One of the basic principles of both conservative and surgical treatment of patients with spastic cerebral palsy is either maintaining or restoring the length of the spastic muscle. In the last twenty years, there has been an expansion in the use of botulinum toxin type A in order to selectively affect the muscle tone in children with cerebral palsy. An integrated approach to administering botulinum toxin type A combines the advantages of administration into multiple sites, redressing plaster cast treatment and the use of physiotherapy and orthoses. The whole concept is based on a relatively high-dose administration of botulinum toxin type A while maximizing the therapeutic effect by means of adjuvant therapy. Our more than ten years of experience with this concept confirm its short-term as well as long-term effect.

of non-compliance a case report of a young patient Wilson’s disease (WD) has to be diagnosed early and the treatment regimen must be followed very carefully. Non-compliance may have fatal consequences. We report a case of a young man with neuropsychiatric symptoms of WD that developed in association with drug abuse. The dominating neurological symptoms included dystonia and even status dystonicus, dysmetria, rigidity, akinesia, motor and vocal tics, impairment of speech and gait. A gradual increase of the penicilamine daily dose led to overall clinical improvement which was reflected by the fact that the patient got married and had a child. Later on, the patient withdrew the medication which was followed by an abrupt worsening of neuropsychiatric symptoms of WD. Despite the re-installment of treatment and repeated changes of penicilamine dosage over the time the clinical status remained unchanged.

The myotonic dystrophies (DM) are primary, progressive and degenerative disorders of skeletal muscles. Besides involvement of skeletal muscles, they cause heart, eyes, endocrine and brain disorders. DM are the most common muscular dystrophies in adults, they cause life shortening and affect quality of life. Main clinical features are myotonia and progressive muscle weakness.The classification distinguishes two main types of DM –myotonic dystrophy type 1 (Steinert´s disease), myotonic dystrophy type 2 (proximal myotonic myopathy, PROMM). A special type of DM1 is congenital myotonic dystrophy. The molecular genetics procedures are the most important diagnostic tools to establish the correct diagnosis. The molecular basis of myotonic dystrophies is expansion of an unstable trinucleotide repeat sequence CTG in noncoding part of a DMPK1 gene in DM1 and tetranucleotide repeat sequence CCTG of ZNF9 gene in DM2. Further, electromyography is an important diagnostic procedure to confirm specific myotonic discharges in skeletal muscles. The causative treatment is not available, but long term cardiological and ophtalmological care is required. The genetic counseling is very important in DM1 and DM2 families.

Clinical results of vagus nerve stimulation in pharmacoresistant epilepsy patients and the data showing the widespread effect of vagus nerve stimulation on central nervous system structures elicits the consideration of vagus nerve implantation in another functional disorders. The most extensive data are available for the treatment of treatment resistant depression, data about other functional disorders are limited to case reports or small group of patients from single departments. Exact definition of indication criteria with respect to deep brain stimulation and definition of clinically usable predictors of clinical response are the prospectives for future research.

In spite of the fact, that we don´t correctly know the ethiopatogenesis of Alzheimer´s disease and thus we are unable to cure this disease, it is important timely diagnostics. At the case of the determination of correct diagnose we are able to start therapy in time. This therapy is able to remove the advanced stadia of the disease, to extend the mild stadia of the disease, to improve of the quality of live of the patiens. For the diagnostics there are still used McKhann´s kriteria, going out of the clinical picture of the disease. In 2007 there were elaborated by prof. Dubois et al. the new criterias, based on the determination of the protein triplet (tau, phospho-tau and beta-amyloid 42) in cerebrospinal fluid, on the MRI scan (volumetry of medial temporal areas and the temporal corners of side brain chambers), on the determination of the cerebral blood flow in the temporal and parietal lobes by positron emission tomography and on the determination of beta-amyloid deposits in the brain by positron emission tomography. Important biomarker of Alzheimer´s disease is early disturbance of episodic memory. In present time there are investigated the new diagnostic methods, for instance the determination of some biomarkers from the blood.

