Acta Neurologica Scandinavica[JOURNAL]
Sun
200 papers
Comorbidities in patients with Unverricht-Lundborg disease (EPM1).
Sipilä JOT, Kälviäinen R
Acta Neurol Scand · 2022 Nov · PMID 36097839 · Full text
BACKGROUND: Unverricht-Lundborg disease (EPM1) typically leads to accumulating disability. Disability may also be caused by comorbidities but there are no data available on these. AIMS OF THE STUDY: To investigate the fr...
BACKGROUND: Unverricht-Lundborg disease (EPM1) typically leads to accumulating disability. Disability may also be caused by comorbidities but there are no data available on these. AIMS OF THE STUDY: To investigate the frequency of comorbidities in EPM1. METHODS: Comorbidity data of a previously described cohort of 135 Finnish patients with EPM1 were retrieved from neurological, surgical (including subspecialities), internal medicine (including subspecialities) and intensive care patient charts of the treating hospitals. RESULTS: Mean follow-up time was 31.4 years (SD 12.4 years, range 6.8-57.8 years), during which at least one comorbidity was observed in 107 patients (79%) and three or more in 53 (39%). The most common diagnostic categories were external injuries, mental and behavioural disorders and endocrine, nutritional and metabolic diseases. The most common single comorbid diagnosis was a fracture of the ankle (in 19% of all patients). The second most common single comorbid diagnosis in the cohort was diabetes (in 13% of all patients), and the third was depression, recorded for 13% of the cohort. Malignancies and cardiovascular end-organ damage were rare, whereas phimosis/paraphimosis appeared more common than in general population. CONCLUSIONS: Patients with EPM1 often have comorbidities. Trauma and mental health risks should be especially followed and acted upon. Further studies are needed to more accurately comorbidity risks, characteristics and patient needs.
Neuroleptic intolerance in the context of anti-N-methyl-D-aspartate receptor encephalitis: A systematic review and synthesis of global case reports.
Pacheco-Barrios K, Navarro-Flores A, de Melo PS … +6 more , Rebello-Sanchez I, Parente J, Asenjo ED, Gordillo I, Zeña-Ñañez S, Failoc-Rojas VE
Acta Neurol Scand · 2022 Nov · PMID 36086808 · Publisher ↗
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis presents commonly with psychiatric symptoms. One cohort of these patients reported that antipsychotic administration led to neuroleptic intolerance (NI) in 19%...
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis presents commonly with psychiatric symptoms. One cohort of these patients reported that antipsychotic administration led to neuroleptic intolerance (NI) in 19% of them, which was preventable by a prompt encephalitis diagnosis. To date, there is no clear description of the "neuroleptic intolerance" spectrum in general or during anti-NMDAR encephalitis. We aimed to synthesize epidemiological and clinical characteristics of patients with NI and confirmed anti-NMDAR encephalitis, the time to the encephalitis diagnosis, the disease course, outcomes at discharge, and associated factors. We systematically searched three databases, to include clinical cases, case series, and observational studies. Additionally, we reported one clinical case. Results were summarized using narrative synthesis and the quality of the included studies was assessed. We included 22 records representing 40 patients (28 females; mean age, 24.6). Overall, the evidence quality was low. Initially, most cases were admitted in psychiatric wards (70%) with purely psychiatric symptoms (37.5%). However, most of them developed subtle concomitant neurological symptoms. The mean time to anti-NMDAR encephalitis diagnosis was 26.7 days, which was triggered by the NI in six patients. We found no association between clinical variables as delayed diagnosis, admission to psychiatric wards or the presence of malignancy with outcome variables as unfavorable outcomes at discharge, ICU, or mechanical ventilation requirement. A thorough neurological examination in young patients with new-onset psychiatric symptoms could help emergency physicians, neurologists, and psychiatrists suspect anti-NMDAR encephalitis earlier. Awareness of NI as a potential side effect during suspected or confirmed anti-NMDAR encephalitis is encouraged.
Clinical features and spectrum of NOTCH3 variants in Finnish patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
Mönkäre S, Kuuluvainen L, Schleutker J … +2 more , Myllykangas L, Pöyhönen M
Acta Neurol Scand · 2022 Nov · PMID 36086804 · Full text
OBJECTIVES: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a cerebral small vessel disease caused by pathogenic variants in the NOTCH3 gene. In Finland, the majori...
