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Protocol of a randomized, double-blind, placebo-controlled, parallel-group, multicentre study of the efficacy and safety of nicotinamide in patients with Friedreich ataxia (NICOFA)
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Kathrin Reetz , Ralf-Dieter Hilgers , Susanne Isfort , Marc Dohmen , Claire Didszun , Kathrin Fedosov , Jennifer Kistermann , Caterina Mariotti , Alexandra Durr , Sylvia Boesch , Thomas Klopstock , Francisco Javier Rodríguez de Rivera Garrido , Ludger Schöls , Thomas Klockgether , Massimo Pandolfo , Rudolf Korinthenberg , Philip Lavin , Geert Molenberghs , Vincenzo Libri , Paola Giunti , Richard Festenstein , Jörg B. Schulz , the EFACTS or NICOFA study group
Yıl :
2019
Neurological Research and Practice
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Abstract Introduction Currently, no treatment that delays with the progression of Friedreich ataxia is available. In the majority of patients Friedreich ataxia is caused by homozygous pathological expansion of GAA repeats in the first intron of the FXN gene. Nicotinamide acts as a histone deacetylase inhibitor. Dose escalation studies have shown, that short term treatment with dosages of up to 4 g/day increase the expression of FXN mRNA and frataxin protein up to the levels of asymptomatic heterozygous gene carriers. The long-term effects and the effects on clinical endpoints, activities of daily living and quality of life are unknown. Methods The aim of the NICOFA study is to investigate the efficacy and safety of nicotinamide for the treatment of Friedreich ataxia over 24 months. An open-label dose adjustment wash-in period with nicotinamide (phase A: weeks 1–4) to the individually highest tolerated dose of 2–4 g nicotinamide/day will be followed by a 2 (nicotinamide group): 1 (placebo group) randomization (phase B: weeks 5–104). In the nicotinamide group, patients will continue with their individually highest tolerated dose between 2 and 4 g/d per os once daily and the placebo group patients will be receiving matching placebo. Safety assessments will consist of monitoring and recording of all adverse events and serious adverse events, regular monitoring of haematology, blood chemistry and urine values, regular measurement of vital signs and the performance of physical examinations including cardiological signs. The primary outcome is the change in the Scale for the Assessment and Rating of Ataxia (SARA) over time as compared with placebo in patients with Friedreich ataxia based on the linear mixed effect model (LMEM) model. Secondary endpoints are measures of quality of life, functional motor and cognitive measures, clinician’s and patient’s global impression-change scales as well as the up-regulation of the frataxin protein level, safety and survival/death. Perspective The NICOFA study represents one of the first attempts to assess the clinical efficacy of an epigenetic therapeutic intervention for this disease and will provide evidence of possible disease modifying effects of nicotinamide treatment in patients with Friedreich ataxia. Trial registration EudraCT-No.: 2017-002163-17, ClinicalTrials.gov NCT03761511.
DOI : 10.1186/s42466-019-0038-9
Anahtar Kelimeler : Clinical study design, Clinical trials, Ataxia, Outcome, Frataxin
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 9
Sınıflandırma :
2
Primary angiitis of the CNS (PACNS) and Behçet disease
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Peter Berlit , Markus Krämer , for the Consensus Group
Yıl :
2019
Neurological Research and Practice
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Abstract Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache. This guideline was developed in order to support clinicians in the diagnosis and treatment of primary angiitis of the CNS (PACNS) and Neuro-Behçet. It is based on a medline research and was developed in a modified Delphi process and approved by the involved societies. This article is an abridged and translated version of the guideline published in DGNeurologie: Berlit, P. & Krämer, M. DGNeurologie (2018) 1: 17. https://doi.org/10.1007/s42451-018-0001-y
DOI : 10.1186/s42466-019-0014-4
Anahtar Kelimeler : Primary angiitis of the CNS (PACNS), Reversible cerebral vasoconstriction syndrome (RCVS), Behçet disease, Neuro-Behçet, Isolated angiitis of the CNS
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 7
3
Human immunodeficiency virus and multiple sclerosis: a review of the literature
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Maria-Ioanna Stefanou , Markus Krumbholz , Ulf Ziemann , Markus C. Kowarik
Yıl :
2019
Neurological Research and Practice
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Abstract Multiple sclerosis (MS) and human immunodeficiency virus (HIV) infection are frequent and well-studied nosological entities. Yet, comorbidity of MS and HIV has only been rarely reported in the medical literature. We conducted a literature search using the databases PubMed, Ovid and Google Scholar, with the aim of identifying published studies and reports concerning HIV and MS. Recent epidemiological studies indicated a negative association between MS and HIV in terms of a reduced risk of developing MS in HIV positive patients. Accumulating clinical evidence additionally suggests a possibly reduced relapse rate of MS in HIV patients. Nevertheless, it remains currently unclear whether this observed inverse correlation could be due to the HIV infection itself, HIV treatment or the combination of both. Among the limited cases of MS in HIV infected patients, MS occurrence was mainly reported during acute HIV infection or during HIV seroconversion. This finding is in line with reports of HIV-related autoimmune disorders, which also occur in early phases of HIV disease. Beneficial effects of antiretroviral therapy on MS activity were reported in few clinical cases. Yet, the single phase II clinical trial (INSPIRE), which investigated the effects of antiretroviral medication (using the integrase inhibitor raltegravir) in patients with relapsing-remitting MS, failed to corroborate any beneficial effects at group level. Nevertheless, recently published experimental evidence suggests that HIV treatments may hold therapeutic potential for MS treatment. Thus, further studies are warranted to firstly, delineate the immunological mechanisms underlying possible efficacy of HIV treatments in MS, and to secondly, assess whether repurposing of HIV drugs for MS could be a worthwhile future research objective.
DOI : 10.1186/s42466-019-0030-4
Anahtar Kelimeler : Human immunodeficiency virus, Multiple sclerosis, Antiretroviral therapy, Chemokine inhibitors, Acquired immune
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 7
Sınıflandırma :
Viroloji %72
4
Acute intermittent porphyria (AIP) in a patient with celiac disease
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Sebastian Nunnemann , Christoph Uibel , Petra Budig , Mathias Mäurer
Yıl :
2020
Neurological Research and Practice
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Abstract We present the case of an 18 year old Caucasian with known celiac disease, who suffered a severe first attack of acute intermittent porphyria (AIP) with neuropsychiatric symptoms, severe tetraparesis and respiratory insufficiency. Treatment with heme arginate and high-dose intravenous glucose and rigorous rehabilitation resulted in a slow but almost complete recovery of her motor symptoms. To our knowledge this is the first case of acute intermittent porphyria triggered by malnutrition in the context of celiac disease. It is remarkable that the patient showed a favourable outcome despite the severity of her initial symptoms. This case shows the importance of early and systematic symptomatic treatment in patients with severe neurologic manifestation of AIP.
