Slovo úvodem

prof. MUDr. Ivan Rektor, CSc., FCMA, FANA

Neurol. praxi. 2017;18(5):295  

Myastenie gravis

MUDr. Stanislav Voháňka, CSc., MBA – editor hlavního tématu

Neurol. praxi. 2017;18(5):299 | DOI: 10.36290/neu.2017.024  

Scales and questionnaires used in myasthenia gravis

MUDr. Magda Horáková, MUDr. Stanislav Voháňka, CSc., MBA

Neurol. praxi. 2017;18(5):301-304 | DOI: 10.36290/neu.2017.035  

Although a routine scoring of patients is not very popular, it can not be overlooked because it provides us a natural course of thedisease, efficacy of treatment and comparison of patients. There are specific attributes of myasthenia outcome measures due toa fluctuating nature of myasthenia. The aim of this article is a review of commonly used myasthenia scales with an emphasis onpractical implementation.

Myasthenia gravis and anesthesia

MUDr. Stanislav Voháňka, CSc., MBA, MUDr. Magda Horáková

Neurol. praxi. 2017;18(5):306-308 | DOI: 10.36290/neu.2017.102  

Myasthenia gravis is an autoimmune disorder of neuromuscular transmission. There are many drugs frequently used in anesthesiologywhich interfere significantly with this disease. The most important are peripheral blocking agents and potent inhaledanesthetic agents. Patients suffering from myasthenia reveal modified sensitivity to these drugs, esp. to the peripheral blockingagents. Regional methods of the anesthesia or combination of both are preferred. Postoperative management is based on thecareful monitoration of patients until full recovery is reached. In patient with myasthenia gravis there is no risk of malignanthyperthermia.

Challenges of ciclosporin and azathioprin dosage for myasthenia gravis

MUDr. Magda Horáková, MUDr. Stanislav Voháňka, CSc., MBA

Neurol. praxi. 2017;18(5):309-313 | DOI: 10.36290/neu.2017.103  

Azathioprin (Imuran) and ciclosporin (Sandimmun Neoral, Equoral) are among the most widely used immunosuppressive drugsfor myasthenia gravis. They are very efficient and help to improve patient’s quality of life. However, such therapy has some risks. Atleast part of the risks can be avoided by managing the right dose, which requires certain experience. The aim of this article is a reviewof their side effects, metabolic pathways and interactions, therapeutic drug monitoring and some dosage recommendations.

Myasthenia gravis with anti-MuSK antibodies

doc. MUDr. Edvard Ehler, CSc., MUDr. Jiří Piťha

Neurol. praxi. 2017;18(5):314-317 | DOI: 10.36290/neu.2017.104  

Myasthenia gravis is an autoimmune disease with formation of antibodies against the postsynaptic part of the neuromuscularjunction. When the presence of anti-acetylcholine receptor antibodies is demonstrated, the condition is referred to as seropositivemyasthenia gravis; when these antibodies are absent, it is seronegative myasthenia gravis. The anti-muscle-specific tyrosine kinase(MuSK) antibody was demonstrated in 35% (0–49%) of the seronegative forms. Anti-MuSK myasthenia gravis exhibits the followingfeatures: a predominance in women, earlier onset, characteristic clinical finding, and worse and inconstant response to treatmentwith cholinesterase...

T- lymphocytes and B-lymphocytes ant their role in multiple sclerosis

MUDr. Zuzana Matejčíková, Ph.D., prof. MUDr. Jan Mareš, Ph.D.

Neurol. praxi. 2017;18(5):318-321 | DOI: 10.36290/neu.2017.131  

The multiple sclerosis (MS) is an autoimmune inflammatory demyelinating disease of central nervous system. The inflammatoryprocesses are present especially in early stages of MS. The neurodegenerative processes play role especially in later stages. Thereare characteristic inflammatory infiltrations (particularly in white matter) in MS. In these lesions are activating cells, ie. T-lymphocytes,macrophages and B- lymphocytes. Autoaggressive T and B lymphocytes have various functions in patogenesis of MS andplay any role in the demyelination of nerve fibers and damage of axons. The knowledge of their function is important for understandingthe MS and for...

Efficacy and safety of ocrelizumab in multiple sclerosis

MUDr. Radek Ampapa

Neurol. praxi. 2017;18(5):322-325 | DOI: 10.36290/neu.2017.105  

Multiple sclerosis is a chronic autoimmune disease affecting the central nervous system. T and B lymphocytes play an important rolein the pathophysiology of the disease. The majority of anti-inflammatory drugs used in the treatment of multiple sclerosis affect theactivation of T lymphocytes in particular. Ocrelizumab is a novel monoclonal antibody that causes selective depletion of CD20 B lymphocytes.The drug has shown its efficacy in treating multiple sclerosis in both relapsing-remitting and progressive forms. In terms ofserious adverse effect rates, no statistically significant differences were found among ocrelizumab, interferon beta-1a, and placebo.

