Neurology for Practice, 2010, issue 2

Editorial

Slovo úvodem

prof. MUDr. Ivan Rektor, CSc.

Neurol. praxi. 2010;11(2):75  

Main topic

Poruchy nervosvalového přenosu

MUDr.Jiří Piťha - editor hlavního tématu

Neurol. praxi. 2010;11(2):80  

Neurophysiology and electrodiagnosis of neuromuscular transmission

prof.MUDr.Zdeněk Ambler, DrSc.

Neurol. praxi. 2010;11(2):81-84  

This review summarizes the principles of normal neuromuscular transmission. Depolarization of the nerve terminal produces an influx of calcium through voltage-gated calcium channels and acetylcholine vesicles then fuse with the presynaptic membrane. After release, acetylcholine (ACh) interacts with the acetylcholine receptor (AChR); this opens the AChR channel, resulting in an influx of sodium. When a sufficient quantity of ACh binds to the receptors, sodium channels in muscle membrane open, leading to depolarization of the muscle and contraction. Electrodiagnostic studies can demonstrate a defect of neuromuscular transmission in two ways....

Autoantibody-mediated disorders of the neuromuscular junction

doc. MUDr. Peter Špalek, PhD.

Neurol. praxi. 2010;11(2):85-89  

The neuromuscular junction is the most accesible synapse in the nervous system and is very susceptible to circulating factors, notably neurotoxins and specific autoantibodies. Research advances have shown that key transmembrane proteins at the neuromuscular junction are vulnerable to antibody-mediated autoimmune attack. Autoantibodies lead to loss of the specific ion channels causing physiological defects in neuromuscular transmission. Main antigenic targets are acetylcholine receptors (AChRs) and muscle specific kinase (MuSK) in myasthenia gravis, voltage-gated calcium channels (VGCCs) in the Lambert-Eaton myasthenic syndrome (LEMS), voltage-gated...

Practical experience with the clinical diagnosis of myasthenia gravis

MUDr.Jiří Piťha

Neurol. praxi. 2010;11(2):90-94  

Myasthenia gravis is a clinically heterogeneous disease that is characterized by fluctuating muscle weakness and fatigue, which is worse after physical exertion or mental stress. This article summarizes the most common clinical manifestations and special investigation that aim to establish the diagnosis of initial stages of development of the disease.

Pharmacotherapy of myastenia gravis

MUDr.Stanislav Voháňka, CSc., MBA

Neurol. praxi. 2010;11(2):95-99  

Therapy of myasthenia gravis is based on two parts: symptomatic and disease modifying procedures. Among cholinestrase inhibitors (symptomatic therapy) pyridostigmine is widely used. This medication is administered in patients with the ocular or very mild form of myasthenia. In other patients immunosuppressive medication should be introduced. The key role plays prednisone and/or other steroid sparing drugs as azathioprine, cyclosporine, or mycophenolate mophetil. Plasma exchange or intravenous immunoglobulin is indicated in patients with myasthenic crisis or other severe resistant symptoms.

Congenital myasthenic syndromes and childhood myasthenia gravis

MUDr.Michala Jakubíková

Neurol. praxi. 2010;11(2):100-103  

We encounter both acquired and congenital disorders of neuromuscular transmission during childhood. Juvenile myasthenia gravis and transient neonatal myasthenia gravis are classified as acquired and autoimmune disorders. Congenital disorders of neuromuscular transmission are heterogenous genetically determined disorders which are known as congenital myasthenic syndromes. This review is dealing with their pathogenesis, classification, clinical and laboratory characteristics, various difficulties in the area of diagnosis including molecular genetic analysis and their treatment. analysis, prenatal diagnosis.

Review articles

Migraine and hormonal contraception

MUDr.Ingrid Niedermayerová

Neurol. praxi. 2010;11(2):104-106  

Migraine in women is a hormonal conditioned diseases and its prevalence changes during the course of life. Migraines typically occur during menses (menstrual migraine). Hormonal changes (pregnancy, lactation, perimenopause, menopause) influence their appearance significantly. Women with menstrual migraine can profit from continual use of combined oral contraception (COC). Migraines with aura in females is more likely to worsen due to COC and are higher at risk of migraine stroke, especially with those who smoke. Administration of hormone replacement therapy leaves the frequency of preexisting migraine in more than half women unchanged. Estrogen...

Neuropatic pain in diabetic neuropathy

doc.MUDr.Edvard Ehler, CSc.

