Acta Neurologica Scandinavica[JOURNAL]
Sun
200 papers
Changing epidemiology of traumatic brain injury among the working-aged in Finland: Admissions and neurosurgical operations.
Posti JP, Luoto TM, Sipilä JOT … +2 more , Rautava P, Kytö V
Acta Neurol Scand · 2022 Jul · PMID 35257358 · Publisher ↗
BACKGROUND: Recent studies from Finland have highlighted an increase in the incidence of traumatic brain injuries (TBI) in older age groups and high overall mortality. We performed a comprehensive study on the changing e...
BACKGROUND: Recent studies from Finland have highlighted an increase in the incidence of traumatic brain injuries (TBI) in older age groups and high overall mortality. We performed a comprehensive study on the changing epidemiology of TBI focusing on the acute events in the Finnish working-age population. METHODS: Nationwide databases were searched for all emergency ward admissions with a TBI diagnosis for persons of 16-69 years of age during 2004-2018. RESULTS: In the Finnish working-age population, there were 52,487,099 person-years, 38,810 TBI-related hospital admissions, 4664 acute neurosurgical operations (ANO), and 2247 cases of in-hospital mortality (IHM). The TBI-related hospital admission incidence was 94/100,000 person-years in men, 44/100,000 in women, and 69/100,000 overall. The incidence rate of admissions increased in women, while in men and overall, the rate decreased. The incidence rate increased in the group of 60-69 years in both genders. Lowest incidence rates were observed in the age group of 30-39 years. Occurrence risk for TBI admission was higher in men in all age groups. Trends of ANOs decreased overall, while decompressive craniectomy was the only operation type in which a rise in incidence was found. Evacuation of acute subdural hematoma was the most common ANO. Mean length of stay and IHM rate halved during the study years. CONCLUSIONS: In Finland, the epidemiology of acute working-aged TBI has significantly changed. The rates of admission incidences, ANOs, and IHM nowadays represent the lower end of the range of these acute events reported in the western world.
Perinatal adversities and risk of epilepsy after traumatic brain injury: A Danish nationwide cohort study.
Lolk K, Dreier JW, Sun Y … +1 more , Christensen J
Acta Neurol Scand · 2022 Jun · PMID 35243615 · Full text
OBJECTIVES: Traumatic brain injury (TBI) and perinatal adversities such as low gestational age at birth, low birth weight, low Apgar, and being born small for gestational age are well-established risk factors for epileps...
OBJECTIVES: Traumatic brain injury (TBI) and perinatal adversities such as low gestational age at birth, low birth weight, low Apgar, and being born small for gestational age are well-established risk factors for epilepsy. We examined whether perinatal adversities modified the risk of epilepsy after TBI in a nationwide cohort study of Danish singletons born from 1982 to 2011. MATERIALS AND METHODS: We categorized perinatal adversities as a composite measure of preterm delivery, low birth weight, low Apgar score, or being born small for gestational age. Cox regression and competing risk regression were used to estimate the risk of epilepsy after TBI according to such perinatal adversities. The study included 1,715,095 singletons (51.1% males). The mean age at end of follow-up was 19.3 years (Interquartile range [IQR] = 12.1-26.3). During follow-up, 85,636 persons (58.2% males) sustained a TBI and 18,064 developed epilepsy (50.7% males), of whom 1329 persons had a preceding TBI. RESULTS: The hazard ratio (HR) of epilepsy in persons with perinatal adversities was 1.19 (95% confidence interval [CI] 1.15-1.24), compared to persons without. The HR of epilepsy in persons with TBI was 2.31 (95% CI 2.18-2.45) compared to persons without TBI, but this risk was not modified by perinatal adversities (p = 0.2460). CONCLUSIONS: Perinatal adversities and TBI both increased the risk of epilepsy, but the risk of epilepsy after TBI was not modified by these perinatal adversities.
Treatment satisfaction with calcitonin gene-related peptide monoclonal antibodies as a new patient-reported outcome measure: A real-life experience in migraine.
López-Bravo A, Oliveros-Cid A, Sevillano-Orte L
Acta Neurol Scand · 2022 Jun · PMID 35243611 · Publisher ↗
BACKGROUND: To evaluate treatment satisfaction with galcanezumab as a patient-reported outcome measure (PROM) in migraine. METHODS: Patients with ≥8 headache days/month that had failed at ≥3 medications were included. De...