Severe spasticity and painfull muscle spasms worsen quality of life, mobility and self-care in patients suffered form chronic spinal cord injury. Poorly managed spasticity leads to serious long-term complications such as pressure sores, chronic pain, infections and fixed muscle contractures. Ashworth scale of muscle hypertonia, frequency of muscle spasms and visual analogue scales are the most common ones used in clinical practice for evaluation of spasticity. The most common treatment of spasticity is oral drug therapy (benzodiazepines, tizanidine, and baclofen). A local administration of botulinum toxin is suitable for focal spasticity in a small muscle group. In severe flexed spasticity, ablation procedures may be performed, such as selective peripheral neurotomy, lateral longitudinal myelotomy and posterior rhizotomy. Physical treatment is also very important. In severe generalized spasticity a long-term treatment often needs higher oral doses of drugs or various drug combinations, which leads to exaggeration of general adverse effects. Intrathecal continuous baclofen administration could resolve this problem. Management of spasticity is a long-term process and it needs multidisciplinary approach to achieve the real treatment goal for each patient.

We have disease modifying drugs (DMDs – interferon beta, glatiramer acetate) for the treatement of multiple sclerosis (MS) for 15 years in Czech Republic. Only 30 % of patients are full responders to this treatment, in the rest the effect is insufficient or partial.The only possibility for treatment escalation until 2008 was mitoxantrone (with only transient effect and serious adverse events). Since 2008 we can use the first monoclonal antibody in neurology, natalizumab, with doubled clinical efficacy in comparison with classical DMDs. Natalizumab has brought the concept of „freedom of disease activity“. New drugs which are currently in registration process (fingolimod) or finished their phase III program (cladribine, teriflunomide, laquinimod, fumarate) will offer higher efficacy or convenience for patients but will demand more knowledge about their mechanism of action and risks, their prevention, diagnosis and management. The same is true for new monoclonal antibodies (alemtuzumab, anti-CD 20, daclizumab). Informed consent and evaluation of compliance will be part of the treatment of well-educated patients. The main concern being the early diagnosis using the new criteria and early treatment. will be the most important factors changing the prognosis of MS patients. anti-CD 20, daclizumab.

According to Health Organization (WHO) adherence is defined as the extent to which the patients behaviour – taking medication, following a diet, and/or executing lifestyle changes – corresponds with agreed recommendations from a healthcare provider. High level of adherence is necessary in the treatment of chronic diseases. Following article deals with adherence in the treatment of multiple sclerosis, especially focusing on the disease modifying drugs of first choice, that are all injections, and need to be taken long term and regularly. Some methods, how to measure adherence are discussed, as well as factors leading to non-adherence and some recommendations how to increase adherence. It is necessary to support adherence all the time in everyday clinical practice. Increasing the effectiveness of adherence interventions may have a far greater impact on the health of the population than any improvement in specific medical treatments.

Amyloidosis is a group of diseases characterized by deposition of amyloid in tissues. Hereditary amyloidosis with defect of transthyretin is a disease with deposition of deficient protein transthyretin. Polyneuropathy, vitritis, kardiomyopathy, gastrointestinal disorder with malabsorbtion, hepatomegaly are dominant in the clinical picture. A liver transplantation is the most effective therapy, because the place of synthesis of deficient protein is removed. I present the case of a woman suffering from this disease and describe the way, how her diagnosis has been proved.

Epilepsy is one of the most common neurologic disease. Despite the improvement of the neuroimaging methods several structural pathologies (especially malformations of the cortical development – MCD), which are in the clear-cut causation of disease, do not have to be detected by classical CT or MRI examination. Because visual evaluation of MRI remains difficult to detect some subtle abnormalities, several neuroimaging approaches, methods and modalities have been established to improve detection and localization of MCD. These methods are called in expert literature as a MRI postprocessing. (Jackson at al., 2010). This specific approaches enable to detect the morphological changes of brain structure not detected by visually inspections of MRI scans and due to their superior contribution in the determination of MCD, they might be the indivisible part of a presurgical evaluation of brain.

Congenital muscular dystrophies are genetic muscle diseases. They present with early hypotonia and muscle weakness. The muscle biopsy is characterized by dystrophic or myopathic changes. There are several subtypes of congenital muscular dystrophy. The vast majority of the diagnoses are confirmed by molecular testing or by immunohistochemistry. Treatment is symptomatic, interdisciplinary. Genetic counselling should be available for teenagers and families.