OBJECTIVES: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a cerebral small vessel disease caused by pathogenic variants in the NOTCH3 gene. In Finland, the majority of CADASIL patients carry the pathogenic founder variant c.397C>T, (p.Arg133Cys), but the spectrum of other NOTCH3 variants has not been investigated previously. The aim of the study was to investigate the spectrum and prevalence of NOTCH3 variants Finnish CADASIL patients and to examine the clinical features associated with them. MATERIALS AND METHODS: The spectrum of NOTCH3 variants and the clinical features associated with them were retrospectively examined in 294 Finnish CADASIL patients tested during January 1996 to October 2021 in the Medical Genetics laboratory of Department of Genomics of Turku University Hospital, where practically all samples of patients with suspected CADASIL in Finland are investigated. RESULTS: The most common NOTCH3 variants in the study cohort were c.397C>T, (p.Arg133Cys) (68%) and c.3206A>G p.(Tyr1069Cys) (18%), but other less common NOTCH3 variants were detected in as many as 14% of the patients. Eight of the detected NOTCH3 variants were novel: c.520T>A,p.(Cys174Ser), c.836A>G,p.(Gln279Arg), c.1369T>G,p.(Cys457Gly), c.1338C>G,p.(Cys446Trp), c.1564T>G,p.(Cys522Gly), c.2848T>G,p.(Cys950Gly), c.6102dup,p.(Gly2035Argfs*60), and c.2410+6C>G. Other NOTCH3 variants than p.Arg133Cys and p.Tyr1069Cys were more often associated with more severe clinical features. CONCLUSION: This study revealed the genetic and clinical spectrum of CADASIL in the Finnish population. Sequencing of the whole NOTCH3 gene performing a gene-panel or exome sequencing is recommended when suspecting CADASIL.
Effects of fast-velocity concentric resistance training in people with multiple sclerosis: A randomized controlled trial.
Andreu-Caravaca L, Ramos-Campo DJ, Chung LH … +3 more , Manonelles P, Abellán-Aynés O, Rubio-Arias JÁ
Acta Neurol Scand · 2022 Nov · PMID 36082806 · Full text
OBJECTIVES: To analyze the effects of a fast-velocity concentric resistance training (FVCRT) program on maximum strength of upper and lower limb, gait speed, walking endurance, fatigue, physical self-perception, and cata...
OBJECTIVES: To analyze the effects of a fast-velocity concentric resistance training (FVCRT) program on maximum strength of upper and lower limb, gait speed, walking endurance, fatigue, physical self-perception, and catastrophizing pain in people with multiple sclerosis (MS). MATERIALS AND METHODS: Participants were randomized to either an experimental [EG] (n = 18) or a control [CG] (n = 12) group. The EG carried out 10-weeks of lower limb FVCRT. The CG did not perform any intervention. The maximum isometric voluntary contraction (MVIC) during knee extension, hand-grip strength, gait speed, walking endurance, fatigue, physical self-perception, and catastrophizing pain were measured. RESULTS: Inter-group differences after intervention were found on the right and left sides in MVIC (p = .032; ES = -0.7 and p = .009; ES = -0.9), and hand grip strength (p = .003; ES = -1.0 and p = .029; ES = -0.7). After FVCRT, there was in increase in MVIC (p < .001; ES = -1.7 and p < .001; ES = -1.3) and hand grip strength (p < .001; ES = -1.3 and p < .001; ES = -1.3) on both right and left sides, respectively. In addition, gait speed (p = .023; ES = 1.3), walking endurance (p < .001; ES = -1.0), symptomatic fatigue (p = .004; ES = 0.6), and catastrophizing pain (p < .001; ES = 1.0) improved in EG. CONCLUSION: Lower limb FVCRT improved the upper and lower limb strength, walking, symptomatic fatigue, and catastrophizing pain in MS participants.
Complementary and alternative medicine for treating epilepsy in China: A systematic review.
Zhao C, Lu L, Liu W … +2 more , Zhou D, Wu X
Acta Neurol Scand · 2022 Dec · PMID 36082744 · Publisher ↗
There are about 10 million people with epilepsy (PWE) in China. In addition to therapies approved by the National Medical Products Administration, the use of complementary and alternative medicine (CAM) is prevalent in C...
There are about 10 million people with epilepsy (PWE) in China. In addition to therapies approved by the National Medical Products Administration, the use of complementary and alternative medicine (CAM) is prevalent in Chines PWE. These CAM therapies mainly comprise traditional Chinese medicine herbs (TCMHs), acupuncture, and music. A retrospective literature search was performed to summarize the updates of CAM in China in the past ten years, and sixty-two papers were finally included. In this following review, we listed the animal and clinical studies to summarize the antiepileptic mechanisms and clinical efficacy of CAM in Chines PWE. The main mechanisms of TCMHs and acupuncture included regulation of neurotransmitters and receptors, voltage-gated ion channels modulation, expression of apoptosis-related genes, antioxidant response, and anti-inflammation. Although there were enormous clinical studies on them, the current available clinical trials were small, short-term, heterogeneous, and had a high risk of bias. With regard to music, a few studies conducted by Chinese scholars suggested that it was beneficial for PWE as an add-on therapy, which was consistent with the results of foreign studies. Further randomized clinical trials in large populations are required to prove the effectiveness and safety of CAM.
