Neurologicka Klinika

Olomouc, Czech Republic

Neurologicka Klinika

Olomouc, Czech Republic
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Vohanka S.,Neurologicka Klinika
Ceska a Slovenska Neurologie a Neurochirurgie | Year: 2017

Myotonic dystrophy is the most frequent muscular dystrophy of adult age characterised by muscle weakness, myotonia, cataracts, and autosomal dominant inheritance. The disease is caused by trinucleotide expansion in the DMPK gene in case of myotonic dystrophy type 1 (MD1) and tetranucleotide expansion in the CNBP/ZNF9 gene in myotonic dystrophy type 2 (MD2). The accumulation of RNA transcripts and its toxicity leads to dysregulation of many other genes, providing a clue for understanding of the broad clinical spectrum of the disease. MD1 manifests from birth (congenital) to adulthood. Severity and time of onset correlate with the number of repeats. On the contrary, MD2 is a disease with an onset during adult age only. Localisation of muscle weakness is also different; the facial muscles, paraspinal, distal muscles of upper and lower limbs are affected in case of MD1 and the proximal muscles, esp. of lower limbs are involved in patients suffering from MD2 - this localisation determined the former name: proximal myotonic myopathy. In contrast to MD1 that has worldwide prevalence, MD2 is predominantly restricted to middle and northern Europe. The heart conduction system (arrhythmias) is affected in patients with either type of the disease. In general, the impact of the disease is more severe in patients with MD1 than in MD2. Due to involvement of many systems, a multidisciplinary approach and team should be involved in the management of these patients.

Kadanka Z.,Neurologicka Klinika
Ceska a Slovenska Neurologie a Neurochirurgie | Year: 2010

Spondylogenic cervical myelopathy (SCM) is a serious disease of the spinal cord of unknown prevalence, but it is not rare, and although a full 40 years have passed since its discovery it remains underdiagnosed. It the most frequent myelopathy in subjects over 50 years of age. It is a consequence of progressive cervical spondylosis involving the osseous, fibrous and cartilaginous structures of the spine and producing stenosis of the canal together with static or dynamic compression of the cord. The clinical picture is extremely variable. MRI and myelo/CT are the best diagnostic tools. Therapy of this potentially devastating disease is still unsatisfactory because of a lack of knowledge about predictive factors for good outcomes from conservative treatment or surgery, while optimal timing for surgery is also ambiguous. The authors summarise the history of the disease and approaches to it, address the current state of knowledge, and add the results of their own experience.

Kadanka Jr. Z.,Neurologicka Klinika
Ceska a Slovenska Neurologie a Neurochirurgie | Year: 2013

Even though EMG of the pelvic floor has predominantly been used in research it is also often indicated in routine clinical practice. The article describes electrophysiological tests used to diagnose pelvic floor lesions and emphasises their beneficial effect and diagnostic value.

Stetkarova I.,Neurologicka Klinika
Ceska a Slovenska Neurologie a Neurochirurgie | Year: 2013

Spasticity is one of the complex motor system disorders and is a sign of central motor neuron disorder caused by trauma, ischemia or haemorrhage, inflammation, degenerative process or tumour. Spasticity is a symptom associated with a lesion within the central motor neuron. In addition to increased muscle activity, muscle contraction and paresis also occurs. Voluntary movement is associated with co-contractions and synkinesis, adversely affecting dexterity and muscle strength. Flexor and extensor spasms also have a negative effect. There are two types of spasticity: cerebral and spinal. Cerebral lesions occur either above the level of brainstem or at the level of brainstem. Due to these lesions, cerebral cortex looses its control over the inhibitory structures of the brainstem. Spinal lesions lead to severe spastic dystonia with predominant involvement of flexors. An objective examination is required to assess the type and degree of spasticity. An objective examination should be performed at the start of treatment and it is the main parameter determining further management. Routine clinical practice mainly relies on clinical evaluation scales. Neurophysiological, physical and biochemical methods are used less frequently and mainly at specialised centres. Rather than complete amelioration of spasticity, the aim of treatment is to alleviate its negative effects on activities that limit the patient. Achievable aims should be set by the patient and the physician at the initiation of treatment. Rehabilitation has a crucial role in the treatment of patients with spasticity. Pharmacotherapy is used, also due to uncomplicated application. Owing to its high efficacy and low incidence of adverse effects, botulinum toxin has an exclusive place in the treatment of focal spasticity. Administration of baclofen via intrathecal pump systems has been proven as highly effective in patients with severe generalized spasticity. New technologies and new treatment modalities, including advanced use of robots, are being developed.