BACKGROUND: To evaluate treatment satisfaction with galcanezumab as a patient-reported outcome measure (PROM) in migraine. METHODS: Patients with ≥8 headache days/month that had failed at ≥3 medications were included. Demographic and medical history were collected. Patient´s satisfaction (effectiveness, safety, convenience, and global satisfaction [GS]) was assessed by the Treatment Satisfaction Questionnaire for Medication version 1.4 (TSQM-1.4©). RESULTS: We included 30 patients with migraine (76.7% chronic migraine). After 12 weeks of galcanezumab treatment, median monthly headache days (MHDs) decreased 11.5 (IQR 14.0) and median monthly migraine days (MMDs) 9.0 (IQR 7.5); at 24 weeks, the change was 15.0 (IQR 12.0) and 8.0 days (IQR 6.0). HIT-6 score decreased from 68.0 (IQR 7.5) to 54.0 (IQR 9.5) at 12 weeks (p < .001) and to 52.0 (IQR12.0) at 24 weeks (p < .001) and MIDAS from 60.0 (IQR 62.7) to 25.5 (IQR 41.2, p = .004) and 7.0 (IQR 18.5, p < .001), respectively. TSQM-1.4© at 12 weeks was higher compared to other preventive therapy in effectiveness (80.6/50.4, p < .001), convenience (83.3/66.7, p = .001), and GS (78.6/50.0, p < .001). These rates of satisfaction were similar at 24 weeks of galcanezumab treatment. Reductions in HIT-6 (r = -.444, p = .014), MIDAS (r = -.423, p = .020), MMDs (r = -.515, p = .004), and MHDs (r = -.477, p = .008) were associated significantly with GS at 12 weeks. This correlation was significantly associated with changes in HIT-6 and MHDs at 24 weeks. CONCLUSIONS: The results of this study suggest that migraine patients receiving galcanezumab are significantly more satisfied compared to other preventive therapies, associating treatment GS with meaningful reductions in frequency, impact, and disability caused by migraine.
Clinimetric study and review of the Reaction Level Scale.
Anestis DM, Monioudis PM, Foroglou NG … +2 more , Tsonidis CA, Tsitsopoulos PP
Acta Neurol Scand · 2022 Jun · PMID 35243607 · Publisher ↗
OBJECTIVES: Although the Reaction Level Scale (RLS) is still used for the assessment of the level of consciousness in distinct centers, its clinical characteristics and significance have been incompletely researched. In...
OBJECTIVES: Although the Reaction Level Scale (RLS) is still used for the assessment of the level of consciousness in distinct centers, its clinical characteristics and significance have been incompletely researched. In the current study, the clinimetric properties, the prognostic value, and the impact of the raters' background on the application of the RLS, in comparison with the Glasgow Coma Scale (GCS), are investigated. MATERIALS AND METHODS: A systematic review on the available clinical evidence for the RLS was first carried out. Next, the RLS was translated into Greek, and patients with neurosurgical pathologies in need of consciousness monitoring were independently assessed with both RLS and GCS, by four raters (two consultants, one resident, and one nurse) within one hour. Interrater reliability, construct validity, and predictive value (mortality and poor outcome, at discharge and at 6 months) were evaluated. RESULTS: Literature review retrieved 9 clinimetric studies related to the RLS, most of low quality, indicating that the scale has not been thoroughly studied. Both versions of the RLS (original and modified) showed high interrater reliability (κ >0.80 for all pairs of raters), construct validity (Spearman's p > .90 for all raters), and prognostic value (areas under the curve >0.85 for all raters and outcomes). However, except for broader patients' coverage, it failed to show any advantage over the GCS. CONCLUSIONS: The RLS has not succeeded in showing any advantage over the GCS in terms of reliability and validity. Available evidence cannot justify its use in clinical practice as a substitute to the widely applied GCS.
Eptinezumab for migraine prevention in patients 50 years or older.
Martin V, Tassorelli C, Ettrup A … +2 more , Hirman J, Cady R
Acta Neurol Scand · 2022 Jun · PMID 35218203 · Full text
OBJECTIVE: To evaluate the efficacy and safety of eptinezumab versus placebo in patients ≥50 years old with episodic (EM) or chronic migraine (CM). MATERIALS AND METHODS: This post hoc analysis included data from two pha...