Effects of anti-seizure therapies on sleep in patients with epilepsy: A literature review.
Ye L, Xu J, Chen C … +2 more , Zhang L, Wang S
Acta Neurol Scand · 2022 Dec · PMID 36071677 · Publisher ↗
Sleep disorder is common in epilepsy. With a recent rapid development in sleep medicine, it has been increasingly recognized that anti-seizure therapies, either anti-seizure medications (ASMs) or non-pharmaceutical appro...
Sleep disorder is common in epilepsy. With a recent rapid development in sleep medicine, it has been increasingly recognized that anti-seizure therapies, either anti-seizure medications (ASMs) or non-pharmaceutical approaches, can take direct or indirect influence on sleep in patients with epilepsy. Here, we systematically review the effect of anti-seizure treatments on sleep. ASMs targeting at different sites exerted various effects on both sleep structure and sleep quality. Non-pharmaceutical treatments including resective surgery, ketogenic diet, and transcranial magnetic stimulation appear to have a positive effect on sleep, while vagus nerve stimulation, deep brain stimulation, and brain-responsive neurostimulation are likely to interrupt sleep and exacerbate sleep-disordered breathing. The potential mechanisms underlying how non-pharmacological approaches affect sleep are also discussed. The limitation of most studies is that they were largely based on small cohorts by short-term observations. Further well-designed and large-scale investigations in this field are warranted. Understanding the effect of anti-seizure therapies on sleep can guide clinicians to optimize epilepsy treatment in the future.
Neuromodulation in drug-resistant epilepsy: A review of current knowledge.
Xue T, Chen S, Bai Y … +3 more , Han C, Yang A, Zhang J
Acta Neurol Scand · 2022 Dec · PMID 36063433 · Publisher ↗
Nearly 1% of the global population suffers from epilepsy. Drug-resistant epilepsy (DRE) affects one-third of epileptic patients who are unable to treat their condition with existing drugs. For the treatment of DRE, neuro...
Nearly 1% of the global population suffers from epilepsy. Drug-resistant epilepsy (DRE) affects one-third of epileptic patients who are unable to treat their condition with existing drugs. For the treatment of DRE, neuromodulation offers a lot of potential. The background, mechanism, indication, application, efficacy, and safety of each technique are briefly described in this narrative review, with an emphasis on three approved neuromodulation therapies: vagus nerve stimulation (VNS), deep brain stimulation of the anterior nucleus of the thalamus (ANT-DBS), and closed-loop responsive neurostimulation (RNS). Neuromodulatory approaches involving direct or induced electrical currents have been developed to lessen seizure frequency and duration in patients with DRE since the notion of electrical stimulation as a therapy for neurologic diseases originated in the early nineteenth century. Although few people have attained total seizure independence for more than 12 months using these treatments, more than half have benefitted from a 50% drop in seizure frequency over time. Although promising outcomes in adults and children with DRE have been achieved, challenges such as heterogeneity among epilepsy types and etiologies, optimization of stimulation parameters, a lack of biomarkers to predict response to neuromodulation therapies, high-level evidence to aid decision-making, and direct comparisons between neuromodulatory approaches remain. To solve these existing gaps, authorize new kinds of neuromodulation, and develop personalized closed-loop treatments, further research is needed. Finally, both invasive and non-invasive neuromodulation seems to be safe. Implantation-related adverse events for invasive stimulation primarily include infection and pain at the implant site. Intracranial hemorrhage is a frequent adverse event for DBS and RNS. Other stimulation-specific side-effects are mild with non-invasive stimulation.
Restless legs syndrome due to brainstem stroke: A systematic review.
Kalampokini S, Poyiadjis S, Vavougios GD … +4 more , Artemiadis A, Zis P, Hadjigeorgiou GM, Bargiotas P
Acta Neurol Scand · 2022 Nov · PMID 36063288 · Publisher ↗
Restless Legs Syndrome (RLS) is a sleep-related movement disorder, which can also result from brainstem pathology. A systematic review of articles published in the electronic databases PubMed and Web of Science was condu...
Restless Legs Syndrome (RLS) is a sleep-related movement disorder, which can also result from brainstem pathology. A systematic review of articles published in the electronic databases PubMed and Web of Science was conducted to summarize the existent literature on RLS associated with a brainstem stroke. We identified eight articles including 19 subjects with RLS due to brainstem ischemic lesion. The symptoms occurred simultaneously with the infarction (66.7%) or few days after (33.3%). The most common location of infarction was pons and less commonly medulla. In most cases (68.4%), symptoms were unilateral. In the majority of those cases (92.3%), the contralateral limb was affected due to a lateral pons infarction. RLS symptoms after infarction improved or resolved in almost 90% of cases within a few days up to 3 months. In almost all patients who received dopaminergic treatment (11 out of 13, 91.7%), the symptoms improved significantly or resolved completely. Screening for RLS has to be considered in patients suffering a brainstem stroke, particularly anteromedial pontine infarction. The appearance of acute unilateral RLS symptoms, usually in association with other sensorimotor deficits, should prompt the clinician to consider a vascular event in the brainstem. RLS in these cases seem to have a favorable outcome and respond well to dopaminergic treatment.