DOI : 10.1186/s42466-020-0049-6
Anahtar Kelimeler : Acute intermittent porphyria, Celiac disease, Polyneuropathy, Neurological rehabilitation
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 2
Sınıflandırma :
5
Position paper: ability to drive in cerebrovascular diseases
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Peter Marx , Gerhard Hamann , Otto Busse , Thomas Mokrusch , Hendrik Niemann , Hartmut Vatter , Bernhard Widder
Yıl :
2019
Neurological Research and Practice
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Abstract The regulations for fitness to drive after a cerebrovascular accident in the German Driving License Regulations (FeV) and the German Evaluation Guidelines for Driving Ability (BGL). are not up to date with the current medical knowledge and not consistent with regulations regarding cardiovascular diseases. This position paper presented by six medical and neuropsychological societies in Germany provides a guideline for the assessment of driving ability after diagnosis of a cerebrovascular disease and addresses three major questions: If there is a functional limitation, how can it be compensated for? What is the risk of sudden loss of control while driving in the future? Are there behavioral or personality changes or cognitive deficiencies interfering with safety while driving? Recommendations for the assessment of driving ability in different cerebrovascular diseases are presented. This article is a translation of the position paper published in Nervenarzt: Marx, P., Hamann, G.F., Busse, O. et al. Nervenarzt 90(4): 388–398.
DOI : 10.1186/s42466-019-0043-z
Anahtar Kelimeler : Stroke, Transient ischemic attack, Intracerebral bleeding, Subarachnoid bleeding, Informed consent, Fitness to drive
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 12
Sınıflandırma :
6
PIMIDES I: a pilot study to assess the feasibility of patient-controlled neurostimulation with the EASEE® system to treat medically refractory focal epilepsy
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Kristina Kravalis , Andreas Schulze-Bonhage
Yıl :
2020
Neurological Research and Practice
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Abstract The study design of PIMIDES, a trial based on patient-individualized transcranial electric neurostimulation of epileptic foci, is reported. Inclusion criteria include a predominant epileptic focus and pharmacoresistance to two antiepileptic drug treatments. The study is prospective, unblinded, and serves to assess the safety of subgaleal implantation and transcranial stimulation.
DOI : 10.1186/s42466-020-00061-5
Anahtar Kelimeler : Medical refractory epilepsy, Neurostimulation (NS), Responsive focus-stimulation
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 3
Sınıflandırma :
7
Autoimmune encephalitis in children and adolescents
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
C. G. Bien , C. I. Bien
Yıl :
2020
Neurological Research and Practice
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Abstract Background Autoimmune encephalitides with neural and glial antibodies have become an attractive field in neurology because the antibodies are syndrome-specific, explain the pathogenesis, indicate the likelihood of an underlying tumor, and often predict a good response to immunotherapy. The relevance and the management of antibody-associated encephalitides in the pediatric age group are to be discussed. Main body Subacutely evolving, complex neuropsychiatric conditions that are otherwise unexplained should raise the suspicion of autoimmune encephalitis. Determination of autoantibodies is the key diagnostic step. It is recommended to study cerebrospinal fluid and serum in parallel to yield highest diagnostic sensitivity and specificity. The most frequently found antibodies are those against the N-methyl-D-asparate receptor, an antigen on the neural cell surface. The second most frequent antibody is directed against glutamic acid decarboxylase 65 kDa, an intracellular protein, often found in chronic conditions with questionable inflammatory activity. Immunotherapy is the mainstay of treatment in autoimmune encephalitides. Steroids, apheresis and intravenous immunoglobulin are first-line interventions. Rituximab or cyclophosphamide are given as second-line treatments. Patients with surface antibodies usually respond well to immunotherapy whereas cases with antibodies against intracellular antigens most often do not. Conclusion With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.
DOI : 10.1186/s42466-019-0047-8
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 8
Sınıflandırma :
8
German hospital capacities for prolonged mechanical ventilator weaning in neurorehabilitation – results of a representative survey
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Thomas Platz , Andreas Bender , Christian Dohle , Anna Gorsler , Stefan Knecht , Joachim Liepert , Thomas Mokrusch , Michael Sailer
Yıl :
2020
Neurological Research and Practice
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Abstract A brief survey among members of the German Neurorehabilitation Society aimed to document the hospital capacities (“beds”) for prolonged weaning from a mechanical ventilator for patients with neuro-disabilities that require simultaneous multi-professional neurorehabilitation treatment. Sixty-eight institutions declared to have capacities with a broad distribution across Germany and its federal states. Overall, 1094 “beds” for prolonged weaning (and neurorehabilitation) were reported, 871 together with further information regarding their identification and hence regional location. These units had on average 16.1 beds for prolonged weaning (95% confidence interval 12.6 to 19.6) with a range from 2 to 68 beds per organization. The data indicate substantial capacities for the combined prolonged weaning and neurorehabilitation treatment in Germany. For most “beds” included in this analysis a basic validation was possible. While a reasonable coverage of these specialized service capacities by the survey is likely, the number reported could still be biased by underreporting by non-response. Both the broad variation of number of “beds” for prolonged weaning per unit and their unequal geographical distribution across federal states (per capita rate) warrant a more refined follow-up survey that will provide insights into reasons for the observed pattern of variation for these specialized hospital capacities.
DOI : 10.1186/s42466-020-00065-1
Anahtar Kelimeler : Neurorehabilitation, Weaning, Neuro-disabilities, Hospital capacity
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 4
Sınıflandırma :
9
Prevention of migraine with monoclonal antibodies against CGRP or the CGRP receptor
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Hans-Christoph Diener , Stefanie Förderreuther , Charly Gaul , Florian Giese , Till Hamann , Dagny Holle-Lee , Tim P. Jürgens , Katharina Kamm , Torsten Kraya , Christian Lampl , Arne May , Uwe Reuter , Armin Scheffler , Peer Tfelt-Hansen
Yıl :
2020
Neurological Research and Practice
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Abstract Monoclonal antibodies against the calcitonin gene-related peptide (CGRP) receptor (Erenumab) or against CGRP (Eptinezumab, Fremanezumab, Galcanezumab) are new substances for the preventive treatment of migraine. They represent an extension of the therapeutic options, which already exist in migraine prevention. In randomized, placebo-controlled studies, the efficacy and good tolerability of these specific substances have been demonstrated in patients with episodic and chronic migraine. The following treatment recommendation presents a summary of the pivotal studies. Recommendations are provided for the targeted selection of patients as well as for the evaluation of therapeutic success and the duration of treatment. Finally, possible restrictions on the use of this new substance group are discussed. This guideline is an abridged and translated version of the guideline published by Diener H-C, May A et al., Prevention of migraine with monoclonal antibodies against CGRP or the CGRP receptor, Supplement to S1 Guideline Therapy of Migraine Attack and Prevention of Migraine, 2019, Deutsche Gesellschaft für Neurologie (eds.), Guidelines for Diagnostics and Therapy in Neurology. A complete version of this guideline can be found on the website of the Deutsche Gesellschaft für Neurologie ( www.dgn.org/leitlinien ) and the AWMF (Arbeitsgemeinschaft wissenschaftlicher Medizinischer Gesellschaften). This guideline has been approved by the German Neurological Society (DGN) and the German Migraine and Headache Society (GMHS) and was reviewed by the two societies.