OBJECTIVE: To evaluate the efficacy and safety of eptinezumab versus placebo in patients ≥50 years old with episodic (EM) or chronic migraine (CM). MATERIALS AND METHODS: This post hoc analysis included data from two phase 3, parallel-group, randomized, double-blind, placebo-controlled studies in adults with EM (PROMISE-1) or CM (PROMISE-2). Patients ≥50 years at baseline treated with eptinezumab 100 mg, 300 mg, or placebo were pooled from both studies to evaluate efficacy and safety. RESULTS: A total of 385/1960 (19.6%) EM and CM patients who were ≥50 years old at baseline (range, 50-71 and 50-65 years, respectively) received eptinezumab 100 mg (n = 132), 300 mg (n = 127), or placebo (n = 126) over Weeks 1-12. Reductions in mean monthly migraine days (MMDs) in ≥50-year-old EM patients were -3.8 (100 mg) and -4.4 (300 mg) with eptinezumab versus -2.6 with placebo. In ≥50-year-old CM patients, mean changes in MMDs were -7.7 (100 mg) and -8.6 (300 mg) with eptinezumab versus -6.0 with placebo. Changes in MMDs were comparable to total study results. A ≥50% MMD reduction over Weeks 1-12 was achieved by 57.9% of eptinezumab-treated versus 35.7% of patients who received placebo, and a ≥75% reduction by 30.5% versus 13.5%, respectively. The incidence of treatment-emergent adverse events (TEAEs) in EM and CM patients ≥50 years old was similar across treatment groups, with ≥96% of TEAEs mild or moderate in severity. CONCLUSIONS: Treatment with eptinezumab was efficacious, tolerable, and safe in patients ≥50 years with EM or CM, congruent with results from the overall study population.
Natural history and remarkable psychiatric state of late-onset amyotrophic lateral sclerosis in China.
Huang S, Zheng M, Lin J … +4 more , Huang P, Chen W, He R, Yao X
Acta Neurol Scand · 2022 Jul · PMID 35187661 · Publisher ↗
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons. The proportion of late-onset ALS in China were low and may have distinct clinical and genetic manifestations. W...
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons. The proportion of late-onset ALS in China were low and may have distinct clinical and genetic manifestations. We aimed to investigate the natural history and remarkable psychiatric state of ALS with age at onset over 60 years in China. MATERIALS AND METHODS: We collected all ALS cases from 2017 to 2020 in our center and focused on late-onset ALS patients particularly, by analyzing the clinical data, including the ALS onset and disease progression. Anxiety, depression, cognitive function, and sleep quality were assessed to reflect the psychiatric state. RESULTS: A total of 193 late-onset ALS patients were included in this study. The median age at onset of late-onset ALS was 65 years with the quartile from 62 to 68 years. When compared with 446 non-late-onset ALS, late-onset ALS showed distinct clinical presentation, with lower ALS Functional Rating Scale-Revised at diagnosis and faster rate of progression. Remarkably, late-onset ALS were suffering from worse psychiatric state, including serious anxiety and depression, as well as worse cognitive function with sleep quality. The abnormal psychiatric state was more pronounced in female patients of late-onset. CONCLUSIONS: In the current study, ALS patients with late-onset showed unique clinical features. Severe psychiatric conditions and faster progression in the early stage of the disease of late-onset ALS indicated the need for more social and psychiatric support in this population.
Is infarct core growth linear? Infarct volume estimation by computed tomography perfusion imaging.
Suomalainen OP, Elseoud Abou A, Martinez-Majander N … +5 more , Tiainen M, Valkonen K, Virtanen P, Forss N, Curtze S
Acta Neurol Scand · 2022 Jun · PMID 35187642 · Publisher ↗
OBJECTIVES: Current guidelines for recanalization treatment are based on the time elapsed between symptom onset and treatment and visualization of existing penumbra in computed tomography perfusion (CTP) imaging. The tim...
OBJECTIVES: Current guidelines for recanalization treatment are based on the time elapsed between symptom onset and treatment and visualization of existing penumbra in computed tomography perfusion (CTP) imaging. The time window for treatment options relies on linear growth of infarction although individual infarct growth rate may vary. We aimed to test how accurately the estimated follow-up infarct volume (eFIV) can be approximated by using a linear growth model based on CTP baseline imaging. If eFIV did not fall within the margins of +/- 19% of the follow-up infarct volume (FIV) measured at 24 h from non-enhanced computed tomography images, the results would imply that the infarct growth is not linear. MATERIALS AND METHODS: All consecutive endovascularly treated (EVT) patients from 11/2015 to 9/2019 at the Helsinki University Hospital with large vessel occlusion (LVO), CTP imaging, and known time of symptom onset were included. Infarct growth rate was assumed to be linear and calculated by dividing the ischemic core volume (CTP ) by the time from symptom onset to baseline imaging. eFIV was calculated by multiplying the infarct growth rate with the time from baseline imaging to recanalization or in case of futile recanalization to follow-up imaging at 24 h, limited to the penumbra. Collateral flow was estimated by calculating hypoperfusion intensity ratio (HIR). RESULTS: Of 5234 patients, 48 had LVO, EVT, CTP imaging, and known time of symptom onset. In 40/48 patients (87%), infarct growth was not linear. HIR did not differ between patients with linear and nonlinear growth (p > .05). As expected, in over half of the patients with successful recanalization eFIV exceeded FIV. CONCLUSIONS: Infarct growth was not linear in most patients and thus time elapsed from symptom onset and CTP appear to be insufficient parameters for clinical decision-making in EVT candidates.