Collateral circulation detected by arterial spin labeling predicts outcome in acute ischemic stroke.
Liu S, Fan D, Zang F … +7 more , Gu N, Yin Y, Ge X, Zhang L, Chen X, Zhang Z, Xie C
Acta Neurol Scand · 2022 Nov · PMID 36062837 · Publisher ↗
BACKGROUND: Robust collateral circulation is strongly associated with good outcomes in acute ischemic stroke (AIS). AIMS: To determine whether collateral circulation detected by arterial spin labeling (ASL) magnetic reso...
BACKGROUND: Robust collateral circulation is strongly associated with good outcomes in acute ischemic stroke (AIS). AIMS: To determine whether collateral circulation detected by arterial spin labeling (ASL) magnetic resonance imaging could predict good clinical outcome in AIS patients with 90 days follow-up. MATERIALS AND METHODS: Total 58 AIS patients with anterior circulation stroke were recruited. Collateral circulation was defined as arterial transit artifact in ASL images. Modified Rankin Scale (mRS), the Barthel Index, and National Institutes of Health Stroke Scale (NIHSS) were employed to evaluate neurological function for the baseline and 90 days follow-up. The percent changes of these scores were also calculated, respectively. Finally, a support vector classifier model of machine learning and receiver operating characteristic curve were employed to estimate the power of ASL collaterals (ASLcs) predicting the clinical outcome. RESULTS: Patients with ASLcs represented higher rate of good outcome (83.30% vs. 31.25%, p < .001) and lower follow-up mRS scores (p < .001), when compared to patients without ASLcs. There were significant differences for percent changes of mRS scores and NIHSS scores between these two groups. Further, the presence of ASLcs could predict good clinical outcome (OR, 1.54; 95% CI, 1.10-2.16), even after controlling for baseline NIHSS scores. The SVC model incorporating baseline NIHSS scores and ASLcs had significant predictive effect (accuracy, 79.3%; AUC, 0.806) on clinical prognosis for AIS patients. DISCUSSION: We targeted on the non-invasive assessment of collateral circulation using ASL technique and found that patients with ASLcs were more likely to have a good clinical outcome after AIS. This finding is of guiding significance for treatment selection and prognostic prediction. CONCLUSIONS: Early ASLcs assessment provides a good powerful tool to predict clinical outcome for AIS patients with 90 days follow-up.
Is there a role for off-label high-efficacy disease-modifying drugs in progressive multiple sclerosis? A network meta-analysis.
Silva GD, Castrillo BB, Apóstolos-Pereira SL … +1 more , Callegaro D
Acta Neurol Scand · 2022 Nov · PMID 36052576 · Publisher ↗
Ocrelizumab and siponimod are the two on-label drugs used for progressive forms of multiple sclerosis (PMS). However, many patients with PMS do not have access to these high-efficacy disease-modifying drugs (DMDs). Off-l...
Ocrelizumab and siponimod are the two on-label drugs used for progressive forms of multiple sclerosis (PMS). However, many patients with PMS do not have access to these high-efficacy disease-modifying drugs (DMDs). Off-label prescription of other high-efficacy DMDs (fingolimod, rituximab and natalizumab) may be a strategy to improve access to immunotherapy for these patients. We aim to compare on-label and off-label high-efficacy drugs for their effect on disability progression in PMS. In December 2021, we searched MEDLINE (PubMed), Embase, Cochrane Central and Scopus databases for randomized clinical trials involving patients with PMS. High-efficacy drugs were considered as intervention and placebos as comparison. The outcome contemplated was risk of Expanded Disability Severity Scale (EDSS) progression at 2 years. A network meta-analysis was performed to compare the relative risk of EDSS progression at 2 years compared with placebo in on-label and off-label drugs. We included five studies with 4526 patients. The median EDSS progression at 2 years in patients that received any immunotherapy was 30%, compared with 35% in placebo groups. Overall, the risk of bias of individual studies was low. Network analysis revealed overlapping confidence intervals in off-label drugs (CI95% 0.51-2.16) versus ocrelizumab (reference) and off-label drugs (CI 95% 0.53-1.96) versus siponimod (reference), suggesting similar efficacy. The same result was found even after excluding studies with the risk of publication bias. Off-label high efficacy immunotherapy in PMS has biological plausibility and presented similar effectiveness to on-label DMDs in this network meta-analysis. The use of fingolimod, rituximab or natalizumab may be a strategy that reduces costs and improves access to immunotherapy for patients with PMS.
