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Creutzfeldt-Jakob disease (CJD) is a form of human prion diseases,fatal neurodegenerative conditions. They can be etiologicallydivided into sporadic, hereditary and acquired forms.Conformational change of the normal (cellular) form of prionprotein (PrPc) to a pathological form (PrPSс) is a central event inthe formation of an infectious agent. In this article, diagnosticcriteria for sporadic CJD are summarized. Case report of probablesporadic CJD is presented. Many therapeutic strategies(based on cell cultures or animals) have been tested as potentialtreatments for prion diseases. However, only few clinical trialsare in progress now or have been completed.

Etiology of multiple sclerosis has both genetic and environmental components. The current conception is that multiple sclerosis pathogenesis comprises an initial inflammatory phase, followed by a phase of demyelination and last, a neurodegenerativephase. The mechanisms of inflammatory reactions, axonal lossand reparative changes have been discussed. There are two schemes of treatments: the escalating approach and the inductiontherapy. One of the main principles of disease modifying therapy of MS is treat-early approach. Results of interferons-b andglatiramer acetate comparison as first-line MS therapy havebeen described. The use immunosuppressive agents and futuredevelopments of MS therapy have been discussed.

Results of the two-year treatment with avonex of 34 patients withrelapsing remitting multiple sclerosis are summarized. This studyprovides evidence for the significant decrease in activity of thepathological process in patients treated with avonex, which is confirmedby the reduction of the relapse rate and the increase of the proportion of patients remaining exacerbation-free. The decreaseof the EDSS score has also been shown. In general, avonex was toleratedwell in patients under study.

Functional MRI (fMRI) is a new method promoting the study of brain functions and relationships between physiological activity and anatomical location. At present cortical reorganization is regarded as one of possible factors of recovery or maintenance of function in the presence of irreversible brain damage in multiple sclerosis (MS). Functional cortical changes have been demonstrated in all MS phenotypes using different fMRI paradigms, but the majority of studies were focused on the motor system. It was shown variability of functional reorganization of the motorcortex in MS depending on the stage of the disease. Cortical reorganization plays a role in limiting the impact of structural damage in MS; conversely, failure of these plastic mechanisms may cause irreversible disability upon the disease progression. Future dynamic fMRI studies will allow to access changes of functional brain activity in different disease severity and different extent of regress of MS symptoms. The improvement of cortical adaptive plasticity represents a potentially significant direction of rehabilitation in MS patients.

Balos concentric sclerosis is a monophase demyelinating diseasecharacterized by alternating rings of demyelinated and myelinatedaxons, and it is most frequently diagnosed by magneticresonance imaging. At the present time Balos sclerosis is discussedin case of MRI detection of two or more concentric areasof demyelination, one or more ring of that is enhanced by thecontrast. For 100-year period of the study there are 4 main theoryof pathogenesis of Balos sclerosis: theory of concentric demyelination,theory of distal oligodendrocytopathy, colloid theoryand theory of astrocytopathy. None of the theories includes alldetails of the disease. One of the details is concentric lesionsin monophasic Balo sclerosis and in Balo-like syndromes inchronic demyelinating disorders.In case of Balos sclerosis andBalo-like syndromes probability of resistant to immunosuppressivtherapy is very high. So glucocorticosteroid therapy must beinitiated right after concentric demyelination determination onMRI. In case of ineffectiveness of glucocorticosteroid therapyplasmapheresis and cytostatic therapy could be applied.

Motor disorders are a frequent syndrome in multiple sclerosis(MS), leading to severe disability, possibly associated with inefficiencyof adaptive mechanisms, probably because of widespreadstructural changes. Hand palsy with fine motor skillsdamage influences greatly on quality of life, because handfunction plays an important role in everyday activities andworking practices. Thus the aim of the study was to investigatefunctional and structural features of hand palsy in homogenoussample of MS patients during the relapse and in three months.Data were acquired from 25 relapsing-remitting MS patientsduring relapse also characterized by unilateral light hand palsy,and in three months. 12 age-matched healthy controls enteredthe control group. All patients underwent neurologica examination,including nine-hole peg test and dynamometry, andmagnetic resonance imaging of cervical spine and brain,including functional MRI and diffusion tensor imaging (DTI)sequences. All patients had lesions in spinal cord on cervicalspine MRI. fMRI data analysis during relapse showed differentlydirected activation changes and allowed to divide allpatients in two subgroups. These subgroups differed from eachother and control group by deep sensitivity and DTI values. Inthree months patients in the first subgroup had less completefine motor skills recovery, which was accompanied also bypreservation of activation changes and deep sensitivity asymmetryin comparison with the second subgroup. Received dataallow us to assume that clinically similar palsy, caused by spinalcord lesion, could be characterized by greater or lesser sensationimpairment contribution. In three months heterogeneousstructural and functional changes were observed, depending,possibly, on initially different pathologic mechanisms.Revealed differences should be taken into consideration whilerehabilitation planning.