Cannabis use in Parkinson's disease-A nationwide online survey study.
Erga AH, Maple-Grødem J, Alves G
Acta Neurol Scand · 2022 Jun · PMID 35178701 · Publisher ↗
OBJECTIVES: The aim of this study was to investigate the frequency of use, attitudes toward, and experiences with cannabis and cannabis-related products among people with Parkinson's disease (PwP) living in Norway. METHO...
OBJECTIVES: The aim of this study was to investigate the frequency of use, attitudes toward, and experiences with cannabis and cannabis-related products among people with Parkinson's disease (PwP) living in Norway. METHODS: Between February and August 2021, PwP and their caregivers were invited to participate in an anonymous online survey study on cannabis use. N = 530 PwP completed the 24-item survey collecting data on the participants' history of cannabis use, perceived benefits and adverse effects of cannabis use, and expectations toward health care professionals. N = 108 caregivers completed a brief survey detailing their experience with cannabis use. RESULTS: A total of 59 (11.3%) of PwP reported previous or current use of cannabis, compared to 7 (6.6%) of caregivers. Cannabis use was associated with increased disease duration, but not age or gender. Improvement in motor function (69.5%), sleep (52.5%), and pain (37.3%) was the most frequently perceived benefits of cannabis use, with benefits more frequently reported by current than previous users. While half (50.8%) of cannabis users had sought advice from a health care professional regarding cannabis use, only 55 (19.9%) of non-users with an interest in cannabis use had discussed the topic with health care professionals. Principal barriers for discussing cannabis use with health care professionals are discussed. CONCLUSIONS: One in 20 PwP reports cannabis use, and non-users report widespread interest in cannabis. The use of cannabis is often not reported and unknown for health care professionals, arguing for a vigilant approach to non-prescribed cannabis use in clinical follow-up of PwP.
Long-term adherence, safety, and efficacy of repeated onabotulinumtoxinA over five years in chronic migraine prophylaxis.
Argyriou AA, Dermitzakis EV, Vlachos GS … +1 more , Vikelis M
Acta Neurol Scand · 2022 Jun · PMID 35170031 · Publisher ↗
BACKGROUND: OnabotulinumtoxinA (BoNTA) demonstrated a positive benefit-risk in chronic migraine (CM) patients in PREEMPT I and II phase III trials and many subsequent real-world studies. We herein aimed at evaluating the...
BACKGROUND: OnabotulinumtoxinA (BoNTA) demonstrated a positive benefit-risk in chronic migraine (CM) patients in PREEMPT I and II phase III trials and many subsequent real-world studies. We herein aimed at evaluating the adherence to repeated BoNTA over a period of five years, while secondary objectives included the assessment of its long-term safety/efficacy and patients' satisfaction to treatment. METHODS: We studied 56 CM patients who had successfully received consequent cycles of BoNTA over five years. Adherence was calculated as the percentage of patients actively choosing to follow with repeated BoNTA treatment, as instructed. Safety and efficacy data were collected throughout the study period. The overall patients' belief in and satisfaction by the efficacy of treatment was assessed at last follow-up, using the self-report 7-point measure patient global impression of change (PGIC). RESULTS: A total of 36 (64.3%) out of 56 patients remained adherent to BoNTA over five years. Long-term BoNTA exposure was safe and well-tolerated, without severe side-effects justifying treatment discontinuation. The mean monthly headache days and associated clinical efficacy outcomes remained consistent and quite low at last follow-up with evidence of continuous improvements in headache monthly frequency between year three and over five years of therapy. All patients who were able to maintain treatment over five years (n = 36), remained very satisfied and scored at least 5 in PGIC. CONCLUSION: Considerably high adherence, considerable satisfaction and sustained safety/efficacy were observed in patients followed up for five years, supporting a favorable benefit/risk profile for consistently delivering long-term BoNTA in CM.