Identification of novel signal of clobazam-associated drug reaction with eosinophilia and systemic symptoms syndrome: A disproportionality analysis.
Sharma A, Kumar A
Acta Neurol Scand · 2022 Nov · PMID 36029138 · Publisher ↗
OBJECTIVES: Clobazam is a well-known benzodiazepine used as an anti-anxiety drug as well as an anti-epileptic, particularly for patients who are not responding to first-line treatments. Recent case reports have indicated...
OBJECTIVES: Clobazam is a well-known benzodiazepine used as an anti-anxiety drug as well as an anti-epileptic, particularly for patients who are not responding to first-line treatments. Recent case reports have indicated the association of clobazam with drug reaction with eosinophilia systemic symptoms syndrome (DRESS Syndrome). However, DRESS syndrome is not known to be associated with clobazam. Thus, the main objective of the current study was to identify the potential signal of clobazam-associated DRESS Syndrome. MATERIALS & METHODS: US FDA Adverse event reporting system (US FAERS), pharmacovigilance data 2004Q1-2021Q3 was extracted using OpenVigil 2.1-MedDRA-v24. The Proportional Reporting Ratio (PRR), Reporting Odds Ratio (ROR) with a Chi-Square value (95% confidence interval), and number of cases (≥3) were used as disproportionality analysis parameters. RESULTS: A total of 141 drug-event combinations were reported and results of disproportionality analysis indicate the positive signal of DRESS syndrome with clobazam. The signal strength was decreased after removing the cases of concomitantly administered drugs (phenytoin, levetiracetam, and valproic acid); however, the association of clobazam with DRESS syndrome remains statistically significant. The subgroup analysis results have shown a greater number of cases in the age group (18-64 years) as compared to other age groups whereas the number of cases in the male and female groups is almost similar. CONCLUSION: The DRESS syndrome is identified as a novel signal with clobazam. However, further causality assessment is required.
Incidence of first-ever transient ischemic attack in Eastern Finland.
Komulainen T, Koivisto A, Jäkälä P
Acta Neurol Scand · 2022 Nov · PMID 36029100 · Full text
OBJECTIVES: The incidence of stroke has been declining in Finland, as well as in Europe. However, it is unclear whether the incidence of transient ischemic attack (TIA) is also decreasing. In fact, the TIA incidence in t...
OBJECTIVES: The incidence of stroke has been declining in Finland, as well as in Europe. However, it is unclear whether the incidence of transient ischemic attack (TIA) is also decreasing. In fact, the TIA incidence in the Finnish population has never been reported. Therefore, here we investigated the incidence of TIA in the Eastern Finnish population in 2017. MATERIALS AND METHODS: All patients with suspected TIA, from a defined catchment area, were referred to a neurological emergency unit at Kuopio University Hospital (KUH) in the Northern Savonia region of Eastern Finland, which had a population of 246,653 in 2017. The original study population comprised TIA patients diagnosed based on the WHO TIA criteria in 2017. Incidence rates were calculated by dividing the number of TIA cases by the number of people in different age groups. RESULTS: Among 432 patients with a suspected TIA referred to the neurological emergency unit at Kuopio University Hospital in 2017, 293 were living in Northern Savonia and were ultimately diagnosed with TIA after neurological examinations. The number of first-ever TIAs was 211. The crude incidence of all TIA was 122/100,000 inhabitants, and of first-ever TIA was 86/100,000. The age-standardized incidence (European population 2010) of the first-ever TIA was calculated to be 64/100,000. The mean age of first-ever TIA patients was 70 years: 72 years for women versus 68 years for men. CONCLUSIONS: We found a high incidence of TIA in Eastern Finland.
Corticosteroids in patients with vestibular neuritis: An updated meta-analysis.
Bogdanova A, Dlugaiczyk J, Heckmann JG … +1 more , Schwab S
Acta Neurol Scand · 2022 Nov · PMID 36029039 · Publisher ↗
Vestibular neuritis is a common neuro-otological entity. Therapeutically, corticosteroids are advised, although the evidence is limited. The objective of this review is to update meta-analyses of clinical trials that add...