DOI : 10.1186/s42466-020-00057-1
Anahtar Kelimeler : Episodic migraine, Chronic migraine, Migraine prevention, Monoclonal antibodies, Guideline
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 6
Sınıflandırma :
10
Cerebral involvement in systemic vasculitides
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Peter Berlit , Markus Krämer , for the Consensus Group
Yıl :
2019
Neurological Research and Practice
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Abstract Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache. This guideline was developed in order to support clinicians in the diagnosis and treatment of cerebral manifestations of systemic vasculitides and rheumatic diseases. It is based on a medline research and was developed in a modified Delphi process and approved by the involved societies. This article is an abridged and translated version of the guideline published in DGNeurologie: Berlit, P. & Krämer, M. DGNeurologie (2018) 1: 17. https://doi.org/10.1007/s42451-018-0001-y
DOI : 10.1186/s42466-019-0016-2
Anahtar Kelimeler : Cerebral vasculitis, Giant cell Arteriitis (GCA), Takayasu arteritis (TA), Polyarteritis nodosa (PAN), ANCA-associated Vasculitides (AAV), Granulomatosis with polyangiitis (GPA)
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 16
11
Diagnosis of peripheral neuropathy
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Helmar C. Lehmann , Gilbert Wunderlich , Gereon R. Fink , Claudia Sommer
Yıl :
2020
Neurological Research and Practice
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Abstract Introduction Peripheral neuropathy represents a spectrum of diseases with different etiologies. The most common causes are diabetes, exposure to toxic substances including alcohol and chemotherapeutics, immune-mediated conditions, and gene mutations. A thorough workup including clinical history and examination, nerve conduction studies, and comprehensive laboratory tests is warranted to identify treatable causes. First steps The variability of symptoms allows distinguishing characteristic clinical phenotypes of peripheral neuropathy that should be recognized in order to stratify the diagnostic workup accordingly. Nerve conduction studies are essential to determine the phenotype (axonal versus demyelinating) and severity. Laboratory tests, including genetic testing, CSF examination, nerve imaging, and nerve biopsy, represent additional clinical tests that can be useful in specific clinical scenarios. Comments We propose a flow chart based on five common basic clinical patterns of peripheral neuropathy. Based on these five clinical phenotypes, we suggest differential diagnostic pathways in order to establish the underlying cause. Conclusions The recognition of characteristic clinical phenotypes combined with nerve conduction studies allows pursuing subsequent diagnostic pathways that incorporate nerve conduction studies and additional diagnostic tests. This two-tiered approach promises higher yield and better cost-effectiveness in the diagnostic workup in patients with peripheral neuropathy.
DOI : 10.1186/s42466-020-00064-2
Anahtar Kelimeler : Peripheral neuropathy, Diagnosis, Nerve conduction studies, Hereditary amyloid transthyretin (ATTRv) amyloidosis
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 7
Sınıflandırma :
12
Symptoms and probabilistic anatomical mapping of lacunar infarcts
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Ewgenia Barow , Hans Pinnschmidt , Florent Boutitie , Alina Königsberg , Martin Ebinger , Matthias Endres , Jochen B. Fiebach , Jens Fiehler , Vincent Thijs , Robin Lemmens , Keith W. Muir , Norbert Nighoghossian , Salvador Pedraza , Claus Z. Simonsen , Christian Gerloff , Götz Thomalla , Bastian Cheng , on behalf of the WAKE-UP investigators
Yıl :
2020
Neurological Research and Practice
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Abstract Background The anatomical distribution of acute lacunar infarcts has mainly been studied for supratentorial lesions. In addition, little is known about the association with distinct stroke symptoms, not summarized as classical lacunar syndromes. We aimed to describe the spatial lesion distribution of acute supra- and infratentorial lacunar infarcts and their association with stroke symptoms in patients eligible for thrombolysis. Methods All patients enrolled in the WAKE-UP trial (efficacy and safety of magnetic resonance imaging [MRI]-based thrombolysis in wake-up stroke) were screened for lacunar infarcts on diffusion-weighted imaging (DWI). The relationship between the anatomical distribution of supra- and infratentorial lacunar infarcts, their demographic characteristics and acute stroke symptoms, defined by the National Institutes of Health Stroke Scale (NIHSS) score, were correlated and compared. Results Maps of lesion distribution from 224 lacunar infarct patients (76 [33.9%] females, mean age [standard deviation] of 63.4 [11.5] years) were generated using computational image mapping methods. Median infarct volume was 0.73 ml (interquartile range [IQR] 0.37–1.15 ml). Median NIHSS sum score on hospital arrival was 4 (IQR 3–6). 165 (73.7%) patients had lacunar infarcts in the supratentorial deep white or grey matter, while 59 (26.3%) patients had infratentorial lacunar infarcts. Patients with supratentorial lacunar infarcts presented with a significantly lower occurrence of deficits in the NIHSS items gaze (p < 0.001) and dysarthria (p = 0.008), but had more often a paresis of the left arm (p = 0.009) and left leg (p = 0.068) compared to patients with infratentorial infarcts. Conclusions The anatomical lesion distribution of lacunar infarcts reveals a distinct pattern and supports an association of localization with different stroke symptoms. Trial registration NCT01525290 .
DOI : 10.1186/s42466-020-00068-y
Anahtar Kelimeler : Lacunar infarct, Magnetic resonance imaging, Lesion distribution, Probabilistic atlas, WAKE-UP
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 7
Sınıflandırma :
13
SOP: antibody-associated autoimmune encephalitis
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Rosa Rössling , Harald Prüss
Yıl :
2020
Neurological Research and Practice
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Abstract Background Antibody-mediated and paraneoplastic autoimmune encephalitides (AE) present with a broad spectrum of clinical symptoms. They often lead to progressing inflammatory changes of the central nervous system with subacute onset and can cause persistent brain damage. Thus, to promptly start the appropriate and AE-specific therapy, recognition of symptoms, initiation of relevant antibody diagnostics and confirmation of the clinical diagnosis are crucial, in particular as the diseases are relatively rare. Aim This standard operating procedure (SOP) should draw attention to the clinical presentation of AE, support the diagnostic approach to patients with suspected AE and guide through the necessary steps including therapeutic decisions, tumour screening and exclusion of differential diagnoses. Method Based on existing diagnostic algorithms, treatment recommendations and personal experiences, this SOP gives an overview of clinical presentation, diagnostic procedures and therapy in AE. Additional information is provided within an accompanying text and a table describing the most important autoantibodies and their characteristics. Results The initial steps of the AE flow chart are based on clinical symptoms and the patient’s history. Assignment to paraneoplastic or antibody-mediated AE is sometimes clinically possible. Diagnostics should include MRI, EEG and CSF analysis with antibody panel diagnostic. Definite AE can be diagnosed if the underlying antibody is compatible with the clinical presentation. Classification of probable AE may be possible even with negative anti-neuronal autoantibodies if the clinical presentation and laboratory abnormalities are highly suggestive of AE. The confirmed AE diagnosis requires immediate initiation of immunotherapy. Conclusion The SOP facilitates the recognition of patients with AE and presents the necessary diagnostic and therapeutic steps.