Cryptogenic stroke and TIA: Suggested diagnostic approach while waiting for evaluation and treatment guidelines.
Ratajczak-Tretel B, Lambert AT, Atar D … +1 more , Aamodt AH
Acta Neurol Scand · 2022 May · PMID 35146751 · Full text
BACKGROUND: Empiric strategies for secondary prevention in cryptogenic stroke and cryptogenic TIA are lacking. The best therapy to prevent recurrence depends on the cause of stroke. Attempting a correct diagnosis is ther...
BACKGROUND: Empiric strategies for secondary prevention in cryptogenic stroke and cryptogenic TIA are lacking. The best therapy to prevent recurrence depends on the cause of stroke. Attempting a correct diagnosis is therefore the fundamental goal of stroke treatment. Further investigation into the source of embolism if suspected, and determination of the etiology, even if demanding, is the needed prerequisite for optimal secondary prevention and risk reduction. AIMS: This paper discusses evaluation and treatment of cryptogenic stroke in light of recent years' clinical trials results and developments in cardiology and neuroradiology. No ethical approval was needed for this work. RESULTS: Cardioembolism due to paroxysmal atrial fibrillation, patent foramen ovale, or cardiomyopathy; occult atherosclerosis from unstable plaques and hypercoagulable conditions seem to be the most common underlying causes to be revealed by further investigations. Treatment of these conditions can reduce the stroke recurrence significantly. CONCLUSIONS: An individual approach and targeted diagnostics using advanced medical technologies in selected patients, who may benefit from a tailored treatment regimen, can help reveal a probable cause in the majority of strokes and TIAs previously classified as cryptogenic.
Epidemiological profile of myasthenia gravis in South Korea using the national health insurance database.
Park JS, Eah KY, Park JM
Acta Neurol Scand · 2022 May · PMID 35141872 · Publisher ↗
OBJECTIVES: Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue. Our objective was to investigate the incidence of MG using the National Health Insurance database of South Korea....
OBJECTIVES: Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue. Our objective was to investigate the incidence of MG using the National Health Insurance database of South Korea. MATERIALS AND METHODS: We conducted a retrospective cohort analysis of patients with the G70.0 code designated as MG and administered with MG medications for >3 months from 2007 to 2018 using nationwide data from South Korea. RESULTS: A total of 8,376 patients with MG during the period of 2010-2018 were identified. There were 3,862 (46.1%) male and 4,517 (53.9%) female patients. The standardized incidence rate was 1.18/100,000 in 2010, and increased to 1.81/100,000 in 2018. The standardized prevalence was 7.50/100,000 in 2010, and changed to 11.15/100,000 in 2018. Pyridostigmine was used to treat 82.3 ± 1.2% of patients with MG during 2010-2018. Among MG patients, 85.7 ± 0.9% used steroids, 31.6 ± 4.8% used azathioprine, 12.9 ± 9.5% used tacrolimus, 7.2 ± 2.1% used cyclosporine, 6.2 ± 1.8% used mycophenolate mofetil, and 0.4 ± 0.1% used methotrexate. Thymectomy was performed in 1,130 MG patients, and the time from MG diagnosis to thymectomy decreased from 2010 to 2018. CONCLUSION: Based on the national registry data from 2010 to 2018, the incidence and prevalence rate in South Korea has increased. Whereas the use of IVIG has remained stable, thymectomy is performed earlier than before, and the distribution of immunosuppressant therapies has changed over the years with an increase in tacrolimus and mycophenolate mofetil. We expect that this study will serve as a basis for future South Korean MG epidemiological studies.
Cognitive screen and employment long-term after infratentorial stroke.
Hofgren C, Samuelsson H, Klasson S … +3 more , Jern C, Sunnerhagen KS, Jood K
Acta Neurol Scand · 2022 May · PMID 35137393 · Publisher ↗
OBJECTIVES: Motor problems are well-described neurological deficits that occur commonly after an infratentorial ischemic stroke. However, the brain stem and cerebellum are also part of the neural interconnections respons...
