Aktualnosci Neurologiczne最新文献

{"title":"Stiff-person syndrome with sensorimotor polyneuropathy – case report","authors":"K. Jastrzębski, Nina Romanowska, Marta Rek-Pacześ, Łukasz Kępczyński, A. Plucińska, Monika Gałecka-Kowalska, Krzysztof Pacześ","doi":"10.15557/an.2021.0007","DOIUrl":"https://doi.org/10.15557/an.2021.0007","url":null,"abstract":"Stiff-person syndrome (SPS) is a rare disorder with an estimated prevalence in the general population of 1–2 cases/1,000,000. It is 2–3 times more common in females, with symptom onset at the age of 20–50 years in most cases. Although stiff-person syndrome is associated with antibodies against glutamic acid decarboxylase and amphiphysin, their presence is not necessary for the diagnosis. The treatment should be multidirectional and include immunomodulation, symptomatic treatment as well as monitoring and treatment of overlapping autoimmune, and surgery. We present a case report of a patient diagnosed with stiff-person syndrome overlapping with axonal and demyelinating sensorimotor polyneuropathy. The diagnostic workup indicated diabetes-related polyneuropathy. About 30% of patients diagnosed with stiff-person syndrome also have diabetes. Polyneuropathy alone is rarely reported to overlap with the disorder. In our opinion, polyneuropathy may have a beneficial effect on the clinical presentation of stiff-person syndrome.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":"72 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67583629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impulse control disorders in Parkinson’s disease","authors":"M. Toś","doi":"10.15557/an.2021.0004","DOIUrl":"https://doi.org/10.15557/an.2021.0004","url":null,"abstract":"Parkinson’s disease is a neurodegenerative disease characterised by typical motor symptoms and a range of non-motor symptoms, among which impulse control disorders, defined by an inability to resist temptations, impulses or urges, despite them being potentially harmful to the patient or caregivers, are gaining an increasing research interest. The most common compulsive activities include pathological gambling, hyper-sexuality, compulsive buying, and binge eating. The prevalence of impulse control disorders varies greatly depending on the country where the study was conducted, probably due to cultural and socioeconomic factors or the research methods used. Non-ergotamine dopamine agonists, and to a lesser extent highdose L-dopa and other antiparkinsonian drugs, are considered to be major risk factors for the development of impulse control disorders. Young age of patients, male gender, and early age of disease onset also increase the risk of developing this type of disorder. A probable cause of impulse control disorders is a state of dopaminergic overstimulation within the mesolimbic pathway and frontal-striatal circuit. The management of impulse control disorders is particularly challenging in view of the possible worsening of motor symptoms. The primary strategy remains dose reduction, discontinuation or switching from a dopamine agonist to another drug. If this type of intervention has failed, it is advisable to add atypical antipsychotics or antiepileptic drugs. Because of the low detection rate of impulse control disorders and their potentially devastating impact on patients’ personal and family lives, every clinician managing patients with Parkinson’s disease should be particularly vigilant for the presence of such disorders.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67583425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The multiple sclerosis patient’s journey in Poland – current diagnosis","authors":"M. Gałązka-Sobotka, J. Gierczyński, J. Gryglewicz, K. Rejdak, J. Sławek, A. Słowik, M. Adamczyk-Sowa, H. Bartosik-Psujek, M. Wieczorek, A. Kułakowska","doi":"10.15557/an.2021.0002","DOIUrl":"https://doi.org/10.15557/an.2021.0002","url":null,"abstract":"The multiple sclerosis patient’s journey in Poland was described based on the data collected by the Therapeutic Programme Monitoring System (System Monitorowania Programów Terapeutycznych, SMPT) between 2014 and 2018 and a National Health Fund (Narodowy Fundusz Zdrowia, NFZ) report entitled “NFZ on Health. Multiple Sclerosis.” It was found that about 90% of patients had their first multiple sclerosis diagnosis code (ICD-10: G35) assigned by a neurologist, usually during hospital stay in the department of neurology. Further patient management was usually continued in a neurology clinic. The waiting time for further outpatient and inpatient specialist (neurology) care after the first G35 diagnosis is more than 100 days. Before G35 diagnosis, patients most often presented with their first symptoms to neurological and ophthalmic clinics and were probably referred from these clinics for a magnetic resonance imaging. The analysis of the data from the Therapeutic Programme Monitoring System showed that the median time from the onset of the first symptoms to the diagnosis of multiple sclerosis was 7.4 months, and the median time from the diagnosis to initiation of first-line disease modifying treatment was 14.82 months. The length of time from the diagnosis to disease modifying therapy had a significant impact on the degree of disability. Patients treated with highly active second-line therapies accounted for 8.5% of all patients treated in drug programmes, and the increase in the number of patients on second-line regimens and expenditure on this programme were disproportionately lower compared to the first-line programme. A programme to improve the diagnosis and the quality of care for patients with multiple sclerosis should be developed in Poland to achieve better treatment outcomes. It is crucial to reduce the waiting time for diagnosis and the time interval between diagnosis and the onset of disease modifying treatment.