DOI : 10.1186/s42466-019-0048-7
Anahtar Kelimeler : Autoimmune, Encephalitis, Limbic encephalitis, Antibody, Paraneoplastic, NMDAR
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 7
Sınıflandırma :
14
SOP: First-ever epileptic seizure in adult patients
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Julian Bösel
Yıl :
2019
Neurological Research and Practice
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Abstract Background About 5% of all adults will have at least one epileptic seizure in their life. The incidence of all unprovoked seizures ranges from approximately 50 to 70 /100,000. The very first epileptic seizure in an adult can be a very decisive event and demands a great deal of responsibility on the part of the treating clinician. Optimal clinical work-up and systematic decision-making are necessary to ensure adequate treatment as well as to avoid unnecessary treatment, such as life-long application of anticonvulsants that may not be indicated. Aim To present a pragmatic standard operating procedure (SOP) for approaching the first seizure in adults. Method Based on current recommendations and personal suggestions, an SOP in the form of a flow chart accompanied with topical explanations and tables was created. Results Approaching the first seizure should start with obtaining bystander information on the seizure and its clinical features. Then, differential diagnoses should be considered. The diagnostic work-up hast to contain a neurological and physical examination, emergency blood tests and cerebral imaging. This should allow to differentiate an unprovoked from an acute symptomatic seizure, i.e. triggered by current specific and identifiable structural or metabolic cause that should be eliminated if possible. In the case of an unprovoked seizure, estimation of seizure recurrence is necessary for the decision to start treatment with antiepileptic drugs. Conclusion The challenge of diagnostic work-up and treatment decisions after a first epileptic seizure in adults may be facilitated by a systematic, SOP-based approach.
DOI : 10.1186/s42466-019-0006-4
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 6
Sınıflandırma :
15
Functional stroke outcomes after mobile stroke unit deployment – the revised protocol for the Berlin Prehospital Or Usual Delivery of acute stroke care (B_PROUD) part 2 study
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Peter Harmel , Martin Ebinger , Erik Freitag , Ulrike Grittner , Irina Lorenz-Meyer , Ira Napierkowski , Christian H. Nolte , Bob Siegerink , Heinrich J. Audebert
Yıl :
2019
Neurological Research and Practice
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Abstract Background Studies investigating the Mobile Stroke Unit (MSU) concept have shown increased thrombolysis rates, reduced alarm-to-treatment times and improved prehospital triage. Yet, so far, there is no definite scientific proof of better functional outcome after MSU deployment compared to regular ambulances. Methods We provide a revised protocol for the second part of the B_PROUD trial as organization of the MSU dispatch did not meet the anticipated standards in the first part. B_PROUD is a pragmatic, prospective study comparing functional outcomes of treatment candidates with or without MSU care. Treatment candidates are defined as patients with a final diagnosis of ischemic stroke or transient ischemic attack, onset-to-dispatch-times ≤4 h, disabling symptoms not resolved at time of ambulance arrival, and the ability to ambulate prior to the qualifying event. These patients are included if their emergency call prompted a stroke alarm at the dispatch center during MSU operation hours (7 am–11 pm, Monday-Sunday) and if the emergency is located within the MSU operation area in Berlin, Germany. The intervention group consists of patients who are cared for by the MSU. When the MSU is already in operation for another emergency, MSU dispatches are handled by regular ambulances (about 45%). These dispatches create the control group. Blinded stroke physicians assess the modified Rankin Scale (mRS) score in recorded structured interviews 3 months after stroke. The primary outcome is the degree of disability and death over the full range of the mRS. As a change to the previously published protocol and only pertinent in case of more than 9% lost-to-follow-up, a co-primary outcome was introduced consisting of the proportions of death, new institutional care or severe disability in patients with additional use of information from registration offices. Perspective The results will inform parties involved in acute stroke care organization on the effectiveness of the MSU concept. Trial registration The protocol is registered in (NCT03931616) and has been approved by the ethical review committee of the Charité – University Medicine Berlin (EA4/109/15) on September 2, 2015. The study protocol of B_PROUD part 1 had been published in the International Journal of Stroke as “Berlin Prehospital Or Usual Delivery of acute stroke care (B_PROUD) – study protocol” (doi: https://doi.org/10.1177/1747493017700152) on March 22, 2017 [1] previous to first patient’s registration.
DOI : 10.1186/s42466-019-0022-4
Anahtar Kelimeler : Stroke, Prehospital, Mobile stroke unit, Thrombolysis, Endovascular thrombectomy, Functional outcome
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 5
Sınıflandırma :
16
Socioeconomic decline and advancement within and between generations and the risk of stroke - a case-control study
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Armin J. Grau , Annette Aigner , Christian Urbanek , Frederik Palm , Florian Buggle , Anton Safer , Heiko Becher
Yıl :
2019
Neurological Research and Practice
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Abstract Background Disadvantageous socioeconomic conditions (SEC) in both childhood and adulthood increase the risk of stroke. We investigated whether intergenerational and lifetime social advancement decreases and/or social descent increases stroke risk. Methods In a case-control study with 466 patients with first-ever ischemic stroke and 807 controls randomly selected from the general population, we compared paternal profession to subjects’ professional education in adolescence and their last profession in adulthood. Furthermore, we constructed a socioeconomic risk score for childhood (based on paternal and maternal profession and occupation, familial, living and material conditions), adolescence (based on highest school degree and professional education), and adulthood (based on last profession, periods of unemployment, and marital status), and compared subjects´ positions at different life stages. Odds ratios were derived based on conditional logistic regression conditioning on age and sex only, after adjustment for medical and lifestyle risk factors, and after additional adjustment for socioeconomic risk score values. Results Intergenerational upward mobility between paternal profession and subject’s professional education was associated with lower ischemic stroke risk independent of medical and lifestyle risk factors (odds ratio (OR) 0.58; 95% confidence interval (CI) 0.41–0.81) and after additional adjustment for socioeconomic conditions in all three life stages (OR 0.67; 95% CI 0.45–0.99). Advancement between fathers´ profession and subject’s last profession was associated with reduced odds of stroke after adjustment for risk factors (OR 0.65; 95% CI 0.47–0.89), but not significantly after additional adjustment for SEC (OR 0.77; 95% CI 0.52–1.13). Social descent between adolescence and adulthood indicated by the transition into a more disadvantageous tertile of socioeconomic risk score was associated with increased odds of stroke after adjustment for all risk factor (OR 2.93; 95% CI 1.21–7.13). Analyses by sex revealed mostly similar results in men and women with only few potential differences. Conclusions Our study results indicate that aspects of social downward mobility during adulthood may be associated with increased risk of stroke, whereas intergenerational upward mobility may be linked to a lower stroke risk. If confirmed by future studies, such results may help to focus stroke prevention measures at high risk populations.