OBJECTIVES: Motor problems are well-described neurological deficits that occur commonly after an infratentorial ischemic stroke. However, the brain stem and cerebellum are also part of the neural interconnections responsible for cognition, emotions, and behavioral responses. We lack studies on long-term cognitive outcomes and patient employment after an infratentorial stroke. In the present study, we described and compared long-term poststroke cognitive outcomes and employment between patients that experienced infratentorial and supratentorial ischemic strokes. MATERIALS AND METHODS: We included consecutive patients that experienced an acute ischemic stroke at ≤58 years of age. Patients were classified according to the stroke location. At seven years poststroke, surviving participants were assessed for neurological deficits (National Institutes of Health Stroke Scale [NIHSS]), functional outcome (modified Rankin Scale [mRS]), cognitive function Barrow Neurological Institute Screen (BNIS), and employment. RESULTS: Among 141 participants, 25 (18%) had infratentorial and 116 (82%) had supratentorial strokes. At the 7-year poststroke follow-up, there was no significant difference in BNIS total scores; with a median of 43 (IQR 40.5-46) and 41 (IQR 38-46) in the infratentorial and supratentorial groups, respectively. This result indicated that cognitive dysfunction occurred frequently in both groups. Similar employment rates were observed in the infratentorial (48%) and supratentorial (55%) groups. Both groups had a median NIHSS score of 0 and a median mRS score of 2 at the 7-year follow-up. CONCLUSION: Patients who survived an infratentorial or supratentorial ischemic stroke had similar rates of long-term cognitive dysfunction and difficulties in returning and/or remaining at work.
New-onset non-lesional aphasic status epilepticus. Clinical description, diagnostic clues, and treatment algorithm.
Jaraba Armas S, Sala-Padró J, Veciana M … +7 more , Arroyo P, Pedro J, Mora J, Fernandez M, Camins À, Rodriguez-Bel L, Falip M
Acta Neurol Scand · 2022 May · PMID 35130366 · Publisher ↗
OBJECTIVES: De novo aphasic status epilepticus (ASE) in patients without a previous history of epilepsy and without cerebral lesions (aphasic NOSE) is rare. The aim of the study is to describe its clinical characteristic...
OBJECTIVES: De novo aphasic status epilepticus (ASE) in patients without a previous history of epilepsy and without cerebral lesions (aphasic NOSE) is rare. The aim of the study is to describe its clinical characteristics, etiologies, and outcome. MATERIALS & METHODS: Single-center study including consecutive patients presenting to the emergency department between 2011 and 2019 with acute aphasia, which was finally diagnosed as aphasic NOSE. Subsequent episodes of aphasia (>5 min) were recorded and divided into confirmed ASE and postictal aphasic episodes (non-ASE). Clinical characteristics of the two types of episodes were compared. RESULTS: Nineteen patients were included, suffering fifty episodes of epileptic aphasia, episodes per patient 2.6 (range 1-7). Fifteen patients (71.4%) were women, mean age at ASE onset was 66.05 years old (SD 6.3). Nine (47%) patients died, 6 of them (66.7%) during the aphasic episode. Ictal EEG was available in 37 episodes, confirming the diagnosis of ASE in 12 episodes; in 8 episodes, the EEG fulfilled the criteria of possible ASE. The most frequent etiologies were inflammatory and vascular. Comparing ASE with non-ASE episodes, ASE was longer than non-ASE (225 vs 65 h, p .024) and was treated more frequently with BZD (76 vs 24%, p .001) but with a longer delay (22.2 vs 1.5 h, p .06). CONCLUSIONS: ASE is a treatable, highly relapsing emergency, with the subsequent relapses ASE or postictal aphasia. EEG is diagnostic in half of the patients, while in others imaging techniques are also useful. Benzodiazepines should be administered. Persistent aphasia, of more than 65 hours' duration, is highly suggestive of ASE.
Very late onset of myasthenia gravis. Comment: Comorbidities in older patients with myasthenia gravis.
Hernandez Fustes OJ
Acta Neurol Scand · 2022 Jun · PMID 35130364 · Publisher ↗
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Phenotypical spectrum of SACS variants: Neuromuscular perspective of a complex neurodegenerative disorder.
Çakar A, İnci M, Özdağ Acarlı AN … +7 more , Çomu S, Candayan A, Battaloğlu E, Tekgül Ş, Başak AN, Durmuş H, Parman Y
Acta Neurol Scand · 2022 May · PMID 35130357 · Publisher ↗
OBJECTIVES: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by the SACS gene variants. Main clinical features include early-onset and progressive cerebellar ataxia, spasticity, sensorimotor p...