Characterization of eating disorder in progressive supranuclear palsy

MUDr. Ján Necpál

Neurol. praxi. 2017;18(5):326-328 | DOI: 10.36290/neu.2017.106  

Progressive supranuclear palsy (PSP) represents an atypical parkinsonian syndrome, presenting with symmetrical parkinsonism,early and progressive postural instability with frequent falls, supranuclear gaze palsy, frontal cognitive disorder and some othersymptoms. A poorly levodopa responsivity is typical. In the early course of disease develops an eating disorder, particularlyprogressive dysphagia, often leading to complications such as aspiration pneumonia, a frequent cause of death. In addition tothat, a distinct eating behavior is typical for the disorder, for example difficulty with judging the amount of food, binge eatingor recently described...

Functional Independence Measure and Functional Assessment Measure (FIM+FAM) as a tool for assessing functional status in neurorehabilitation

Mgr. Anna Stiborová

Neurol. praxi. 2017;18(5):330-333 | DOI: 10.36290/neu.2017.107  

In neurorehabilitation, more and more emphasis is being placed on demonstrating the efficacy of treatment and improving thepatient’s functional status. One of the options of detecting functional status changes is testing by means of standardized tests.Functional Independence Measure and Functional Assessment Measure (FIM+FAM) is an assessment tool specially developedfor neurorehabilitation needs. The test considers a lot of areas that can be affected in acquired brain injury including physical,mental, cognitive, and social functions. The assessment involves members of an interdisciplinary team: occupational therapist,clinical speech therapist,...

Stevens-Johnson syndrome: complications as part of neurointensive care

MUDr. Irena Doležalová, Ph.D., prof. MUDr. Ivan Rektor, CSc., prof. MUDr. Milan Brázdil, Ph.D.

Neurol. praxi. 2017;18(5):336-340 | DOI: 10.36290/neu.2019.049  

Stevens-Johnson syndrome (SJS) is a rare disease with an immunopathological background, most frequently occurring in associationwith a medication administered. The disease is characterized by skin manifestations, specifically maculopapular exanthema, blisters,and large skin defects. Skin signs are accompanied by general ones (temperature, anaemia, leukopenia, hypoalbuminaemia). Themortality is high, with patients most commonly dying from infectious complications. We present a case of a female patient who developedSJS as part of neurointensive care. The aim of this case report is to introduce this rare syndrome to neurological professionals.

Case reports of sporadic variants of Creutzfeldt-Jakob disease

MUDr. Tereza Lukoszová

Neurol. praxi. 2017;18(5):342-345 | DOI: 10.36290/neu.2017.013  

Prion diseases are the group of rare neurodegenerative diseases affecting both humans and animals, characterized by a longincubation period, rapidly progressing symptoms and infaust prognosis without the possibility of influencing the course of diseaseby therapy. The basic pathophysiological process is accumulation of "infectious" protein – prion in the brain tissue with itsirreversible spongiform damage. The most common human prion disease is Creutzfeldt-Jakob disease. It should be considered indifferential diagnostic process especially in cases of rapidly progressive dementia in combination with other neurological symptoms.The aim of this article...

In the shadow of cerebral palsy: a case of ADCY5 related dyskinesias

MUDr. Ján Necpál, MUDr. Matej Škorvánek, PhD.

Neurol. praxi. 2017;18(5):348-351 | DOI: 10.36290/neu.2017.041  

We report a case of 39 years old woman diagnosed with dyskinetic cerebral palsy and perinatal hypoxic encephalopathy. Two yearsafter a complicated delivery, paroxysmal choreatic limb movements, which became generalized and persistent later on, graduallydeveloped. After many years, a mutation in ADCY5 gene was identified through whole exome sequencing, which is probably thefirst reported case of ADCY5 related dyskinesias in Slovakia. We present a case report of the patient and a brief summary of theessential facts about this recently described condition.

XII. konference neurogenních poruch komunikace dospělých se konala v Brně

PhDr. Milena Košťálová, Ph.D.

Neurol. praxi. 2017;18(5):358  

Má lékař možnost odmítnout chronickou péči o pacienta pro osobní averzi, konflikt nebo neporozumění?

Neurol. praxi. 2017;18(5):352  

Neurozobrazovací kazuistika

MUDr. Martin Pail, Ph.D., MUDr. Marta Pažourková, prof. MUDr. Milan Brázdil, Ph.D.

Neurol. praxi. 2017;18(5):352 | DOI: 10.36290/neu.2017.108  

Edward Flatau (*27. 12. 1868–7. 6. 1932)

MUDr. Jan Bardoň, prof. MUDr. Petr Kaňovský, CSc., FEAN

Neurol. praxi. 2017;18(5):357