Vestibular neuritis is a common neuro-otological entity. Therapeutically, corticosteroids are advised, although the evidence is limited. The objective of this review is to update meta-analyses of clinical trials that address the question of whether patients with vestibular neuritis treated with corticosteroids show better recovery than control patients. The electronic databases Medline, Scopus and Cochrane were searched for clinical trials for the years 1970-2020 without language restriction. Data were extracted, and outcome parameters were subjected to conventional and cumulative meta-analysis using a commercially available software program (www.meta-analysis.com). Finally, 15 trials with 363 participants in the treatment and 489 in the control groups were identified and could be included. Eight studies were judged to be at high risk of bias. The odds ratio (OR) for good outcome in the acute phase was 3.1 (95% CI 1.2-7.8; p = .015) in favour of steroid treatment leading to the number needed to treat (NNT) = 6 (95% CI 4-23). The odds ratio (OR) for restoration of vestibular function in the follow-up was 2.4 (95% CI 1.3-4.4; p = .004) for the benefit of steroid treatment resulting in a NNT = 7 (95% CI 5-18). The results of the cumulative statistics did not differ. The risk of adverse effects was higher in patients treated with steroids with an OR of 10.9 (95% CI 1.3-93.8; p = .015) and an estimated number needed to harm (NNH) = 4 (95% CI 3-19). The advantage for corticosteroids remained when differentiating between patients who participated in randomized or non-randomized clinical trials. Steroid treatment in vestibular neuritis resulted in a statistically significant benefit compared to control therapies. However, broad heterogeneity of the studies, mostly low-grade quality of studies, high risk of bias and broad confidence intervals put the findings into perspective allowing only a careful judgement of some benefit of corticosteroids. The findings, however, support the call for an adequately powered and well-designed randomized controlled trial to re-evaluate the effectiveness of corticosteroids.
Thrombectomy in large vessel occlusion stroke-Does age matter?
Rezai MK, Dalen I, Advani R … +4 more , Fjetland L, Kurz KD, Sandve KO, Kurz MW
Acta Neurol Scand · 2022 Nov · PMID 36029034 · Full text
OBJECTIVES: Endovascular treatment (EVT) is the gold standard treatment for emergent large vessel occlusion (LVO). The benefit of EVT for emergent LVO in elderly patients (>80 years old) is still debated as they have bee...
OBJECTIVES: Endovascular treatment (EVT) is the gold standard treatment for emergent large vessel occlusion (LVO). The benefit of EVT for emergent LVO in elderly patients (>80 years old) is still debated as they have been under-represented in randomized controlled trials. Elderly patients with an emergent LVO are a growing population warranting further study. MATERIALS & METHODS: We included 225 consecutive patients treated with EVT for LVO either in the anterior or posterior circulation. The clinical outcome was assessed using the National Institute of Health Stroke Scale (NIHSS). Long-term functional outcome was assessed using 90-day modified ranking scale (mRS). RESULTS: Neurological improvement: A five-year higher age predicted a 0.43 higher mean NIHSS score after EVT (p = .027). After adjusting for confounders (influencing variables), the association between age and post-interventional NIHSS was reduced and non-significant (p = .17). At discharge, a five-year higher age predicted a 0.74 higher mean NIHSS (p = .003). After adjusting for confounders this association was reduced and non-significant (p = .06). Long-term functional outcome: A five-year higher age predicted a 0.20 higher mRS at three months (p < .001). When adjusting for confounders this number was reduced to 0.16, yet still highly significant (p < .001). CONCLUSIONS: Age seems to have a minor role in predicting neurological improvement after EVT but has an impact on long-term functional outcome. The decision to perform or withhold EVT should therefore not solely be based on age.
Interest in and exposure to headache disorders among neurology residents in Denmark: A nationwide cross-sectional survey.
Kristensen MGH, Do TP, Pozo-Rosich P … +1 more , Amin FM
Acta Neurol Scand · 2022 Nov · PMID 36004408 · Full text
OBJECTIVE: Headache disorders constitute a leading cause of disability worldwide, but there is a consistent absence of awareness and educational activities for healthcare providers across regions. Thus, we found it timel...
OBJECTIVE: Headache disorders constitute a leading cause of disability worldwide, but there is a consistent absence of awareness and educational activities for healthcare providers across regions. Thus, we found it timely to identify potential structural challenges and factors that may affect acquisition of knowledge of headache disorders and their management during their 4-year residency. MATERIALS & METHODS: We conducted a nationwide cross-sectional survey of residents in neurology in Denmark including, but not limited to, questions on interest in neurological subspecialties and disorders, adequacy of training in headache disorders, exposure to headache disorders during training including time spent on headache disorders, exposure to specialist outpatient clinics, whether their hospital have a tertiary headache clinic, training in specific procedures (anesthetic blockade, e.g., greater occipital nerve blockade, and onabotulinumtoxinA for headache), and an estimate of proportion of cases with headache among patients managed in the last week. RESULTS: The survey was distributed to 127 residents in Denmark between March 2022 and April 2022. Of these, 59 (47%) completed all questions of the survey. Headache disorders were the fourth most popular subspecialties among respondents (n = 15 [25%]) following movement disorders (n = 27 [46%]), vascular neurology (n = 26 [44%]), and neuromuscular disorders (n = 25 [42%]). The mean number of hours spent in a course or a structured educational activity in headache disorders during residency was 12.1 h. Half of respondents (n = 27 [46%]) reported that they perceived their training in headache disorders to be inadequate. CONCLUSIONS: Even in Denmark, a country with excellent headache services, half of residents in neurology report an inadequate training despite a higher-than-average number of hours of structured educational activities. These findings should incentivize stakeholders to make structural changes to improve education in headache disorders during the most fundamental years of training.
