Nordic Neurology

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: 25 June 2024 | Viewed by 9242

Special Issue Editor

1. North Karelia Central Hospital, Joensuu, Finland
2. Clinical Neurosciences, University of Turku, 80210 Turku, Finland
Interests: clinical neuroepidemiology; clinical (adult) neurology; movement disorders; Huntington's disease; Parkinson's disease; Guillain-Barré syndrome; multiple sclerosis; stroke; amyotrophic lateral sclerosis, myoclonic epilepsy

Special Issue Information

Dear Colleagues,

In recent decades, clinical neurology and neuroscience have witnessed both marvelous advances and frustrating disappointments despite best efforts. The prognosis of some disorders has turned from dismal to (nearly) curable, whereas some diseases still lack treatment. Moreover, the etiology and pathomechanisms of a wide range of neurological disease remain uncertain—especially degenerative ones. This research theme covers a wide range of disorders affecting the nervous system, ranging from acute (strokes, traumatic brain injuries, infectious and autoimmune neurological diseases, etc.) to chronic (rare diseases, neurodegenerative disorders, autoimmune nervous system diseases, CNS tumors, etc.), as well as those in between (e.g., functional neurological disorders).

A collated Nordic perspective on these is expected to provide new insights. Nordic countries boast advanced welfare societies with equality and high-quality healthcare, although even they are affected by social and health disparities and uncertainty in this changing world. Advances and innovations in treatment protocols have been necessitated by low population densities and long distances and made possible by advanced public healthcare systems. Furthermore, the environment is quite different from where homo sapiens apparently originated, with populations small and often far apart. There are marked differences in the epidemiology of many diseases within Nordic countries as well as when compared to the rest of the world. These differences provide opportunities for etiological and pathogenesis research. All these research aspects are facilitated by Nordic high-quality registries.

This Special Issue aims to gather research that elucidates the current epidemiological landscapes, clinical pictures, pathological and genetic correlates, as well as treatment pathways and their outcomes in the Nordic region. We welcome original articles, reviews, and other acceptable types of articles We encourage authors to provide insights into the development status and research fronts of neurology, neuroepidemiology, and clinical neuroscience. We also welcome all suggestions of possible other authors for this theme issue.

Dr. Jussi Sipilä
Guest Editor

Manuscript Submission Information

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Keywords

  • clinical neurology
  • delivery of health care
  • genetics
  • neuroepidemiology
  • neurosciences
  • scandinavian 
  • nordic countries

Published Papers (7 papers)

