been demonstrated pathologically in a similar series of patients. Based on these and other considerations, we sought to determine whether seed‐competent α‐Syn can be identified in spinal fluid of patients with ALS including familial, sporadic, and Guamanian forms of the disease.

Methods

Based on the finding that α‐Syn has been found to be a prion‐like protein, we have utilized a validated α‐Synuclein seed amplification assay to determine if seed‐competent α‐Syn could be detected in the spinal fluid of patients with ALS.

Results

Toxic species of α‐Syn were detected in CSF in 18 of 127 ALS patients, 5 of whom were from Guam. Two out of twenty six samples from patients with C9orf72 variant ALS had positive seed‐amplification assays (SAAs). No positive tests were noted in superoxide dismutase type 1 ALS subjects ( = 14). The SAA was negative in 31 control subjects.

Conclusions

Our findings suggest that a sub‐group of ALS occurs in which self‐replicating α‐Syn is detectable and likely contributes to its pathogenesis. This finding may have implications for the diagnosis and treatment of this disorder.

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Authors:
Richard Smith, Hanna Hovren, Robert Bowser, Nadine Bakkar, Ralph Garruto, Albert Ludolph, John Ravits, Lia Gaertner, Davan Murphy and Russ Lebovitz
Article first published online:
25 Jan 2024 | DOI: 10.1111/ene.16206

ORIGINAL ARTICLE

Background and purpose

Myotonia congenita (MC) is a muscle channelopathy in which pathogenic variants in a key sarcolemmal chloride channel Gene () cause myotonia. This study used muscle magnetic resonance imaging (MRI) to quantify contractile properties and fat replacement of muscles in a Danish cohort of MC patients.

Methods

Individuals with the Thomsen (dominant) and Becker (recessive) variants of MC were studied. Isometric muscle strength, whole‐body MRI, and clinical data were collected. The degree of muscle fat replacement of thigh, calf, and forearm muscles was quantitively calculated on Dixon MRI as fat fractions (FFs). Contractility was evaluated as the muscle strength per contractile muscle cross‐sectional area (PT/CCSA). Muscle contractility was compared with clinical data.

Results

Intramuscular FF was increased and contractility reduced in calf and in forearm muscles compared with controls (FF = 7.0–14.3% vs. 5.3–9.6%, PT/CCSA = 1.1–4.9 Nm/cm vs. 1.9–5.8 Nm/cm [ < 0.05]). Becker individuals also showed increased intramuscular FF and reduced contractility of thigh muscles (FF = 11.9% vs. 9.2%, PT/CCSA = 1.9 Nm/cm vs. 3.2 Nm/cm [ < 0.05]). Individual muscle analysis showed that increased FF was limited to seven of 18 examined muscles ( < 0.05). There was a weak correlation between reduced contractility and severity of symptoms.

Conclusions

Individuals with MC have increased fat replacement and reduced contractile properties of muscles. Nonetheless, changes were small and likely did not impact clinically on their myotonic symptoms.

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Authors:
Laura Nørager Jacobsen, Mads Godtfeldt Stemmerik, Sofie Vinther Skriver, Jonas Jalili Pedersen, Nicoline Løkken and John Vissing
Article first published online:
25 Jan 2024 | DOI: 10.1111/ene.16207

ORIGINAL ARTICLE

Background and purpose

Depth electroencephalography (dEEG) is an emerging neuromonitoring technology in acute brain injury (ABI). We aimed to explore the concordances between electrophysiological activities on dEEG and on scalp EEG (scEEG) in ABI patients.

Methods

Consecutive ABI patients who received dEEG monitoring between 2018 and 2022 were included. Background, sporadic epileptiform discharges, rhythmic and periodic patterns (RPPs), electrographic seizures, brief potentially ictal rhythmic discharges, ictal–interictal continuum (IIC) patterns, and hourly RPP burden on dEEG and scEEG were compared.