Progress of clinical research studies on tuberous sclerosis complex-related epilepsy in China.
Liu T, Chen F, Zhai F … +1 more , Liang S
Acta Neurol Scand · 2022 Dec · PMID 36000491 · Publisher ↗
Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome, with 75.6%-83.5% and 54.1% patients presenting with epilepsy and drug-resistant epilepsy (DRE), respectively. Clinical studies on TSC, pa...
Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome, with 75.6%-83.5% and 54.1% patients presenting with epilepsy and drug-resistant epilepsy (DRE), respectively. Clinical studies on TSC, particularly surgical interventions, have achieved rapid and substantial progress. The TSC-Task Force Committee of the China Association Against Epilepsy (CAAE-TFTSC) was founded in 2012, and annual academic conferences on the surgical treatment of TSC-related epilepsy have been held since 2013. 'China experts' consensus on surgical treatment of TSC-related epilepsy' was published in 2019. This review focuses on surgical treatment, including resective surgery, neuromodulations, corpus callosotomy and mini-invasive ablations, as well as studies on phenotype, genotype and anti-seizure therapies of mammalian target of rapamycin inhibitor, vigabatrin and ketogenic diet in patients with TSC-related DRE in China.
Natural history of circulating miRNAs in Duchenne disease: Association with muscle injury and metabolic parameters.
Almeida-Becerril T, Rodríguez-Cruz M, Hernández-Cruz SY … +6 more , Ruiz-Cruz ED, Mendoza CRS, Cárdenas-Conejo A, Escobar-Cedillo RE, Ávila-Moreno F, Aquino-Jarquin G
Acta Neurol Scand · 2022 Nov · PMID 36000352 · Publisher ↗
OBJECTIVES: This study aimed to evaluate whether the expression of circulating dystromiRs and a group of oxidative stress-related (OS-R) miRNAs is associated with muscle injury and circulating metabolic parameters in Duc...
OBJECTIVES: This study aimed to evaluate whether the expression of circulating dystromiRs and a group of oxidative stress-related (OS-R) miRNAs is associated with muscle injury and circulating metabolic parameters in Duchenne muscular dystrophy (DMD) patients. METHODS: Twenty-four DMD patients were included in this cross-sectional study. Clinical scales to evaluate muscle injury (Vignos, GMFCS, Brooke, and Medical Research Council), enzymatic muscle injury parameters (CPK, ALT, and AST), anthropometry, metabolic indicators, physical activity, serum dystromiRs (miR-1-3p, miR-133a-3p, and miR-206), and OS-R miRNAs (miR-21-5p, miR-31-5p, miR-128-3p, and miR-144-3p) levels were measured in ambulatory and non-ambulatory DMD patients. RESULTS: DystromiRs (except miR-1-3p) and miRNAs OS-R levels were lower (p-value <.05) in the non-ambulatory group than the ambulatory group. The expression of those miRNAs correlated with Vignos scale score (For instance, rho = -0.567, p-value <0.05 for miR-21-5p) and with other scales scores of muscle function and strength. CPK, AST, and ALT concentration correlated with expression of all miRNAs (For instance, rho = 0.741, p-value <.05 between miR-206 level and AST concentration). MiR-21-5p level correlated with glucose concentration (rho = -0.369, p-value = .038), and the miR-1-3p level correlated with insulin concentration (rho = 0.343, p-value = .05). CONCLUSIONS: Non-ambulatory DMD patients have lower circulating dystromiRs and OS-R miRNAs levels than ambulatory DMD patients. The progressive muscle injury is associated with a decrease in the expression of those miRNAs, evidencing DMD progress. These findings add new information about the natural history of DMD.
Seizure control in successive pregnancies in Australian women with epilepsy.
Vajda FJE, O'Brien TJ, Graham J … +4 more , Hitchcock AA, Perucca P, Lander CM, Eadie MJ
Acta Neurol Scand · 2022 Nov · PMID 35986483 · Full text
OBJECTIVES: To investigate control of epileptic seizures during pairs of successive pregnancies in antiseizure medication (ASM)-treated women with epilepsy. MATERIALS AND METHODS: Analysis of seizure freedom rates during...