DOI : 10.1186/s42466-019-0012-6
Anahtar Kelimeler : Socioeconomic conditions, Risk factor, Ischemic stroke
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 6
Sınıflandırma :
17
Ulnar nerve integrity predicts 1-year outcome in cervical spinal cord injury
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Andreas Hug , Christian Schuld , Bettina Mürle , Markus Böttinger , EMSCI study group , Norbert Weidner , Rüdiger Rupp
Yıl :
2019
Neurological Research and Practice
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Abstract Background Accurate predictors of neurological recovery after cervical spinal cord injury are needed. Particularly, to tailor adequate rehabilitation plans. However, objective and quantifiable predictors are sparse. Methods Within the prospective European Multicenter Study about Spinal Cord Injury (EMSCI) registry, cervical spinal cord injury patients are monitored at fixed follow up visits (2, 4, 12, 24, and 48 weeks after injury) clinically and with ulnar nerve electroneurography. Associations of ulnar nerve compound muscle action potential amplitudes (CMAP) with American Spinal Cord Injury Association (ASIA) impairment scale (AIS) grades over time were analyzed using linear mixed modeling. Applying logistic regression, the prognostic value of within 4-week ulnar nerve CMAP for 1-year AIS was analyzed. To account for missing data, (1) last observation carried forward and (2) multiple imputation methods were applied. For model derivation, our centers’ cohort (EMSCI-HD) was analyzed. For model validation the cohort of other centers (EMSCI-nonHD) was used. Results In the EMSCI-HD cohort, the median age (interquartile range (IQR)) was 52 (34–67) years. 58% were male. The initial AIS distribution was: A = 31%, B = 17%, C = 30%, and D = 22%). In the EMSCI-nonHD cohort, the median age was 49 (32–65) years. Compared to the EMSCI-HD cohort more patients were male (79%, p = 0.0034). The AIS distribution was: A = 33%, B = 13%, C = 21%, and D = 33%). In complete-case mixed model analyses (EMSCI-HD: n = 114; EMSCI-nonHD: n = 508) higher ulnar nerve CMAP were associated with better AIS grades over the entire follow up period. In complete-case logistic regression (EMSCI-HD: n = 90; EMSCI-nonHD: n = 444) higher ulnar nerve CMAP was an independent predictor of better AIS grades. The odds ratio for within 4-week ulnar nerve CMAP to predict 1-year AIS grade D versus A-C in the EMSCI-HD cohort was 1.24 per millivolt (confidence interval 1.07–1.44). The model was validated in an independent cervical spinal cord injury (EMSCI-nonHD) cohort (odds ratio 1.09, confidence interval 1.03–1.17). Conclusions In cervical spinal cord injury, the consideration of early ulnar nerve CMAP improves prognostic accuracy, which is of particular importance in patients with clinical grading uncertainties.
DOI : 10.1186/s42466-019-0017-1
Anahtar Kelimeler : Spinal cord injury, Electroneurography, Ulnar nerve, Prognosis, Outcome
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 8
Sınıflandırma :
18
HDMTX-based induction therapy followed by consolidation with conventional systemic chemotherapy and intraventricular therapy (modified Bonn protocol) in primary CNS lymphoma: a monocentric retrospective analysis
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Sabine Seidel , Agnieszka Korfel , Thomas Kowalski , Michelle Margold , Fatme Ismail , Roland Schroers , Alexander Baraniskin , Hendrik Pels , Peter Martus , Uwe Schlegel
Yıl :
2019
Neurological Research and Practice
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Abstract Background To evaluate outcome and toxicity of High-dose methotrexate (HDMTX)-based induction therapy followed by consolidation with conventional systemic chemotherapy and facultative intraventricular therapy (modified Bonn protocol) in patients with primary CNS lymphoma (PCNSL). Methods Between 01/2005 and 12/2013 113 patients with newly diagnosed PCNSL presented at our center; 98 of those qualified for HDMTX based chemotherapy, received a modified Bonn protocol and were included in the analysis. The treatment regimen was based on the “Bonn protocol”, but modified by omission of systemic drugs not able to cross the intact blood brain barrier. Intraventricular therapy was postponed until completion of three induction chemotherapy cycles or was replaced by intrathecal liposomal AraC and rituximab was added to induction from 2010 onwards. Results Median patient age was 67 years (range 38–83). Complete response/complete response unconfirmed (CR/CRu) was achieved in 59/98 patients (60%), partial response (PR) in 9/98 patients (9%). Twenty-four patients (23%) had progressive disease (PD), 6 (6%) died on therapy. Median progression-free survival (PFS) for all patients was 11.4 months, median overall survival (OS) 29.1 months. A trend to better outcome for intraventricular therapy versus intrathecal liposomal AraC was found in patients < 65 years (HR 0.53 [0.19–1.47] for OS and 0.46 [0.21–1.02] for PFS. Ommaya reservoir infection occurred in 3/33 patients (9%). Conclusions The data of this single center experience suggest that the outcome with a modified Bonn protocol was comparable to that of the previous regimen, showed fewer Ommaya reservoir infections and may have a trend for better outcome with intraventricular therapy.
DOI : 10.1186/s42466-019-0024-2
Anahtar Kelimeler : PCNSL, Methotrexate, Consolidation chemotherapy, Intraventricular therapy, Bonn protocol
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 9
19
Inter-rater and test-retest reliability of the “standardized endoscopic swallowing evaluation for tracheostomy decannulation in critically ill neurologic patients”
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Tobias Warnecke , Paul Muhle , Inga Claus , Jens B. Schröder , Bendix Labeit , Sriramya Lapa , Sonja Suntrup-Krueger , Rainer Dziewas
Yıl :
2020
Neurological Research and Practice
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Abstract Background Removal of a tracheostomy tube in critically ill neurologic patients is a difficult issue, particularly due to the high incidence of oropharyngeal dysphagia. For an objective evaluation of decannulation readiness the “Standardized Endoscopic Swallowing Evaluation for Tracheostomy Decannulation in Critically Ill Neurologic Patients” (SESETD) – a stepwise evaluation of ‘secretion management’, ‘spontaneous swallows’ and ‘laryngeal sensibility/cough’ – has been introduced. With the recent study detailed data on inter-rater and test-retest reliability are presented. Methods To obtain inter-rater reliability levels both in a group of raters with at least 5 years of experience (‘experts’) and in a group of raters with no or only minor experience using the SESETD (‘non-experts’), for each single item of the protocol and the sum score α-, respectively κ-levels were determined. The ‘experts’ assessed the same videos after a four-week interval to determine test-retest reliability. Ten videos from tracheostomized neurological patients completely weaned from mechanical ventilation were assessed independently by six ‘experts’. 27 ‘non-experts’ applied the SESETD on 5 videos from the same patient population after introduction to the protocol in a one-hour workshop. Results For the items ‘secretion management’ and ‘spontaneous swallows’ α-levels were identified at > 0.800 both in the group of ‘experts’ and ‘non-experts’. With regard to the item ‘laryngeal sensibility/cough’ in both groups, the α-level was ≥0.667. With κ-levels of 1.0 for ‘secretion management’, 0.93 for ‘spontaneous swallows’ and 0.76 for ‘laryngeal sensibility/cough’ test-retest reliability showed at least substantial agreement for each item. Intraclass correlation coefficient for the sum score was excellent in both groups (α ≥ 0.90). Conclusions The SESETD demonstrates good to excellent agreement for each single item included as well as the sum score in experienced and unexperienced raters supporting its usefulness for implementation in daily clinical routine and as an outcome measure for clinical trials.
DOI : 10.1186/s42466-020-00055-3
Anahtar Kelimeler : Tracheostomy, Decannulation, Intensive care, Swallowing, Dysphagia
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 7
Sınıflandırma :
20
Neuro-Sweet syndrome - a rare differential diagnosis in aseptic meningoencephalitis
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Elgin Hoffmann , Christian Boßelmann , Stephan Forchhammer , Holger Lerche , Tobias Freilinger
Yıl :
2019
Neurological Research and Practice
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Abstract Acute febrile neutrophilic dermatosis (Sweet‘s syndrome) is a dermatological entity, which may be associated with malignancies, drugs, and infections and which is characterized by high fever, elevated neutrophils, and tender erythematous skin lesions. Involvement of the nervous system – Neuro-Sweet syndrome (NSS) - is rare, manifesting most commonly with an encephalitic syndrome in addition to fever and dermal lesions. Here, we report an unusual case of NSS in a Caucasian male patient in the setting of B-cell-lymphocytosis, with encephalitis preceding dermal lesions. Symptoms resolved completely in response to corticoids. NSS is a rare, but important differential diagnosis in the work-up of febrile aseptic meningoencephalitis unresponsive to anti-infectious treatment. Due to its rarity and clinical variability, diagnosis of NSS might be challenging. Knowledge of this entity may facilitate proper diagnosis and differentiation from conditions with similar clinical presentation, especially Neuro-Behçet‘s disease. It may further lead to early detection of a potentially underlying malignancy and help in initiating adequate therapy.