Results

Sixty‐one ABI patients with a median dEEG monitoring duration of 114 h were included. dEEG significantly showed less continuous background (75% vs. 90%,  = 0.03), higher background amplitude ( < 0.001), more frequent rhythmic spike‐and‐waves (16% vs. 3%,  = 0.03), more IIC patterns (39% vs. 21%,  = 0.03), and greater hourly RPP burden (2430 vs. 1090 s/h,  = 0.01), when compared to scEEG. Among five patients with seizures on scEEG, one patient had concomitant seizures on dEEG, one had periodic discharges (not concomitant) on dEEG, and three had no RPPs on dEEG. Features and temporal occurrence of electrophysiological activities observed on dEEG and scEEG are not strongly associated. Patients with seizures and IIC patterns on dEEG seemed to have a higher rate of poor outcomes at discharge than patients without these patterns on dEEG (42% vs. 25%,  = 0.37).

Conclusions

dEEG can detect abnormal electrophysiological activities that may not be seen on scEEG and can be used as a complement in the neuromonitoring of ABI patients.

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Authors:
Fang Yuan, Charlotte Damien, Sophie Schuind, Michele Salvagno, Fabio Silvio Taccone, Benjamin Legros and Nicolas Gaspard
Article first published online:
25 Jan 2024 | DOI: 10.1111/ene.16208

ORIGINAL ARTICLE

Background

Computed tomography angiography (CTA) has been investigated as a confirmatory study (CS) for the diagnosis of brain death (BD). International consensus regarding its use, study parameters, and evaluation criteria is lacking. In the German BD guideline, a CTA protocol was first introduced in 2015.

Methods

The authors obtained a comprehensive dataset of all BD examinations in adults from the German organ procurement organization to investigate implementation, results, and impact of CTA on BD determination during the first 4 years.

Results

In 5152 patients with clinically absent brain function, 1272 CTA were reported by 676 hospitals. Use of CTA increased from 17.2% of patients in the first year to 29.7% in the final year. CTA replaced other CS such as electroencephalography without increasing overall CS frequency. Technical failure was rare (0.9%); 89.3% of studies were positive. Negative results (9.8%) were more frequent with secondary brain injury, longer duration of the clinical BD syndrome, or unreliable clinical assessment. Median time to diagnosis was longer with CTA (2.6 h) versus other CS (1.6 h). CTA had no differential impact on the rate of confirmed BD and did not improve access of small hospitals to CS for BD determination.

Conclusions

CTA expands the range of available CS for the diagnosis of BD in adults. Real‐world evidence from a large cohort confirms usability of the German CTA protocol within the guideline‐specified context.

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Authors:
Olaf Hoffmann, Farid Salih and Florian Masuhr
Article first published online:
12 Jan 2024 | DOI: 10.1111/ene.16209

LETTER TO THE EDITOR

Response to letter on European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of Guillain–Barré syndrome

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Authors:
Pieter A. van Doorn, Peter Y. K. Van den Bergh and Robert D. M. Hadden
Article first published online:
07 Feb 2024 | DOI: 10.1111/ene.16230

LETTER TO THE EDITOR

Possible benefit of second intravenous immunoglobulin course in Guillain−Barré syndrome patients with potentially poor prognosis

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Authors:
Hai‐Feng Li and Zuneng Lu
Article first published online:
05 Feb 2024 | DOI: 10.1111/ene.16231

EDITORIAL

Generative artificial intelligence in neurology: Opportunities and risks

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Authors:
Antonio Cerasa and Byron Crowe
Article first published online:
08 Feb 2024 | DOI: 10.1111/ene.16232

EDITORIAL

Benefits of influenza vaccine on stroke beyond preventing infection: Paradigm change or sheer bias?

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Authors:
Mitchell S. V. Elkind and Francisco J. de Abajo
Article first published online:
08 Feb 2024 | DOI: 10.1111/ene.16239