Anticoagulant therapy plays an important role in thromboembolic disorders, in primary and secondary prevention as well as treatment, and especially in the prevention of brain systemic embolism in patients with non-valvular atrial fibrillation and other sources of cardioembolic strokes. Unless contraindicated, warfarin is recommended for the treatment of these patients. Warfarin significantly reduces the risks of stroke and death, but increases that of haemorrhage. Life-threatening intracerebral haemorrhage is the most serious complication of oral anticoagulant therapy, with a mortality that can exceed 50%. The need for frequent laboratory monitoring, a narrow therapeutic window, and a plethora of interactions with food and drugs are also reasons to seek new, safe, effective anticoagulant agents that are convenient to use. New oral direct thrombin inhibitors and factor Xa inhibitors present promising alternatives to warfarin. Unfractionated heparin or low-molecular-weight heparins are not recommended for the prevention of early stroke recurrence in patients with acute ischemic stroke of cardioembolic origin. Low-dose unfractionated subcutaneous heparin or low-molecular-weight heparins should be considered for patients with acute ischemic stroke at high risk of deep venous thrombosis or pulmonary embolism.

Kotas R.,Neurologicka Klinika
Bolest | Year: 2010

The survey deals with all important aspects of migraine. At the beginning the epidemiology, impact of the disease on the individual and the whole society and clinical picture are mentioned. In the next part the pathophysiology of migraine is described including cortical spreading depression, trigeminal vascular system, vasoactive neuropeptides, especially calcitonin gene-related peptide, vasodilatation and sterile neurogenic inflammation, transmission of the pain along the trigeminal pathway, mechanisms of peripheral and central sensitization, significance of brainstem generator, serotonin and nitric oxide. Genetics of migraine and provoking factors are described. Menstrual migraine is specially mentioned. Then the pharmacotherapy of migraine follows, which is divided into the acute and prophylactic treatment. From the acute therapy except other medications especially triptans are described in detail. From the prophylactic treatment all drug groups used in migraine are listed. The paper continues with the treatment of menstrual migraine, migraine in pregnancy and pediatric migraine. The last part is dedicated to the future therapy which includes receptor antagonists of calcitonin gene-related peptide, glutamate receptor antagonists, especially memantine, then tonabersat, some anti-epileptic drugs and inhibitors of nitric oxide synthase.

Otruba P.,Neurologicka Klinika
Diabetologie Metabolismus Endokrinologie Vyziva | Year: 2015

Diabetic neuropathy represents a chronic complication of diabetes mellitus and is the most common peripheral neuropathy. Current therapeutic options for neuropathic pain according to actual guidelines include anticonvulsants and antidepressants along with compensation of underlying disease and maintenance of normoglycemia. Neuritogen, food supplement, contains polyphenolic antioxidants curcumin, resveratrol and Mangifera indica extract, as well as vitamins B2 and B6, lecithin and piperine. Available information on the effects of the individual components suggest that the product exhibits antioxidant and neuroprotective properties. The aim of the prospective non-interventional study was to assess the efficacy and safety of Neuritogen in patients with painful diabetic neuropathy who had previously either no treatment of diabetic neuropathic pain (DNB) or already had an established treatment of DNB. 120 patients (59 % men and 41 % women) with DNB were followed at 11 specialized centers in the Czech Republic for 2 months. Neuritogen was administered at a dose of 2 tablets per day. The main evaluated parameters were pain intensity (visual analogue scale VAS) and quality of life measured by a validated questionnaire EQ-5D (EuroQol-5D). The results showed after both one and two months, a statistically highly significant (p <0.01) decrease in pain intensity (baseline VAS 5.34, the first and second control 4.48 and 3.97 respectively), a statistically highly significant (p <0, 01) increase in quality of life (baseline 0.703, the first and second control 0.745 and 0.762 respectively) and statistically highly significant increase of the EQ-VAS scale (baseline 54.2, the first and second control 60.6 and 66.5 respectively). Reported adverse drug reactions were not serious, 3.3% after the first month and 5.5% after the second month of use. The positive effect was recorded in both subgroups - in patients who were previously untreated, and also in patients with already established anticonvulsant, antidepressant, or combined medication. Promising results of the study, however, need to be verified by further follow-up in randomized double blind clinical trial.