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":"9 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67583672","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reasons for switching immunomodulatory therapy in patients with relapsing-remitting multiple sclerosis","authors":"M. Popiel, H. Bartosik-Psujek","doi":"10.15557/an.2021.0001","DOIUrl":"https://doi.org/10.15557/an.2021.0001","url":null,"abstract":"Aim: The aim of the study was to assess switching disease-modifying therapy in patients with relapsing-remitting multiple sclerosis. Materials and methods: This observational single-centre study involved a retrospective evaluation of data of patients with relapsing-remitting multiple sclerosis receiving disease-modifying therapy under drug programmes reimbursed by the National Health Fund. Demographic data, the level of disability, disease activity and treatment methods were analysed with a particular emphasis on the reasons for switching the therapy. Results: Data were collected from patients with a mean age of 39.6 years, with women accounting for 68%. Therapy was switched in 32% (n = 118) of patients. In this group, 72% (n = 85) of patients modified their first-line treatment, and 28% (n = 33) required switching to a second-line drug. Treatment failure (52.5%, n = 62), followed by treatment intolerance (47.5%, n = 56) were the main reasons for switching. Patients on first line-treatment mostly required switching from interferon beta-1a, which was usually replaced with dimethyl fumarate and glatiramer acetate. The most common adverse events included skin reactions and needle fatigue, which were the most common reasons for switching treatment due to intolerance. Conclusions: The currently used immunomodulatory treatment is relatively well tolerated, and its failure is a more frequent reason for therapy switching. This indicates the need for diseasemodifying treatments that are highly effective, safe and well tolerated.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47456924","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ischaemic stroke caused by spontaneous unilateral carotid artery dissection in patient with connective tissue disorder","authors":"Weronika Pużyńska, A. Mirończuk, K. Kapica-Topczewska, A. Łukasiewicz, A. Czarnowska, J. Kochanowicz, A. Kułakowska","doi":"10.15557/an.2021.0006","DOIUrl":"https://doi.org/10.15557/an.2021.0006","url":null,"abstract":"Background: Spontaneous cervical artery dissection is a major cause of ischaemic stroke in young patients. It is supposed that genetic variants associated with connective tissue disorders often lead to neurovascular complications, the management of which remains a challenge. Case presentation: A 30-year-old female was admitted to the Department of Neurology with an episode of visual disturbances, speech problems, and concomitant headache. Computed tomography angiography revealed left internal carotid artery dissection. Magnetic resonance of the brain showed cortical and subcortical acute ischaemic lesions in the left cerebral hemisphere. The patient was diagnosed with vascular Ehlers–Danlos syndrome based on the clinical course of the disease. Conservative treatment was administered with full neurological recovery. Conclusion: It is very important for clinicians to consider the coexistence of genetically determined connective tissue disorders in young patients who develop arterial dissections.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47777187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy of cognitive-behavioural psychotherapy in the treatment of chronic fatigue in patients with multiple sclerosis – a literature review","authors":"J. Wiśniowska, Kamilla Puławska","doi":"10.15557/an.2021.0005","DOIUrl":"https://doi.org/10.15557/an.2021.0005","url":null,"abstract":"Fatigue is one of the most common symptoms seen in patients with multiple sclerosis. Cognitive-behavioural psychotherapy can be a non-pharmacological approach for these patients. Van Kessel and Moss-Morris developed a cognitive-behavioural model to explain multiple sclerosis-related fatigue (2006). According to this model, inflammatory and demyelinating factors present in the central nervous system trigger fatigue, while cognitive interpretation, anxiety, or depressive symptoms and resting lifestyle are maintaining factors. Based on the cognitive-behavioural model of fatigue in