OBJECTIVES: To investigate control of epileptic seizures during pairs of successive pregnancies in antiseizure medication (ASM)-treated women with epilepsy. MATERIALS AND METHODS: Analysis of seizure freedom rates during 436 pairs of successive pregnancies in Australian women with epilepsy, in nearly all instances long-standing epilepsy. RESULT: There was a higher rate of seizure-free second pregnancies compared with first paired pregnancies (63.1% vs. 51.4%; Relative Risk (R.R.) = 1.2277; 95% CI 1.0930, 1.3789) and of seizure-free pre-pregnancy years before second as compared with first paired pregnancies in the same women (63.6% vs. 52.4%; R.R. = 1.2616; 95% CI 1.1337, 1.4040). In 108 women whose ASM therapy was unaltered throughout both of their pregnancies, the seizure-freedom rate was higher in the second of the paired pregnancies (82.4% vs. 69.4%; R.R. = 1.1867, 95% CI 1.0189, 1.3821). CONCLUSIONS: Altered ASM therapy after the first of a pair of successive pregnancies did not fully account for the better overall seizure control in the corresponding second pregnancies. Some additional factor may have been in operation, possibly a greater preparedness to undertake a further pregnancy if seizures were already fully controlled.
Intra- or peri-procedural rupture in the endovascular treatment of intracranial aneurysms.
Delgado Acosta F, Bravo Rey I, Jiménez Gómez E … +3 more , Saucedo VR, Toledano A, Oteros Fernández R
Acta Neurol Scand · 2022 Nov · PMID 35975464 · Publisher ↗
INTRODUCTION: Intra- or peri-procedural aneurysm rupture is one of the most feared adverse effects associated with embolization. Our aim was to report the characteristics of patients suffering intra- or peri-procedural r...
INTRODUCTION: Intra- or peri-procedural aneurysm rupture is one of the most feared adverse effects associated with embolization. Our aim was to report the characteristics of patients suffering intra- or peri-procedural ruptures during embolization of cerebral aneurysms. PATIENTS AND METHODS: Between March 1994 and October 2021, 648 consecutive cerebral aneurysms were treated by endovascular procedure at our facility. Medical records were reviewed retrospectively with emphasis on procedure description, potential risk factors, and clinical outcomes related to intra- or peri-procedural rupture. RESULTS: Of the 648 patients, 17 (2.6%) suffered an intra- or peri-procedural hemorrhagic event. The most common location was the anterior communicating artery. There was no significant difference between previously ruptured and unruptured aneurysms in the incidence of bleeding. In four patients, bleeding was evident within 24 h after the procedure. The clinical evolution at three months was poor and only four patients presented a positive evolution. There were 11 deaths (64.71%). Balloon remodeling was associated with an increased frequency of ruptures, while stenting was a safer treatment. CONCLUSION: Aneurysmal rupture during endovascular therapy is unpredictable, and its occurrence can be devastating. The incidence is quite low although the outcome is frequently poor. Early detection and proper management, including prompt occlusion of the aneurysm, are important to achieve a positive outcome. Anterior communicating artery aneurysms and those treated with balloon catheters have a higher incidence of rupture. A small number of ruptures of uncertain origin occur that go unnoticed in digital subtraction angiograms.
Slow vital capacity as a useful indicator of the prognosis after percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis.
Ueno T, Haga R, Arai A … +1 more , Tomiyama M
Acta Neurol Scand · 2022 Nov · PMID 35975358 · Publisher ↗
OBJECTIVES: Forced vital capacity (FVC) is recommended as a respiratory function test in patients with amyotrophic lateral sclerosis (ALS). However, in ALS associated with orofacial palsy, FVC may be an unreliable test....
OBJECTIVES: Forced vital capacity (FVC) is recommended as a respiratory function test in patients with amyotrophic lateral sclerosis (ALS). However, in ALS associated with orofacial palsy, FVC may be an unreliable test. Slow vital capacity (SVC) is an easier and more reliable test even in cases with bulbar symptoms. However, it remains unclear whether respiratory function tests using SVC and FVC are associated with prognosis after percutaneous endoscopic gastrostomy (PEG) placement. This study aimed to confirm whether both SVC and FVC are related to prognosis after PEG placement in patients with ALS. MATERIALS AND METHODS: We conducted this retrospective observational cohort study of 69 consecutive patients diagnosed with sporadic ALS who underwent PEG placement between July 2007 and February 2020. We analyzed the association with mortality 6 months after PEG placement and evaluated long-term prognosis. RESULTS: Forty-four patients met the inclusion criteria. In cases with decreased SVC (p < .01) and FVC (p < .01), a significant difference was observed in mortality 6 months after PEG placement, with an optimal cut-off of SVC ≤57.4% (sensitivity, 0.828; specificity, 0.867) and FVC ≤57.3% (sensitivity, 0.828; specificity, 0.867). Multivariate analysis showed that onset age ≥ 65 years (p < .05), SVC ≤57.4% (p < .01), and FVC ≤57.3% (p < .01) were associated with survival after PEG placement. CONCLUSIONS: SVC, like FVC, is an important prognostic factor after PEG placement in patients with ALS, and there is a possibility that evaluation using SVC can complement respiratory function testing even in cases where the evaluation of FVC is limited.
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