DOI : 10.1186/s42466-019-0041-1
Anahtar Kelimeler : Differential diagnosis to meningoencephalitis, Neuro-sweet syndrome, Neuro-Behçet disease, Paraneoplastic syndromes
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 3
Sınıflandırma :
21
GCS 15: when mild TBI isn’t so mild
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Latha Ganti , Tej Stead , Yasamin Daneshvar , Aakash N. Bodhit , Christa Pulvino , Sarah W. Ayala , Keith R. Peters
Yıl :
2019
Neurological Research and Practice
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Abstract Objective The present study characterizes patients with the mildest of mild traumatic brain injury (TBI), as defined by a Glasgow coma score (GCS) of 15. Methods This is an IRB approved observational cohort study of adult patients who presented to the emergency department of a Level-1 trauma center, with the primary diagnosis of TBI and a GCS score of 15 on arrival. Data collected included demographic variables such as age, gender, race, mechanisms of injury, signs and symptoms including associated vomiting, seizures, loss of consciousness (LOC), alteration of consciousness (AOC), and post-traumatic amnesia (PTA).Pre- hospital GCS, Emergency Department (ED) GCS, and results of brain CT scans were also collected as well as patient centered outcomes including hospital or intensive care unit (ICU) admission, neurosurgical intervention, and in hospital death. Data were stored in REDCap (Research Electronic Data Capture), a secure, web- based application. Descriptive and inferential analysis was done using JMP 14.0 for the Mac. Results Univariate predictors of hospital admission included LOC, AOC, and PTA, all p < 0.0001. Patients admitted to ICU were significantly more likely to be on an antiplatelet or anticoagulant (P < 0.0001), have experienced PTA (p = 0.0025), LOC (p < 0.0001), or have an abnormal brain CT (p < 0.0001). Patients who died in the hospital were significantly more likely to be on an antiplatelet or anticoagulant (P = 0.0005. All who died in the hospital had intracranial hemorrhage on ED head CT, despite having presented to the ED with GCS of 15. Patients were also significantly more likely to have had vomiting (p < 0.0001). Patients who underwent neurosurgical intervention were significantly more likely to be male (P = 0.0203), to be on an antiplatelet or anticoagulant (P = < 0.0001) likely to have suffered their TBI from a fall (P = 0.0349), and experienced vomiting afterwards (P = 0.0193). Conclusions This study underscores: 1) the importance of neuroimaging in all patients with TBI, including those with a GCS 15. Fully 10% of our cohort was not imaged. Extrapolating, these would represent 2.5% bleeds, and 1.47% fractures. 2) The limitations of GCS in classifying TBI, as patients with even the mildest of mild TBI have a high frequency of gross CT abnormalities.
DOI : 10.1186/s42466-018-0001-1
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 8
22
Computer-aided imaging analysis in acute ischemic stroke – background and clinical applications
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Yahia Mokli , Johannes Pfaff , Daniel Pinto dos Santos , Christian Herweh , Simon Nagel
Yıl :
2019
Neurological Research and Practice
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Kaydet Alıntıla
Abstract Tools for medical image analysis have been developed to reduce the time needed to detect abnormalities and to provide more accurate results. Particularly, tools based on artificial intelligence and machine learning techniques have led to significant improvements in medical imaging interpretation in the last decade. Automatic evaluation of acute ischemic stroke in medical imaging is one of the fields that witnessed a major development. Commercially available products so far aim to identify (and quantify) the ischemic core, the ischemic penumbra, the site of arterial occlusion and the collateral flow but they are not (yet) intended as standalone diagnostic tools. Their use can be complementary; they are intended to support physicians’ interpretation of medical images and hence standardise selection of patients for acute treatment. This review provides an introduction into the field of computer-aided diagnosis and focuses on the automatic analysis of non-contrast-enhanced computed tomography, computed tomography angiography and perfusion imaging. Future studies are necessary that allow the evaluation and comparison of different imaging strategies and post-processing algorithms during the diagnosis process in patients with suspected acute ischemic stroke; which may further facilitate the standardisation of treatment and stroke management.
DOI : 10.1186/s42466-019-0028-y
Anahtar Kelimeler : Acute ischemic stroke, Imaging, Computer aided diagnosis, Artificial intelligence
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 13
Sınıflandırma :
23
New alternative splicing variants of the ATXN2 transcript
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Isabel Lastres-Becker , David Nonis , Joachim Nowock , Georg Auburger
Yıl :
2019
Neurological Research and Practice
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Kaydet Alıntıla
Abstract Background Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant disorder with progressive degeneration of cerebellar Purkinje cells and selective loss of neurons in the brainstem. This neurodegenerative disorder is caused by the expansion of a polyglutamine domain in ataxin-2. Ataxin-2 is composed of 1312 amino acids, has a predicted molecular weight of 150-kDa and is widely expressed in neuronal and non-neuronal tissues. To date, the putative functions of ataxin-2 on mRNA translation and endocytosis remain ill-defined. Differential splicing with a lack of exons 10 and 21 was described in humans, and additional splicing of exon 11 in mice. In this study, we observed that the molecular size of transfected full-length wild-type ataxin-2 (22 glutamines) is different from endogenous ataxin-2 and that this variation could not be explained by the previously published splice variants alone. Methods Quantitative immunoblots and qualitative reverse-transcriptase polymerase-chain-reaction (RT-PCR) were used to characterize isoform variants, before sequencing was employed for validation. Results We report the characterization of further splice variants of ataxin-2 in different human cell lines and in mouse and human brain. Using RT-PCR from cell lines HeLa, HEK293 and COS-7 throughout the open reading frame of ataxin-2 together with PCR-sequencing, we found novel splice variants lacking exon 12 and exon 24. These findings were corroborated in murine and human brain. The splice variants were also found in human skin fibroblasts from SCA2 patients and controls, indicating that the polyglutamine expansion does not abolish the splicing. Conclusions Given that Ataxin-2 interacts with crucial splice modulators such as TDP-43 and modulates the risk of Amyotrophic Lateral Sclerosis, its own splice isoforms may become relevant in brain tissue to monitor the RNA processing during disease progression and neuroprotective therapy.