multiple sclerosis, a protocol encompassing 8 treatment sessions was developed. For over 10 years, studies have been conducted to verify the effectiveness of cognitive-behavioural psychotherapy in the treatment of fatigue in patients with multiple sclerosis. The so far obtained results show that cognitive-behavioural psychotherapy has a moderate short-term effect on reducing fatigue, while the effect size in the long-term is small. The obtained results were undoubtedly influenced by several factors: the heterogeneity of the procedures used, the size of the research groups, and the large number of disease-related intermediary variables. Further research should be conducted to identify specific factors responsible for the effectiveness of cognitive-behavioural psychotherapy in the treatment of fatigue and to assess the long-term effects of therapy.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49097789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of neuropsychological examination in the diagnosis and treatment of adult patients with epilepsy","authors":"Magdalena Gugała-Iwaniuk, K. Sławińska","doi":"10.15557/an.2021.0003","DOIUrl":"https://doi.org/10.15557/an.2021.0003","url":null,"abstract":"Individuals with epilepsy may experience various difficulties in cognitive and emotional functioning. The neuropsychological examination plays a significant role in the diagnosis and management of patients with epilepsy. It should be conducted by psychologists with appropriate competencies and experience, preferably by specialists who have undergone dedicated training in clinical psychology and gained clinical experience with neurological patients. Conclusions from neuropsychological examination provide information about the influence of epilepsy on cognitive and behavioral functioning of the patient. Neuropsychological assessment enables delineating the neuropsychological profile, including the description of the type and level of cognitive deficits, as well as examining the emotional state and psychosocial functioning of patients. Neuropsychological evaluation plays an important part in qualifying patients with epilepsy for neurosurgical treatment, and helps in the process of monitoring the state of patients in terms of postsurgical outcomes. In this paper, the authors present the aims of neuropsychological assessment in patients with epilepsy. Various factors are listed that can affect the profile of cognitive and emotional functioning of patients during the examination. On the basis of guidelines formulated by experts of the International League Against Epilepsy, the indications for neuropsychological examination are described, and the core cognitive and emotional dimensions that should be evaluated are presented. A list of psychometric tests and clinicalexperimental tasks addressing cognitive functions as well as a selection of tools assessing the emotional state that are available and used in Poland are also provided.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47134456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of aerobic training on changes in neuroplasticity marker levels in patients with multiple sclerosis – a pilot study","authors":"Marta Milewska-Jędrzejczak, Dominika Ksiazek-Winiarek, Piotr Szpakowski, I. Bednarski, E. Miller, A. Głąbiński","doi":"10.15557/an.2020.0021","DOIUrl":"https://doi.org/10.15557/an.2020.0021","url":null,"abstract":"Introduction: Neuroplasticity is the function of the central nervous system that allows structural and functional reorganisation in response to external and internal factors. It is a crucial aspect that allows for the restoration of motor functions in people with limited brain damage. Patients with multiple sclerosis may obtain a special benefit from activities that support brain neuroplasticity. In order to stimulate neuroplasticity, several attempts were made to identify neuroplasticity-influencing factors. Aim: The objective of the study was to measure the effect of aerobic training on plasma neurotrophin levels that are potentially responsible for the brain plasticity phenomenon in patients with multiple sclerosis. Materials and methods: The study was conducted on a group of 22 patients with multiple sclerosis remaining in the drug programs of the National Health Fund. The study group underwent a 4-week rehabilitation scheme that consisted of training on a horizontal ergometer twice a day for 15 minutes 6 days a week, whereas the control group did not participate in any form of training. The plasma levels of neurotrophins (BDNF, PDGF, NGF, GDNF) and fractalkine (chemokine CX3CL1) were tested in all patients, the patients were assessed in the motor disability scale, the cognitive function screening and Beck depression inventory at the beginning of the study and after 4 weeks of rehabilitation. Results: The study showed no statistically significant difference in the levels of neurotrophins assessed before and after 4 weeks of aerobic training. A significant improvement in the clinical condition evaluated with the Expanded Disability Status Scale (EDSS) and Mini-Mental State Examination (MMSE) was observed in patients from the study group. Conclusion: Further studies on larger groups and with more homogenous participants are necessary to gain more insights into this aspect.