Prihodova I.,Neurologicka Klinika
Ceska a Slovenska Neurologie a Neurochirurgie | Year: 2012

Sleep-related rhythmic movements (RM) (jactatio capitis et corporis nocturna) are mostly transient physiological phenomena in infants and toddlers. They are characterized by stereotyped, repetitive rhythmic movements of head, neck and trunk, less commonly of extremities (head banging, body rocking, head and body rolling, leg banging or rolling). In some cases they can persist into childhood or adulthood and may be associated with sleep fragmentation, impaired daytime functioning or with a risk of injury. RM with clinical consequences are known as rhythmic movement disorder (RMD). RM occur during sleep onset or during superficial sleep but they can be present during any sleep stage. RM can be very intense and disturb sleep of the entire family. Aetiology and pathogenesis are unknown. RM in infants and toddlers do not require any treatment. Various psychotherapeutic techniques and short-term administration of benzodizepines are used to manage RMD. Obstructive sleep apnea or restless legs syndrome can play a role as a trigger of RM and nocturnal videopolysomnography may be helpful for accurate diagnosis of RMD and associated sleep disorders.

Gdovinova Z.,Neurologicka Klinika
Ceska a Slovenska Neurologie a Neurochirurgie | Year: 2013

Creutzfeldt-Jacob disease (CJD) is a prion disease. Prion diseases are fatal neurodegenerative conditions affecting humans and a wide variety of animals. In prion diseases, normal, cellular protein (PrPC) is converted into insoluble, protease-resistant scrapie prion protein (PrP sc). PrP is encoded by the prion protein gene (PRNP) localised on the short arm of chromosome 20. There are several forms of CJD, sporadic CJD (sCJD), seen in about 85% of patients, being the most common one. Some cases are genetic (gCJD) that occur as a consequence of various mutations (including point mutations, insertions and deletions); when the mutation is present in more than two members of a family, the disease is called familial (fCJD). Remaining two forms, i.e. iatrogenic CJD (iCJD) and variant CJD (vCJD), are acquired. iCJD (now called accidentally transmitted) is acquired through contaminated transplants and instruments. vCJD is a novel form of human prion disease first reported in the United Kingdom in 1996. It is likely that bovine prions from "mad cows", affected with bovine spongiform encefalopathy (BSE), were passed to humans through consumption of beef products from affected animals.

In developed countries, nutritional deficiency only rarely causes polyneuropathy or other disorders of the nervous system. When it does occur, it appears in alcoholics or in the malnourished - people with long-term psychiatric disorders, with gastro-intestinal system disorders or on extreme diets. Paradoxically, certain initially obese patients may become malnourished. Pathological obesity is addressed in various ways, with the shared target of restricting food intake. Bariatric surgery is one of the options. However, such a profound diminution of nutrition may induce unpredictable imbalance or insufficiency in nutrients important for normal functioning of the nervous system. The number of bariatric operations is steadily increasing, both in the Czech Republic and elsewhere; in the USA, they already outnumbered cholecystectomies. Complications associated with bariatric surgery include polyneuropathy, plexopathy, radiculopathy, myelopathy, encephalopathy and optic neuropathy, all of which may appear both early as well as many years later. The aetiology of these disorders remains unclear, although vitamin insufficiency (thiamine, B12, E), copper deficiency and hypoglycaemia are often held responsible. However, many factors remain hidden and supplementation of the "missing" factors sometimes fails to affect the course of the disease. Damage to the immune system or inflammation may contribute to the aetiology. Weight loss, gastro-intestinal complaints, albumin and transferrin decrease and rehospitalisation for digestive symptoms are considered risk factors. Regular post-surgical monitoring in nutrition centres together with specific treatment of complications is recommended.

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