DOI : 10.1186/s42466-019-0025-1
Anahtar Kelimeler : ATXN2, Alternative splicing, Spinocerebellar Ataxia type 2, PolyQ expansion
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 9
Sınıflandırma :
24
Letter to the editor: risk comorbidities of COVID-19 in Parkinson’s disease patients in Germany
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Daniel Richter , Dirk Bartig , Christos Krogias , Lars Tönges
Yıl :
2020
Neurological Research and Practice
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Kaydet Alıntıla
DOI : 10.1186/s42466-020-00069-x
Anahtar Kelimeler : COVID-19, Parkinsonu2019s disease, Infection risk
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 3
Sınıflandırma :
25
Remarkable improvement of primary orthostatic tremor using perampanel
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Marcus Grobe-Einsler , Oliver Kaut
Yıl :
2020
Neurological Research and Practice
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Abstract Management of primary orthostatic tremor (POT) remains challenging, and medication is often ineffective. We report the case of a 53-year-old female with orthostatic tremor for 6 years who was refractory to gabapentin, clonazepam, primidone and propranolol. After treatment with 4 mg/day perampanel, she reported almost complete resolution of tremor. The diagnosis of POT was confirmed by tremor analysis using surface electromyography. Our report shows the potential use of the novel AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor antagonist perampanel for the treatment of POT. To date, only two similar patients, one refractory to treatment and the other previously treated with clonazepam only, have been reported. We would like to note that our patient was refractory to all previous therapy and responded to a low dose of perampanel without side effects. The striking clinical improvement suggests a putative role of glutamate in the pathophysiology of orthostatic tremor.
DOI : 10.1186/s42466-020-0050-0
Anahtar Kelimeler : Orthostatic tremor, Tremor, Perampanel
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 3
Sınıflandırma :
26
Signatures of immune reprogramming in anti-CD52 therapy of MS: markers for risk stratification and treatment response
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Laura Bierhansl , Tobias Ruck , Steffen Pfeuffer , Catharina C. Gross , Heinz Wiendl , Sven G. Meuth
Yıl :
2019
Neurological Research and Practice
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Kaydet Alıntıla
Abstract Background Multiple sclerosis is one of the most prevalent neurological diseases in young adults affecting over 2 million people worldwide. Alemtuzumab is a highly effective therapy in relapsing remitting MS. Alemtuzumab is a monoclonal CD52 antibody that proved its efficacy against an active comparator (interferon [IFN]-β1a) in a phase II trial and two phase III trials regarding clinical and MRI outcomes. Nevertheless, the exact mode of action is still unknown. Alemtuzumab is commonly associated with secondary autoimmune disorders significantly affecting the risk-benefit ratio. Therefore, new biomarkers predicting treatment response and adverse events are urgently needed. This study aims to further elucidate the mechanism of action of the neuroprotective potential of alemtuzumab in relapsing-remitting multiple sclerosis (RRMS). Methods/Design This is a 3-year multicentre, explorative study including overall 150 patients comprising three different groups: (i) de novo patients prior and after alemtuzumab treatment initiation, (ii) patients under alemtuzumab treatment and (iii) patients requiring more than two alemtuzumab infusions. Peripheral blood and serum samples will be collected semi-annually for several in vitro/ex vivo assays to detect and characterize immune cells including their functional activity. Furthermore, data of MRI scans and disease-related impairment (using EDSS and MSFC), as well as the number and time of relapses, will be assessed. The clinical study is registered at clinicaltrials.gov (NCT04082260). Perspective Our study will provide deep insights into the underlying immunological changes in a longitudinal analysis of alemtuzumab treated RRMS patients. By combining clinical, radiological and functional immune-phenotype data, we will be able to identify biomarkers and/or immune signatures predicting treatment response and adverse events. Thereby, the understanding of the mechanisms of action of alemtuzumab will improve its efficacy and safety for present and future patients.
DOI : 10.1186/s42466-019-0045-x
Anahtar Kelimeler : Relapse-remitting multiple sclerosis, Alemtuzumab, Biomarker, mechanism of action, secondary autoimmune disease, Disease-modifying therapy, risk stratification
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 9
Sınıflandırma :
27
The German trial on Aciclovir and Corticosteroids in Herpes-simplex-virus-Encephalitis (GACHE): a multicenter, randomized, double-blind, placebo-controlled trial
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Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
U. Meyding-Lamadé , C. Jacobi , F. Martinez-Torres , T. Lenhard , B. Kress , M. Kieser , C. Klose , K. Einhäupl , J. Bösel , M-B Mackert , V. Homberg , C. Koennecke , G. Weißheit , D. Claus , B. Kieseier , J. Bardutzky , T. Neumann-Haefelin , M. W. Lorenz , H. Steinmetz , C. Gerloff , D. Schneider , A. Grau , M. Klein , R. Dziewas , U. Bogdahn , W. Jakob , R. Linker , K. Fuchs , A. Sander , S. Luntz , T. Hoppe-Tichy , D. F. Hanley , R. von Kummer , E. Craemer
Yıl :
2019
Neurological Research and Practice
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Kaydet Alıntıla
Abstract Introduction Comprehensive treatment of Herpes-simplex-virus-encephalitis (HSVE) remains a major clinical challenge. The current therapy gold standard is aciclovir, a drug that inhibits viral replication. Despite antiviral treatment, mortality remains around 20% and a majority of survivors suffer from severe disability. Experimental research and recent retrospective clinical observations suggest a favourable therapy response to adjuvant dexamethasone. Currently there is no randomized clinical trial evidence, however, to support the routine use of adjuvant corticosteroid treatment in HSVE. Methods The German trial of Aciclovir and Corticosteroids in Herpes-simplex-virus-Encephalitis (GACHE) studied the effect of adjuvant dexamethasone versus placebo on top of standard aciclovir treatment in adult patients aged 18 up to 85 years with proven HSVE in German academic centers of Neurology in a randomized and double blind fashion. The trial was open from November 2007 to December 2012. The initially planned sample size was 372 patients with the option to increase to up to 450 patients after the second interim analysis. The primary endpoint was a binary functional outcome after 6 months assessed using the modified Rankin scale (mRS 0–2 vs. 3–6). Secondary endpoints included mortality after 6 and 12 months, functional outcome after 6 months measured with the Glasgow outcome scale (GOS), functional outcome after 12 months measured with mRS and GOS, quality of life as measured with the EuroQol 5D instrument after 6 and 12 months, neuropsychological testing after 6 months, cranial magnetic resonance imaging findings after 6 months, seizures up to day of discharge or at the latest at day 30, and after 6 and 12 months. Results The trial was stopped prematurely for slow recruitment after 41 patients had been randomized, 21 of them treated with dexamethasone and 20 with placebo. No difference was observed in the primary endpoint. In the full analysis set (n = 19 in each group), 12 patients in each treatment arm achieved a mRS of 0–2. Similarly, we did not observe significant differences in the secondary endpoints (GOS, mRS, quality of life, neuropsychological testing). Conclusion GACHE being prematurely terminated demonstrated challenges encountered performing randomized, placebo-controlled trials in rare life threatening neurological diseases. Based upon our trial results the use of adjuvant steroids in addition to antiviral treatment remains experimental and is at the decision of the individual treating physician. Unfortunately, the small number of study participants does not allow firm conclusions. Trial registration EudraCT-Nr. 2005–003201-81.