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67583321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Executive dysfunction profile in children and adolescents with fetal alcohol spectrum disorder – systematic review","authors":"Justyna Urbańska-Grosz, S. Konieczna, Maciej Walkiewicz, E. Sitek","doi":"10.15557/an.2020.0019","DOIUrl":"https://doi.org/10.15557/an.2020.0019","url":null,"abstract":"The paper aims at reviewing data on the executive functioning in children and adolescents with fetal alcohol spectrum disorder (FASD) in the context of Suchy’s model of executive functioning. Suchy hypothesized that individuals with FASD would present with features of dysexecutive, disinhibited, apathetic and inappropriate, but not disorganized, syndromes. Eight papers satisfied the inclusion criteria for the review. In most respects, the performance of children with FASD was found to be lower than in the control group. According to Suchy’s model of executive functioning, children with FASD demonstrate features of dysexecutive syndrome (with deficits of executive cognitive functions) and of apathetic (deficit in initiation and maintenance) and disinhibited syndromes (deficit in response selection) as well as impaired social cognition that could correspond to socially inappropriate syndromes. None of the reviewed studies included measures of multi-tasking that would address features of disorganized syndrome. The results suggest a mixed pattern of executive deficits in FASD. Difficulties in delineating the executive functioning profile in children and adolescents with FASD may be related to heterogeneous patient populations (different clinical criteria, variable overall intellectual functioning and different living conditions) and also the complex nature of most of the executive measures that rarely tap one aspect of the executive functioning and typically engage a variety of cognitive processes.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47657255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of fatigue, cognition and depression in correlation with the applied immunomodulating treatment based on the Lodz registry of patients with multiple sclerosis","authors":"Sylwia Janiak, I. Bednarski, A. Głąbiński","doi":"10.15557/an.2020.0020","DOIUrl":"https://doi.org/10.15557/an.2020.0020","url":null,"abstract":"Introduction: Multiple sclerosis is the most common chronic inflammatory demyelinating disease of the central nervous system, mainly affecting young people. A breakthrough in the treatment of multiple sclerosis was the introduction of immunomodulating drugs that affect the natural course of the disease as well as improve the quality of life of patients. Aim of the study: To establish a possible association between the level of cognitive disorders, fatigue, depression and the used immunomodulating treatment (disease-modifying treatment) in patients with the relapsing-remitting form of multiple sclerosis. Materials and methods: The research material consisted of 169 patients with multiple sclerosis (diagnosed according to the 2010 McDonald criteria) with the relapsing-remitting form of multiple sclerosis. The subjects were divided into two groups: the test group – receiving disease-modifying treatment (n = 132), undergoing immunomodulatory therapy, and the control group – untreated multiple sclerosis (n = 37), patients who did not receive immunomodulating therapy. Methods: multiple sclerosis patient registry – RejSM questionnaire; Beck Depression Inventory II (BD-II); postural function assessment – MMSE (Mini-Mental State Examination); FACIT quality of life (Functional Assessment of Chronic Illness Therapy – Fatigue; FACIT Fatigue Scale, FFS). Patients from both groups underwent tests (BD-II, MMSE, FFS) at two time points: at baseline (I) and after 12 months of immunomodulating treatment (II). Results: The post-hoc test (Tukey’s) showed a decrease in Beck Depression Inventory and FACIT-F scores which was statistically significant only in the disease-modifying treatment group. The results indicate that the use of interferon beta causes a significant reduction in depression (p < 0.0001) and fatigue (p < 0.0001) between the measured time points. The FACIT scores were significantly different between the time points for those treated with glatiramer acetate (p < 0.0001). In the case of MMSE evaluation, the patients receiving disease-modifying treatment did not differ significantly between the beginning and end of the study (p = 0.1210). Conclusions: The results of our research indicate that disease-modifying treatment used in patients with the relapsing-remitting form of multiple sclerosis has a beneficial effect in terms of improving their quality of life and reducing the symptoms of depression.","PeriodicalId":41161,"journal":{"name":"Aktualnosci Neurologiczne","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47471260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}