Neurol. praxi. 2018;19(2):79
Neurol. praxi. 2018;19(2):86 | DOI: 10.36290/neu.2018.082
Neurol. praxi. 2018;19(2):88-91 | DOI: 10.36290/neu.2018.083
Surgical treatment of epilepsy represents an important therapeutic option in patients with drug-resistant epilepsy. At Departmentof Paediatric Neurology, Motol Epilepsy Center, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospitalpaediatric epilepsy surgery has been practiced since 2000. Up to 1st November 2017 263 surgeries in 232 patients have beenperformed. This article aims to summarize essential principles both epilepsy surgery and preoperative diagnostic work-up as wellas results of our paediatric epilepsy surgery programme including recent trends. 80,7 % children in our series are seizure-free at 2year follow-up....
Neurol. praxi. 2018;19(2):92-95 | DOI: 10.36290/neu.2018.146
Current progress in genetic technologies increases the detection rate of genetic causes of epilepsies, especially of early infantileonset epilepsies associated with comorbidities. Cooperation of neurologists with clinical geneticists and molecular biologist isessential for correct interpretation of the results.
Neurol. praxi. 2018;19(2):96-99 | DOI: 10.36290/neu.2018.084
Autoimmune disorders affecting central nervous system are potentially treatable. They are rare in childhood and a clinical courseis often serious. The classification and diagnostic criteria are developing as well as therapeutic approaches. The aim is to determinea diagnosis of autoimmune condition and to initiate an accurate therapy as soon as possible. This review is focused on updatesof the most common clinical diagnoses and the terminology in this area.
Neurol. praxi. 2018;19(2):100-103 | DOI: 10.36290/neu.2018.085
The treatment of the inherited metabolic disorders with primary biochemical defects in the CNS has undergone a considerableprogress, recently. Our work brings a brief summary of currently available therapeutic possibilities including intrathecal applicationof enzyme replacement therapy, small molecules treatment, hematopoietic stem cells transplantation and the gene therapy. Theearly diagnosis and introduction to the therapy are crucial for the therapeutic success.
Neurol. praxi. 2018;19(2):104-107 | DOI: 10.36290/neu.2018.086
Neurocutaneous disorders is a group of congenital, genetically conditioned, multisystem diseases that primarily affect the skinand nervous system, and also other body systems. They arise from the abnormal neuroectodermal tissue in the wrong courseof neural criste development. All neurocutaneous disorders are among the rare diseases. They manifest from the neonatal ageto adulthood and have very variable clinical course, with a slow progression. An important feature is the frequent occurrence oftumor processes, in childhood primarily benign character, in adulthood the risk of malignant tumors increases. The most commonneurocutaneous disease is neurofibromatosis...
Neurol. praxi. 2018;19(2):108-113 | DOI: 10.36290/neu.2018.087
Neuromuscular disorders are disorders affecting peripheral nerves, neuromuscular junction, and muscles. Clinically, these disordersmost frequently cause progressive muscle weakness. By incidence, these disorders are rare. Nevertheless, they are verysevere; they cause high invalidity, and can lead to loss of ambulation, the necessity of ventilation support, and shortening of lifeexpectancy together with keeping normal cognition. In recent years, mostly due to the development of genetics, new knowledgeabout the pathogenesis has grow exponentially and new drugs have been developed. The new therapy based on modificationof gene transcription and translation...
Neurol. praxi. 2018;19(2):114-122 | DOI: 10.36290/neu.2019.031
Major depression has a relatively high incidence in patients with multiple sclerosis (MS). It needs to be recognized and treatedin a timely manner, as it significantly improves the quality of life and the course of the main disease. From an etiological point ofview, it is not yet clear why major depression in MS occurs so often. Biological, psychological and social factors are involved in itsdevelopment, but it is also influenced by MS treatment itself (corticoids, interferons). Major clinical signs of depression includedisturbances of mood, thinking, perception, and psychomotor impairment. In clinical practice we usually start with structuredinterview...
Neurol. praxi. 2018;19(2):130-134 | DOI: 10.36290/neu.2018.025
Postherpetic neuralgia is a neuropathic pain caused by the reactivation varicela zoster virus. Current international and nationalguidelines recommend pain treatment in a hierarchial manner, with antiepileptics, tricyclic antidepressants and topical lidocain.
Neurol. praxi. 2018;19(2):135-138
Encephalitis with antibodies to N-methyl-D-aspartate receptors (NMDAR) is currently the second most common autoimmuneencephalitis in adolescents and young adults (after ADEM – acute disseminated encephalomyelitis). Due to the rapid developmentof laboratory diagnostics and the discovery of the nature of this disease, namely antibodies to N-methyl-D-aspartate receptors,the diagnosis has been taken out of a subset of autoimmune encephalitis and has become a separate, independent unit (Dalmauet al., 2007). Because of the above, the exact incidence and prevalence of the disease is not yet known and an underdiagnosis ofthe patients is anticipated....
Neurol. praxi. 2018;19(2):139-141 | DOI: 10.36290/neu.2018.060
Anterior choroidal artery syndrome is a rare clinical syndrome consisting of a triad of contralateral severe hemiparesis/hemiplegia,hemiparesthesia and homonymous hemianopia, typically as a result of a cerebral infarction in the anterior choroidal artery territory.A 72-year-old woman was admitted for a left-sided hemiplegia, hemianesthesia, facial palsy, neglect syndrome/anosognosia andleft homonymous hemianopia. Control magnetic resonance has showed acute ischemic changes in the posterior limb of internalcapsule corresponding to the territory of the right anterior choroidal artery.
Neurol. praxi. 2018;19(2):123-129 | DOI: 10.36290/neu.2018.088
Progress in developing new monoclonal antibodies allows us to affect pathological immune processes on many different levels.For treating MS, we now have available monoclonal antibodies capable of influencing the lymphocytes migration or inducingtheir depletion. Also, the development of new humanized and human antibodies caused significant improvement of their safetyprofile. Currently there are four types of monoclonal antibodies available in Europe for treating MS: natalizumab, alemtuzumab,daclizumab and ocrelizumab. The last one has also shown effectivity on suppressing progression of primary progressive form of MS.
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