A 39-year-old female patient was admitted to our department for traumatic compression fracture of the Th9 vertebral body. The clinical finding as well as MRI finding favoured percutaneous kyphoplasty. A rare complication – cement leakage into the soft paravertebral tissues – occurred during this procedure. Because of the declared properties of the implanted material, particularly the biodegradability of bone cement, observation of the patient was carried out. Over the course of days, progressive back pain and difficult breathing occurred. Imaging methods revealed progressive pleuropulmonary changes. A circumscribed inflammatory focus and pleural effusion with subsequent respiratory insufficiency developed. The patient was indicated for abscess evacuation, extirpation of the cement from the osseous tissue and subsequent thoracic drainage. However, the patient’s condition continued to deteriorate as did the imaging finding and a second revision was necessary which involved pulmonary lobe decortication. Following this procedure, the further clinical course was favourable. thoracotomy.

Early recognition of the dementia syndrome is the most important part in the diagnostics of Alzheimer‘s disease. It is followed by determination of Alzheimer‘s disease as the most likely cause of it. A clinical transitional state between the normal ageing and dementia is referred to as mild cognitive impairment. The auxiliary diagnostic methods are not only used to rule out other causes of dementia, but they can directly support the diagnosis of Alzheimer‘s disease. The clinical trials have proved that current drugs slow the progression of the disease. We can use acetylcholinesterase inhibitors for treatment of mild to moderate Alzheimer‘s disease, whereas severe stages of the disease are treated by memantin – NMDA receptor partial antagonist.

The article deals with the causal association between cerebral palsy (CP) and peri-/neonatal morbidity. CP is the most common and significant motor impairment in childhood. Prevalence ranges from 1,7–2,1/1 000 live births, and increases with decreasing birth weight and gestational age. The etiology is multifactorial, including prenatal, peri- and neonatal factors. Diagnosis is mainly clinical, special exams and tests are under consideration for the uncertain history and nonspecific clinical manifestations. Imaging methods will help establish a brain lesion and its topography. CP often combines with other deficits (i. e. mental, epilepsy, sensory, speech etc.). Its classification is based both on the assessment of the tone with dominating physical abnormalities and functional disability. Causal treatment is not possible, so preventive strategies focus mainly on reducing prematurity, fetal growth retardation, perinatal hypoxia and infections.

Seizure freedom is one of the main goals of therapy in epilepsy patients as it is associated with significant quality of life improvement. There is still no consensual definition of seizure remission regarding the period of its duration. For patients, long-life remission is definitely the most acceptable. The way how to reach seizure freedom starts with initial monotherapy with adequately chosen antiepileptic drug. Most of the patients but not all are responding to moderate or even modest dosing, in some higher or maximal dosing is necessary to reach the full benefit. In initial monotherapy failure the further step is alternative monotherapy or polytherapy. Though monotherapy is tradionally viewed as a gold standard, both approaches should be considered in individual patient. In certain cases adding another drug may be less risky than the trial of converting from one monotherapy to another, mainly in patients with frequent or severe seizures. Monitoring and assessment of adverse effects of therapy is an inevitable part of epilepsy treatment as these can strongly influence the quality of life. Polytherapy seems to carry a higher risk of adverse effects however with new mechanism of action of modern drugs and with individual tailoring of drug dosing the adverse effects are comparable to monotherapy.

Spontaneous spinal epidural hematoma is a rare clinical entity. It represents less than 1 % of all lesions located in the epidural space of the spinal canal. Our case report describes 63-year-old patient admitted for a suddenly incurred intense neck pain and paresthesia in the right upper limb with progression to severe right spasic hemiparesis. The magnetic resonance showed epidurally located mass in the area C3–4, dorsal to the spinal cord with its significant compression. We urgently evacuated the expansion. No possible cause of hematoma was discovered during the surgery and the histopathological exam disclosed only parts of hematoma. Four years after the surgery the patient is self sufficient with slight spastic monoparesis of the right lower limb. The magnetic resonance shows appropriate width of the spinal canal in the primarily affected area.