Neurol. praxi. 2010;11(2):107-111  

Diabetic neuropathy may be associated with neuropathic pain. Type, intensity and localization of pain differ according to the type of neuropathy or the pathophysiology of neuropathic pain. In treatment of diabetic neuropathy and following neuropathic pain there is necessary to compensate diabetes and associated metabolic abnormalities. On the basis of many randomized clinical trials there was proved effectivity on neuropathic pain in diabetic neuropathy for tricyclic antidepressants, gabapentin/pregabalin, serotonin-noradrenalin- reuptake inhibitors and opioids. Monotherapy is successful in 30–40 % of patients, but the most patients should...

From the boundary of neurology

Speech disorders in Parkinson’s disease

Mgr.Gabriela Zamišková, MUDr.Pavel Ressner, Ph.D., Mgr.Jana Dlouhá, Dana Šigutová

Neurol. praxi. 2010;11(2):112-116  

The issues of speech disorders in Parkinson’s disease (PD) are discussed. The characteristic symptomatology in individual speech areas accompanying hypokinetic dysarthria (HD) is mainly dealt with. The symptomatology of HD is classified in terms of respiration, phonation, and facial musculature. The diagnostic and therapeutic procedures used in speech and language therapy are mentioned which are suitable in patients with PD and reported in the foreign literature as well as, and in particular, those used in our conditions. The Lee Silverman Voice Treatment (LSVT) method is dealt with in detail. Diagnosis and therapy of speech disorders...

Drug interactions of antiepileptic and antiparkinsonism drugs

MUDr.Jiří Slíva

Neurol. praxi. 2010;11(2):117-120  

The risk of drug interactions may very significantly tie our common medical praxis. Their eventual occurrence leads not only to the clinical complications in patient, however, it is associated with the increase of burden. The author depicts the most important drug interactions of both anticonvulsants and antiparkinsonian drugs.

Sexual dysfunction in Parkinson’s disease

PhDr.Petra Kotková, prof.PhDr.Petr Weiss, Ph.D.

Neurol. praxi. 2010;11(2):121-125  

Sexual life is an important aspect of the quality of life. The symptoms and treatment of the PD influence the sexuality of the people with this disease. This topic, however, has been devoted minor scientific attention, and conclusions of the researchers that have been carried out so far are often contradictory. The article therefore sums up the most important researches of the question oriented on bio-psychosocial factors causing a deterioration of the patiens´ sexual functions. The article contains frequently sexual dysfunctions of the men and women with Parkinson´s disease and basic information and recommendation about the treatment of these...

Case report

Anton-Babinski syndrome

MUDr.Michal Miler, doc.MUDr.Jan Roth, CSc., MUDr.Petr Dušek, Mgr.Ondřej Bezdíček, MUDr.Marie Kofránková, prof.MUDr.Evžen Růžička, DrSc.

Neurol. praxi. 2010;11(2):126-128  

We are presenting a rare case-report of a patient with two succesive hemorrhagic strokes in the area of both posterior cerebral arteries. She has suffered bilateral lesions of occipital lobes. However, due to the size of the lesions, the neighbouring structures of temporal and parietal lobes have been affected too, more severly on the left side. Clinically we found Anton-Babinski syndrome (cortical blindness, anosognozia, state of confusion, confabulations). We also could notice a cognitive impairment of the patient and Gerstmann syndrome (acalculia, agraphia, right-left disorientation, finger agnosia). We consider the cause of hemorrhagic...

Primary brain lymphoma

MUDr.Aleš Kopal, doc.MUDr.Edvard Ehler, CSc., MUDr.Zoltán Kerekes, MUDr.Milan Mrklovský

Neurol. praxi. 2010;11(2):129-132  

Primary central nervous system lymphoma (PCNSL) makes 1–2 % of all primary cerebral tumors. In contrast to lymphomas arising from nodal lymphatic tissues, PCNSL affect cerebral tissue. These tumors belong to a variety of non-Hodgkin lymphomas. Their occurrence is especially in immunocompromised patients. PCNSL is in principle good curable with chemotherapy and radiotherapy. The tumor sometimes does not show on follow-up imaging examination after intensive immunosuppressive therapy. Therefore, this tumor is also marked as a vanishing tumor. The diagnosis of PCNSL is verified by the immunohistochemical examinations which certify lymphoid...

Comments

Komentář k článku „The psychosocial impact of epilepsy in Czech children: what are causative factors of differences during ten years interval?“ Vladimír Komárek, Jana Šmídová; Epileptic Disorders 2007; 9(Suppl.1): S2-S8.

Mgr. Dana Brabcová1, doc. PhDr. Jana Miňhová, CSc.2

Neurol. praxi. 2010;11(2):134  


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