OBJECTIVES: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by the SACS gene variants. Main clinical features include early-onset and progressive cerebellar ataxia, spasticity, sensorimotor polyneuropathy. However, the phenotypic spectrum expanded with the increased availability of next-generation sequencing methods. MATERIALS AND METHODS: Herein, we describe the clinical features of nine patients from seven unrelated families with SACS variants from the cohort of the Neuromuscular Disorders Unit of the Neurology Department of the Istanbul University, Istanbul Faculty of Medicine. RESULTS: Seven patients were male. Seven patients in our cohort had disease onset in the first decade of life. Eight patients were born to consanguineous marriages. Distal weakness in the lower limbs was a prominent feature in all of our patients. Seven patients had ataxia, and six patients had spasticity. Interestingly, one patient showed an isolated Charcot-Marie-Tooth-like phenotype. Five patients showed sensorimotor demyelinating polyneuropathy in the nerve conduction studies. Linear pontine hypointensity was the most frequent cranial magnetic resonance imaging (MRI) abnormality. Two patients with a later disease onset had a homozygous c.11542_11544delATT (p.Ile3848del) variant. The rest of the identified variants were scattered throughout the SACS gene. CONCLUSIONS: Atypical clinical features in our patients highlight that the phenotypic spectrum of ARSACS can be observed in a wide range.
Small fiber neuropathy.
Finsterer J, Scorza FA
Acta Neurol Scand · 2022 May · PMID 35130356 · Publisher ↗
Small fiber neuropathy (SFN) is a peripheral nervous system disease due to affection of A-delta or C-fibers in a proximal, distal, or diffuse distribution. Selective SFN (without large fiber affection) manifests with pai...
Small fiber neuropathy (SFN) is a peripheral nervous system disease due to affection of A-delta or C-fibers in a proximal, distal, or diffuse distribution. Selective SFN (without large fiber affection) manifests with pain, sensory disturbances, or autonomic dysfunction. Though uniform diagnostic criteria are unavailable, most of them request typical clinical features and reduced intra-epidermal nerve fiber density on proximal or distal skin biopsy. Little consensus has been reached about the treatment of SFN, why this narrative review aims at summarizing and discussing treatment options for SFN. Treatment of SFN can be classified as symptomatic, pathophysiologic, or causal. Prerequisites for treating SFN are an established diagnosis, knowledge about the symptoms and signs, and the etiology. Pain usually responds to oral/intravenous pain killers, antidepressants, anti-seizure drugs, or topical, transdermal specifications. Some of the autonomic disturbances respond favorably to symptomatic treatment. SFN related to Fabry disease or hATTR are accessible to pathogenesis-related therapy. Immune-mediated SFN responds to immunosuppression or immune-modulation. Several of the secondary SFNs respond to causal treatment of the underlying disorder. In conclusion, treatment of SFN relies on a multimodal concept and includes causative, pathophysiologic, and symptomatic measures. It strongly depends on the clinical presentation, diagnosis, and etiology, why it is crucial before initiation of treatment to fix the diagnosis and etiology. Due to the heterogeneous clinical presentation and multi-causality, treatment of SFN should be individualized with the goal of controlling the underlying cause, alleviating pain, and optimizing functionality.
Definition of drug-resistant epilepsy: A reappraisal based on epilepsy types.
Asadi-Pooya AA, Farazdaghi M
Acta Neurol Scand · 2022 May · PMID 35130355 · Publisher ↗
OBJECTIVE: To re-assess the definition of drug-resistant epilepsy based on the evidence from a large-scale, long-term study including both adults and children. We categorized the patients as idiopathic generalized epilep...
OBJECTIVE: To re-assess the definition of drug-resistant epilepsy based on the evidence from a large-scale, long-term study including both adults and children. We categorized the patients as idiopathic generalized epilepsies (IGEs), focal epilepsies, or structural-metabolic-genetic generalized epilepsies [symptomatic generalized epilepsies (SGEs)] and provided the definition of drug-resistance based on the epilepsy types of the patients. METHODS: This was a longitudinal study of a prospectively developed and maintained database. All patients with an electro-clinical diagnosis of IGE, focal epilepsy, or SGE, who received treatment from 2008 to 2021, were recruited at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Shiraz, Iran. All patients had to be followed at our center for at least 24 months. The receiver operating characteristic curve (ROC curve) was used for the statistical analysis. RESULTS: The included patients were as follows: 523 with focal epilepsy, 218 with IGE, and 211 with SGE. For all epilepsy types, the ROC curves of the number of appropriately prescribed antiseizure medications (ASMs) were acceptable indicators to anticipate drug-resistance. The best cutoff point for focal epilepsies was at 4 ASMs (sensitivity: 0.56, specificity: 0.81); for IGE, at 3 ASMs (sensitivity: 0.51, specificity: 0.80); and for SGEs, at 4 ASMs (sensitivity: 0.78, specificity: 0.58). CONCLUSION: The definition of drug-resistant epilepsy should be different in various epilepsy types. It is the time for the scientific community to reappraise the definition of drug-resistant epilepsy in the light of the new evidence that has become available in the past 11 years since the previously published definition.