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Research

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8 pages, 976 KiB  
Article
Characterization of the Increase in Narcolepsy following the 2009 H1N1 Pandemic in Sweden
J. Clin. Med. 2024, 13(3), 652; https://doi.org/10.3390/jcm13030652 - 23 Jan 2024
Viewed by 445
Abstract
(1) Background: In the context of the H1N1 pandemic and the Pandemrix vaccination campaign, an increased number of narcolepsy cases were noted in several countries. In Sweden, this phenomenon was attributed to the effect of the Pandemrix vaccination in the first place. Studies [...] Read more.
(1) Background: In the context of the H1N1 pandemic and the Pandemrix vaccination campaign, an increased number of narcolepsy cases were noted in several countries. In Sweden, this phenomenon was attributed to the effect of the Pandemrix vaccination in the first place. Studies from China indicated that narcolepsy could occur as a consequence of the H1N1 infection itself. We performed an analysis of the increase, with a specific interest in age and sex distribution. We also aimed to validate the origin of the excess cases, post hoc. (2) Methods: Data for narcolepsy patients (ICD code G 47.4, both type 1 and type 2) distributed by sex and age at 5-year intervals, annually between 2005 and 2017, were retrieved from the National Patient Register. Information on the total population was collected from the Swedish Population Register. (3) Results: The number of narcolepsy cases increased markedly from 2009 to 2014 compared to the period before 2009. A particular increase in 2011 among children and teenagers was observed. The sex ratio did not change significantly during the study period. (4) Conclusions: Our results support an association between the increased prevalence of narcolepsy cases and Pandemrix vaccination, but the effect of the virus itself cannot be ruled out as a contributing factor. Full article
(This article belongs to the Special Issue Nordic Neurology)
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12 pages, 951 KiB  
Article
Relapses and Serious Infections in Patients with Neuromyelitis Optica Spectrum Disorder Treated with Rituximab: A Swedish Single-Center Study
J. Clin. Med. 2024, 13(2), 355; https://doi.org/10.3390/jcm13020355 - 08 Jan 2024
Viewed by 698
Abstract
Neuromyelitis optica spectrum disorder (NMOSD) is a rare immune-mediated relapsing-remitting disease of the central nervous system. The usage of rituximab, as relapse-preventive therapy, in NMOSD is common. We performed a single-center retrospective cohort study to assess the risk of relapses and severe infectious [...] Read more.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare immune-mediated relapsing-remitting disease of the central nervous system. The usage of rituximab, as relapse-preventive therapy, in NMOSD is common. We performed a single-center retrospective cohort study to assess the risk of relapses and severe infectious events (SIEs) in rituximab-treated NMOSD patients. This study included 24 aquaporin-4 IgG+ (AQP4+), 8 myelin-oligodendrocyte-protein IgG+ (MOG+), and 10 double-seronegative NMOSD patients. Relapses were observed in 50% of all patients during a mean treatment time of 4.0 (range: 0.5–8.25) years. The incidence risk ratio (IRR) of relapse was three times higher in MOG+ compared to AQP4+ patients (IRR: 3.0, 95% confidence interval (CI); 1.2–7.7). SIEs occurred in 40% of all patients during follow-up. AQP4+ patients conferred an increased risk of SIEs compared to MOG+ patients (IRR; 5.3, 95% CI; 1.2–24.3). Incomplete CD19+ B-lymphocyte suppression was not correlated with relapse risk (hazard ratio; 1.9, 95% CI; 0.7–5.2), and there was no correlation between IgG-levels and SIE risk (odds ratio; 2.0, 95% CI; 0.8–4.8). In conclusion, considerable risks of both relapses and SIEs were observed in NMOSD patients exposed to rituximab, which underlines the need for close clinical vigilance of disease activity and infections during treatment. Full article
(This article belongs to the Special Issue Nordic Neurology)
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11 pages, 248 KiB  
Article
Absence of Oligoclonal Bands in Multiple Sclerosis: A Call for Differential Diagnosis
J. Clin. Med. 2023, 12(14), 4656; https://doi.org/10.3390/jcm12144656 - 13 Jul 2023
Cited by 1 | Viewed by 1667
Abstract
Background: Immunoglobulin gamma (IgG) oligoclonal bands (OCB) in the cerebrospinal fluid (CSF) are absent in a small group of multiple sclerosis (MS) patients. According to previous research, OCB-negative MS patients differ genetically but not clinically from OCB-positive MS patients. However, whether OCB-negative MS [...] Read more.
Background: Immunoglobulin gamma (IgG) oligoclonal bands (OCB) in the cerebrospinal fluid (CSF) are absent in a small group of multiple sclerosis (MS) patients. According to previous research, OCB-negative MS patients differ genetically but not clinically from OCB-positive MS patients. However, whether OCB-negative MS is a unique immunological and clinical entity remains unclear. The absence of OCB poses a significant challenge in diagnosing MS. (1) Objective: The objective of this study was twofold: (1) to determine the prevalence of OCB-negative MS patients in the Uppsala region, and (2) to assess the frequency of misdiagnosis in this patient group. (2) Methods: We conducted a retrospective study using data from the Swedish MS registry (SMSreg) covering 83% of prevalent MS cases up to 20 June 2020 to identify all MS patients in the Uppsala region. Subsequently, we collected relevant information from the medical records of all OCB-negative MS cases, including age of onset, gender, presenting symptoms, MRI features, phenotype, Expanded Disability Status Scale (EDSS) scores, and disease-modifying therapies (DMTs). (3) Results: Out of 759 MS patients identified, 69 had an OCB-negative MS diagnosis. Upon re-evaluation, 46 patients had a typical history and MRI findings of MS, while 23 had unusual clinical and/or radiologic features. An alternative diagnosis was established for the latter group, confirming the incorrectness of the initial MS diagnosis. The average EDSS score was 2.0 points higher in the MS group than in the non-MS group (p = 0.001). The overall misdiagnosis rate in the cohort was 33%, with 22% of misdiagnosed patients having received DMTs. (4) Conclusions: Our results confirm that the absence of OCB in the CSF should raise suspicion of possible misdiagnosis in MS patients and prompt a diagnostic reassessment. Full article