DOI : 10.1186/s42466-019-0031-3
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 9
Sınıflandırma :
28
Vojta therapy improves postural control in very early stroke rehabilitation: a randomised controlled pilot trial
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Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Corina Epple , Barbara Maurer-Burkhard , Mari-Carmen Lichti , Thorsten Steiner
Yıl :
2020
Neurological Research and Practice
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 Piri Tam Metin Bulucu
Kaydet Alıntıla
Abstract Background It is still unclear, which physiotherapeutic approaches are most effective in stroke recovery. Vojta therapy is a type of physiotherapy that was originally developed for children and adolescents with cerebral palsy. Vojta therapy has been reported to improve automatic control of body posture. Because acute stroke patients are characterised by a disturbance in the ability to adapt to changes in body position, requiring automatic postural adjustment, we decided to investigate Vojta therapy in the early rehabilitation of stroke patients. Aim of the trial was to test the hypothesis that Vojta therapy - as a new physiotherapeutic approach in early stroke recovery - improves postural control and motor function in patients with acute ischaemic stroke (AIS) or intracerebral haemorrhage (ICH). Methods This prospective, randomised controlled trial included patients with imaging-confirmed AIS or ICH, severe hemiparesis and randomly assigned them to Vojta therapy or standard physiotherapy within 72 h after stroke onset. Main exclusion criterion was restricted ability to communicate. Primary endpoint was the improvement of postural control measured by the Trunk Control Test (TCT) on day 9 after admission. Secondary endpoint among others was improvement of arm function (measured with Motor Evaluation Scale for Upper Extremity in Stroke Patients [MESUPES]). Results Forty patients (20 per group) were randomised into the trial. Median age was 75 (66–80) years, 50% were women. The median improvement in TCT within 9 days was 25.5 points (=25.5%) (interquartile range [IQR] 12.5–42.5) in the Vojta group and 0 (IQR 0–13) in the control group (p = 0.001). Patients treated with Vojta therapy achieved a greater improvement in the MESUPES than patients in the control group (20% vs 10%, p = 0.006). Conclusion This first randomised controlled trial of Vojta therapy in acute stroke patients demonstrates improvement of postural control through Vojta therapy compared to standard physiotherapy. Although this trial has some methodical weaknesses, Vojta therapy might be a promising approach in early stroke rehabilitation and should be studied in larger trials. Trial registration ClinicalTrials.gov; Unique identifier: NCT03035968 . Registered 30 January 2017 – Retrospectively registered; http://www.clinicaltrials.gov .
DOI : 10.1186/s42466-020-00070-4
Anahtar Kelimeler : Vojta therapy, Stroke recovery, Postural control, Rehabilitation, Acute stroke, Physiotherapy
ISSN: 2524-3489
Cilt: 2
Sayı: 1
Sayfa: 1 - 11
Sınıflandırma :
29
Safety and clinical impact of FEES – results of the FEES-registry
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Rainer Dziewas , Matthias auf dem Brinke , Ulrich Birkmann , Götz Bräuer , Kolja Busch , Franziska Cerra , Renate Damm-Lunau , Juliane Dunkel , Amelie Fellgiebel , Elisabeth Garms , Jörg Glahn , Sandra Hagen , Sophie Held , Christine Helfer , Mirko Hiller , Christina Horn-Schenk , Christoph Kley , Nikolaus Lange , Sriramya Lapa , Christian Ledl , Beate Lindner-Pfleghar , Marion Mertl-Rötzer , Madeleine Müller , Hermann Neugebauer , Duygu Özsucu , Michael Ohms , Markus Perniß , Waltraud Pfeilschifter , Tanja Plass , Christian Roth , Robin Roukens , Tobias Schmidt-Wilcke , Beate Schumann , Julia Schwarze , Kathi Schweikert , Holger Stege , Dirk Theuerkauf , Randall S. Thomas , Ulrich Vahle , Nancy Voigt , Hermann Weber , Cornelius J. Werner , Rainer Wirth , Ingo Wittich , Hartwig Woldag , Tobias Warnecke
Yıl :
2019
Neurological Research and Practice
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
Abstract Background At present, the flexible endoscopic evaluation of swallowing (FEES) is one of the most commonly used methods for the objective assessment of swallowing. This multicenter trial prospectively collected data on the safety of FEES and also assessed the impact of this procedure on clinical dysphagia management. Methods Patients were recruited in 23 hospitals in Germany and Switzerland from September 2014 to May 2017. Patient characteristics, professional affiliation of the FEES examiners (physicians or speech and language therapists), side-effects and cardiorespiratory parameters, severity of dysphagia and clinical consequences of FEES were documented. Results 2401 patients, mean age 69.8 (14.6) years, 42.3% women, were included in the FEES-registry. The most common main diagnosis was stroke (61%), followed by Parkinson’s disease (6.5%). FEES was well tolerated by patients. Complications were reported in 2% of examinations, were all self-limited and resolved without sequelae and showed no correlation to the endoscopist’s previous experience. In more than 50% of investigations FEES led to changes of feeding strategies, in the majority of cases an upgrade of oral diet was possible. Discussion This study confirmed that FEES, even when performed by less experienced clinicians is a safe and well tolerated procedure and significantly impacts on the patients’ clinical course. Implementation of a FEES-service in different clinical settings may improve dysphagia care. Trial registration ClinicalTrials.gov NCT03037762, registered January 31st 2017.
DOI : 10.1186/s42466-019-0021-5
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 8
Sınıflandırma :
30
SOP myasthenic crisis
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Neurological Research and Practice
Yazar(lar) :
Henning Stetefeld , Michael Schroeter
Yıl :
2019
Neurological Research and Practice
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
Abstract Introduction The overall prevalence of myasthenic crisis is quite low at 30/1 million inhabitants because myasthenia gravis is a rare disease per se. But it should be noted that 15–20% of patients with myasthenia gravis experience at least one crisis in their lives. Most often, the crisis occurs within the first 2 years of the disease or is even the first manifestation of a yet undiagnosed myasthenia gravis in up to 20%. Median duration of MC is about 2 weeks (median 12–14 days of ventilation) under sufficient treatment, but prolonged courses are not uncommon and often due to comorbidities and complications, so that about 20% are still mechanically ventilated after 1 month. The lifetime risk of recurrence of a crisis is approx. 30%. Data on mortality differ between about 2–5% to even more than 16%. Lethal outcomes are almost never caused by the crisis itself, but because comorbidities or complications eventually become limiting. Definition Myasthenic crisis (MC) is the life-threatening maximal manifestation of myasthenia gravis (MG) necessitating mechanical ventilation, supportive feeding and (neuro-)intensive care. Weakness may develop within minutes to days and encompass flaccid tetraparesis with immobility, severe dyspnea, respiratory insufficiency and aspiration. Globus events may be life threatening due to rapidly exhausting coughing and swallowing. First steps: immediate measures ● Check and secure vital functions Comments ● not applicable Conclusion The main symptom of (imminent) myasthenic crisis is the rapidly progressive weakness of the respiratory and bulbar muscles, which lead to a decompensation with aspiration and respiratory insufficiency. Clinical examination and clinical history should lead early to the diagnosis of MG with (impending) crisis. The detection of red flags and the dynamic deterioration of symptoms entail admission to the intensive care unit. Due to bulbar symptoms with aspiration and/or respiratory insufficency, early intubation to secure the airway is essential. Therapy includes symptomatic treatment with pyridostigmine or neostigmine and acute causal treatment by immunoadsorption/plasmapheresis or alternatively with immunoglobulins. If used early, intubation may still be prevented and clinical improvement can be achieved within a few days. At the same time, immunosuppression with corticosteroids and azathioprine should be initiated or optimized. For escalation rituximab is an option. The early diagnosis and consequent treatment of infections and other complications such as delirium influence the further course.
DOI : 10.1186/s42466-019-0023-3
ISSN: 2524-3489
Cilt: 1
Sayı: 1
Sayfa: 1 - 6
Sınıflandırma :
Biyotıp %100
Hematoloji %91

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