Four Swedish cases of CSF1R-related leukoencephalopathy: Visualization of clinical phenotypes.
Rosenstein I, Andersen O, Victor D … +7 more , Englund E, Granberg T, Hedberg-Oldfors C, Jood K, Fitrah YA, Ikeuchi T, Danylaité Karrenbauer V
Acta Neurol Scand · 2022 May · PMID 35119108 · Full text
UNLABELLED: Colony stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy is a rare, genetic disease caused by heterozygous mutations in the CSF1R gene with rapidly progressive neurodegeneration, behavioral, c...
UNLABELLED: Colony stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy is a rare, genetic disease caused by heterozygous mutations in the CSF1R gene with rapidly progressive neurodegeneration, behavioral, cognitive, motor disturbances. OBJECTIVE: To describe four cases of CSF1R-related leukoencephalopathy from three families with two different pathogenic mutations in the tyrosine kinase domain of CSF1R and to develop an integrated presentation of inter-individual diversity of clinical presentations. METHODS: This is an observational study of a case series. Patients diagnosed with CSF1R encephalopathy were evaluated with standardized functional estimation scores and subject to analysis of cerebrospinal fluid biomarkers. Brain computed tomography (CT) and magnetic resonance imaging (MRI) were evaluated. We performed a functional phosphorylation assay to confirm the dysfunction of mutated CSF1R protein. RESULTS: Two heterozygous missense mutations in the CSF1R gene were identified, c.2344C>T; p.Arg777Trp and c.2329C>T; p.Arg782Cys. A phosphorylation assay in vitro showed markedly reduced autophosphorylation in cells expressing mutations. According to ACMG criteria, both mutations were pathogenic. A radiological investigation revealed typical white matter lesions in all cases. There was inter-individual diversity in the loss of cognitive, motor-neuronal, and extrapyramidal functions. CONCLUSIONS: Including the present cases, currently three CSF1R mutations are known in Sweden. We present a visualization tool to describe the clinical diversity, with potential use for longitudinal follow-up for this and other leukoencephalopathies.
Possible mechanisms of neuropathies associated with the COVID-19 vaccines.
Maria Mirabela M, Iulia E, Sorin T
Acta Neurol Scand · 2022 May · PMID 35119106 · Publisher ↗
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Theory of mind: A clue for the interpretation of functional movement disorders.
Silveri MC, Di Tella S, Lo Monaco MR … +7 more , Petracca M, Tondinelli A, Antonucci G, Pozzi G, Di Lazzaro G, Calabresi P, Bentivoglio AR
Acta Neurol Scand · 2022 May · PMID 35112347 · Full text
OBJECTIVES: Functional movement disorders (FMD) refer to a heterogeneous group of manifestations incongruent with known neurological diseases. Functional neuroimaging studies in FMD indicate the overlap between cerebral...
OBJECTIVES: Functional movement disorders (FMD) refer to a heterogeneous group of manifestations incongruent with known neurological diseases. Functional neuroimaging studies in FMD indicate the overlap between cerebral regions in which abnormal activation occurs and those considered crucial for theory of mind (ToM), the ability to attribute mental states. The aim of this study was to explore whether FMD might be related to ToM disorders to the extent that they reduce the ability to make inferences about the mental states underlying motor behaviour during social interaction. MATERIALS & METHODS: Eighteen subjects with FMD and 28 matched healthy controls (HC) were given a ToM battery. The severity of FMD was rated by the Simplified-FMD Rating Scale (S-FMDRS). Dissociative symptoms were evaluated by the Dissociative Experiences Scale (DES-II). RESULTS: FMD scored worse than the HC in most ToM tasks: second-order False Beliefs (p = .005), Faux-Pas Recognition Test (p < .001) and Reading the Mind in the Eyes Test (p = .020); control questions elicited normal scores. The DES-II indicated dissociative-borderline psychopathology and negatively correlated with accuracy on the second-order False Belief (Spearman's rho = -.444; p = .032); the positive correlation between DES-II and severity of motor symptoms (S-FMDRS) approached significance (Spearman's rho test = .392; p = .054). ToM disorders were not correlated with S-FMDRS, due to the typical variability in FMD over time with regard to the severity of symptoms and the district of body involved. CONCLUSIONS: Our results are consistent with the hypothesis that FMD are related to ToM deficits, and future studies are needed to define the specific nature of this relationship.
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