(This article belongs to the Special Issue Nordic Neurology)
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10 pages, 4865 KiB  
Article
Longitudinal Optical Coherence Tomography Measurement of Retinal Ganglion Cell and Nerve Fiber Layer to Assess Benign Course in Multiple Sclerosis
J. Clin. Med. 2023, 12(6), 2240; https://doi.org/10.3390/jcm12062240 - 14 Mar 2023
Viewed by 1341
Abstract
A benign form of multiple sclerosis (BMS) is not easily diagnosed, but changes of the retinal ganglion cell layer-inner plexiform layer (GCL-IPL) and retinal nerve fiber layer (RNFL) may be sensitive to the disease. The aim of this study was to use optical [...] Read more.
A benign form of multiple sclerosis (BMS) is not easily diagnosed, but changes of the retinal ganglion cell layer-inner plexiform layer (GCL-IPL) and retinal nerve fiber layer (RNFL) may be sensitive to the disease. The aim of this study was to use optical coherence tomography (OCT) to investigate longitudinal changes of GCL-IPL and RNFL in BMS. Eighteen patients with BMS and 22 healthy control (HC) subjects were included, with a mean follow-up period of 32.1 months in BMS and 34.3 months in HC. Mean disease duration in BMS was 23.3 years, with 14 patients left untreated. Unilateral optic neuritis (ON) was found in eight patients. Non-ON eyes showed thinner GCL-IPL layer in the BMS group relative to HC (p < 0.001). The thinning rate of GCL-IPL in non-ON BMS, however, was −0.19 ± 0.15 µm/year vs. 0 ± 0.11 µm/year for HC (p = 0.573, age-adjusted). Thinning rate of RNFL in non-ON BMS was −0.2 ± 0.27 µm/year vs. −0.05 ± 0.3 µm/year for HC (p = 0.454, age adjusted). Conclusions: Thinning rate of the GCL-IPL and RNFL in BMS is similar to the healthy population but differs from the thinning rate in relapsing-remitting MS, presenting a non-invasive OCT-based criterion for assessing a benign course in multiple sclerosis. Full article
(This article belongs to the Special Issue Nordic Neurology)
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9 pages, 1771 KiB  
Communication
The Importance of Optical Coherence Tomography in the Diagnosis of Atypical or Subclinical Optic Neuritis: A Case Series Study
J. Clin. Med. 2023, 12(4), 1309; https://doi.org/10.3390/jcm12041309 - 07 Feb 2023
Viewed by 1652
Abstract
Background: Optic neuritis (ON) is an inflammatory condition of the optic nerve. ON is associated with development of demyelinating diseases of the central nervous system (CNS). CNS lesions visualized by magnetic resonance imaging (MRI) and the finding of oligoclonal IgG bands (OB) in [...] Read more.
Background: Optic neuritis (ON) is an inflammatory condition of the optic nerve. ON is associated with development of demyelinating diseases of the central nervous system (CNS). CNS lesions visualized by magnetic resonance imaging (MRI) and the finding of oligoclonal IgG bands (OB) in the cerebrospinal fluid (CSF) are used to stratify the risk of MS after a “first” episode of ON. However, the diagnosis of ON in absence of typical clinical manifestations can be challenging. Methods and Materials: Here we present three cases with changes in the optic nerve and ganglion cell layer in the retina over the disease course. (1) A 34-year-old female with a history of migraine and hypertension had suspect amaurosis fugax (transient vision loss) in the right eye. This patient developed MS four years later. Optical coherence tomography (OCT) showed dynamic changes of the thickness of peripapillary retinal nerve fiber layer (RNFL) and macular ganglion cell-inner plexiform layer (GCIPL) over time. (2) A 29-year-old male with spastic hemiparesis and lesions in the spinal cord and brainstem. Six years later he showed bilateral subclinical ON identified using OCT, visual evoked potentials (VEP) and MRI. The patient fulfilled diagnosis criteria of seronegative neuromyelitis optica (NMO). (3) A 23-year-old female with overweight and headache had bilateral optic disc swelling. With OCT and lumbar puncture, idiopathic intracranial hypertension (IIH) was excluded. Further investigation showed positive antibody for myelin oligodendrocyte glycoprotein (MOG). Conclusions: These three cases illustrate the importance of using OCT to facilitate quick, objective and accurate diagnosis of atypical or subclinical ON, and thus proper therapy. Full article
(This article belongs to the Special Issue Nordic Neurology)
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9 pages, 654 KiB  
Communication
Treatment Courses of Patients Newly Diagnosed with Multiple Sclerosis in 2012–2018
J. Clin. Med. 2023, 12(2), 595; https://doi.org/10.3390/jcm12020595 - 11 Jan 2023
Cited by 1 | Viewed by 1124
Abstract
Treatment options for multiple sclerosis (MS) are now numerous, but it is unclear which Disease-Modifying Treatment (DMT) is the optimal choice for a given patient. Treatment switches are common, both because of side effects and because of lack of efficacy. There are few [...] Read more.
Treatment options for multiple sclerosis (MS) are now numerous, but it is unclear which Disease-Modifying Treatment (DMT) is the optimal choice for a given patient. Treatment switches are common, both because of side effects and because of lack of efficacy. There are few data available on the treatment courses of patients newly diagnosed with MS in the current DMT era. All patients newly diagnosed with MS in 2012–2018 at North Karelia Central Hospital were identified (N = 55), and those with complete follow-up data available (N = 43) were included. The minimum follow-up from diagnosis was 44 months with a maximum of 9 years. Seven patients (16%) had no DMT at any time during the follow-up. Treatment was most often initiated with interferon or glatiramer acetate (69%), but 72% of these treatments were discontinued. After cladribine, teriflunomide and fingolimod showed the best treatment persistence. Patients who experienced their first MS symptoms at ≥40 years of age all continued with their initial treatment category until the end of the follow-up. In a third of the patients who had received a DMT, at the end of the follow-up, the treatment had been escalated to fingolimod, cladribine or natalizumab. Only 13 patients (28%) continued with their initial DMT until the end of the follow-up. Full article
(This article belongs to the Special Issue Nordic Neurology)
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Review