Magnetic resonance imaging (MRI) is one of the main methodsof multiple sclerosis (MS) diagnostic, differential diagnostic anddisease course monitoring. Conventional MRI techniques havelow sensitivity in the assessment of diffuse damage of normalappearing white matter and focal/diffuse damage of grey matterwhich are of great interest in MS. Advanced MRI techniquesallow to get over these limitations and to obtain more specificinformation about MS pathogenesis. Thus MR-spectroscopymetabolites analysis helps to assess inflammation, myelin structure,remyelination and axonal loss or dysfunction, i.e. lets ussee pathochemical changes in MS. Demyelination and axonalloss differentiation is possible due to MR magnetization transfertechnique and also diffusion tensor imaging, which characterizewater diffusion in the brain tissue restricted by cell membranesand axonal cytoskeleton. Grey matter damage could be assessedusing sequences with one or two inverted impulses and morphometricanalysis of atrophy. Functional MRI performance usingdifferent paradigms characterizes cortical reorganization inresponse to functional injury. Vascular aspects of MS are alsowidely discussed nowadays. MR-perfusion and susceptibilityweighted imaging allow assessing perfusion and venous flowingchanges. State-of-the art neuroimaging methods let us performdetailed analysis of tissue damage in MS including the cell level,obtain more precise information about functional, metabolicand pathologic features, therapy influence on the inflammatoryreactions and neuroprotection. Perspective use of high-field MRscanners (more than 1.5 T) will lead to the increase of sensitivityof different pathologic mechanisms detection.

The article contains recent literature data concerning vascularaspects of multiple sclerosis (MS). The review provides a summaryof three vascular abnormalities described in MS which includeincreased risk of developing ischaemic stroke, widespread decreasein cerebral perfusion and chronic cerebral venouse insufficiencywith the assessment of the endovascular interventions efficacy.

In the paper, the results of 1-year treatment with avonex, a newimmunomodulator from the group of interferons b, in 34patients with relapsing remitting multiple sclerosis have beenreported. In the treated patients it was shown significantdecrease of the activity of the pathological process, which wasevidenced by reduction of the severity of exacerbations and therelapse rate. The decrease of disability of patients with multiplesclerosis (by EDSS score) was revealed. In general, one cannotice good tolerability of avonex.

Creutzfeldt-Jakob disease and others human prion diseases arefatal neurodegenerative conditions. Etiologic classificationincludes sporadic, hereditary and acquired forms.Conformational change of the normal (cellular) form of prionprotein (PrPc) to a pathological form (PrPSс) is considered centralto formation of the infectious agent. In this article themolecular classification of sporadic CJD, the phenotypic variabilityand the major pathogenetic pathways in prion diseaseshave been analyzed. The unique resistance of prions to classicmethods of decontamination, and evidence that prion diseasescan be transmitted iatrogenically pose a serious control todecontamination procedures. Many therapeutic strategies havebeen tested as potential treatments for prion diseases in cell culturesand in animals. But only few trials of human prion diseasehave been published or ongoing.

Trigeminal neuralgia is a rare symptom of multiple sclerosis affecting the disability. Multiple sclerosis related trigeminal neuralgia has been attributed to a demyelinating lesion in the pons. When the adequate pain drug-relieve therapy is not possible or when the patient becomes refractory to the treatment or can not continue pharmacological treatment because of the side effects, surgical intervention, including percutaneous radiofrequency rhizotomy is being discussed. Literature review and the data upon the efficiency and safety of this neurosurgical treatment in 16 patients with multiple sclerosis have been analyzed. Percutaneous radiofrequency rhizotomy has been proved to be a safe, reproducible and effective method of the symptomatic surgical treatment of trigeminal neuralgia in patients with multiple sclerosis in cases of the intolerance/inefficiency of the pharmacological therapy.

The current conception is that multiple sclerosis pathogenesiscomprises an initial inflammatory phase, followed by a phase ofdemyelination and, last, a neurodegenerative phase. The mechhanisms of inflammatory reactions and axonal loss have been disscussed. The crucial role of the immune system in disease pathogenesis has important therapeutic implications. The immunological effects of glucocorticosteroids underlie the beneficialeffect on multiple sclerosis relapse. Two classes of immunomodulators (interferon, glatiramer acetate) are approved for longterm treatment of multiple sclerosis. Immunosuppressive agentsand immunoglobulins used in treatment have been shown toexert immunomodulatory effects. Future developments of multiple sclerosis therapy have been discussed.