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19 pages, 1577 KiB  
Review
Adult-Onset Neuroepidemiology in Finland: Lessons to Learn and Work to Do
J. Clin. Med. 2023, 12(12), 3972; https://doi.org/10.3390/jcm12123972 - 11 Jun 2023
Viewed by 1521
Abstract
Finland is a relatively small genetic isolate with a genetically non-homogenous population. Available Finnish data on neuroepidemiology of adult-onset disorders are limited, and this paper describes the conclusions that can be drawn and their implications. Apparently, Finnish people have a (relatively) high risk [...] Read more.
Finland is a relatively small genetic isolate with a genetically non-homogenous population. Available Finnish data on neuroepidemiology of adult-onset disorders are limited, and this paper describes the conclusions that can be drawn and their implications. Apparently, Finnish people have a (relatively) high risk of developing Unverricht-Lundborg disease (EPM1), Multiple Sclerosis (MS), Amyotrophic Lateral Sclerosis (ALS), Spinal muscular atrophy, Jokela type (SMAJ) and adult-onset dystonia. On the other hand, some disorders, such as Friedreich’s ataxia (FRDA) and Wilson’s disease (WD), are almost absent or completely absent in the population. Valid and timely data concerning even many common disorders, such as stroke, migraine, neuropathy, Alzheimer’s disease and Parkinson’s disease, are unavailable, and there are virtually no data on many less-common neurological disorders, such as neurosarcoidosis or autoimmune encephalitides. There also appear to be marked regional differences in the incidence and prevalence of many diseases, suggesting that non-granular nationwide data may be misleading in many cases. Concentrated efforts to advance neuroepidemiological research in the country would be of clinical, administrative and scientific benefit, but currently, all progress is blocked by administrative and financial obstacles. Full article
(This article belongs to the Special Issue Nordic Neurology)
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