2010, Vol 10, No 2
Chemokines in pathogenesis of ischaemic stroke
AKTUALN NEUROL 2010, 10 (2), p. 79-84
ABSTRACT

Chemokines are cytokines which attract certain supopulations of leukocytes. They constitute the family of 50 proteins of low molecular weight (8-12 kDa). They are divided into four groups: CXC, CC, CX3C, C. Chemokines acts via receptors C-R, CC-R, CXC-R, CX3C-R. About 20 receptors for chemokines are described so far. Many chemokines may bind to one receptor and one chemokine may target more than one receptor. Chemokines exert many physiological activities as well as can be involved in pathogenesis of ischaemic stroke. The main role of chemokines is engagement into development of inflammatory reaction. Chemokines are engaged also in maturation and function of immunological system as well. Further they are engaged into pathogenesis of many other pathologies like myocardial infarction, ischaemic stroke, multiple sclerosis, Alzheimer’s disease, brain tumours. The increased expression of chemokines in the brain is induced by different stimulus as ischaemia, axonal damage, or presence of neurotoxic substances. Till now, many chemokines were investigated because of their participation in development of atheromatous plaque in carotid arteries of animal models and humans is increased as well. Expression of selected chemokines on atheromatous plaques from patients operated because of critical stenosis of internal carotid artery was described. Chemokines belonging to different classes (CCL2, CXCL1, CX3CL1, CCL5, CXCL1) with proven but not finally investigated participation in atherogenesis and its complications were analysed. Furthermore concentration of selected chemokines in peripheral blood and expression of some chemokines on peripheral blood mononuclear cells from patients with and without restenosis was also published. Chemokines are engaged also in complications of atherosclerosis such as ischaemic stroke or myocardial infarction. The goal of this review was to highlight the role of various chemokines and their receptors in such conditions. Experimental data with knock-out genes or agonists of chemokine receptors give the hope to development of new therapies for brain ischaemia.

Keywords: chemokines, ischaemic stroke, atherosclerosis, chemokine receptors, cervical artery
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Interferon beta treatment failure in multiple sclerosis: criteria, characteristics and attempt of minimization
AKTUALN NEUROL 2010, 10 (2), p. 112-117
ABSTRACT

Background: The greater part of patients with RRMS treated with IFN beta shows beneficial modification of the disease. Treatment failure (TF) to IFN beta occurs in 7-49% of RRMS patients depending on the criteria used to define a non-response. Objective: Presentation of criteria, characteristics, prediction of a non-response to IFN beta and brief description of attempts to minimize TF. Method and patients: A critical survey of 35 articles on natural course and IFN beta treatment of RRMS patients was carried out.Results: Criterion of permanent TF based on disability progression (≥1 EDSS step confirmed at 6 months) in RRMS patients receiving IFN beta over 2 years had high sensitivity (85%) in defining a not temporary non-response. Prior to the IFN beta treatment non-responders had lower relapse rate (1.4), higher disability score (2.7 at EDSS) and the greater number of enhancing lesions (3) than responders (1.8, 1.9, 0); p<0.05. Permanent TF is more probable during the first 2 years of IFN beta treatment if there is confirmed disability worsening (>1 step at EDSS). Unchanged TF is less probable if there are new relapses or at least 3 new T2-related lesions. Attempts to minimize TF include appropriate selection of patients, maintenance of symptomatic treatment, management of side effects, no IFN beta treatment interruptions, increase of a dose, lowering of IFN beta neutralizing antibody titre, switching to the 1st and the 2nd line immunomodulating drugs or to monoclonal antibodies (natalizumab). Conclusions: Defining permanent TF depends on criteria of worsening disability. Prediction of enduring TF may be established on previous disability deterioration and the greater number of antecedent, active enhancing lesions (Gd+). The minimization of TF to IFN beta is complex, not always effective procedure and should aim at eliminating the cause of a non-response.

Keywords: multiple sclerosis, interferon beta, a non-response, minimization of treatment failure
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Clinimetrics in cervical spondylotic myelopathy
AKTUALN NEUROL 2010, 10 (2), p. 89-93
ABSTRACT

Cervical spondylotic myelopathy (CSM) is a common disease. It is caused by increasing narrowing of the spinal canal causing compression on the nerve roots and spinal cord. The first symptoms usually are: whiplash neck pain, numbness of the hands, gait disorders, sphincter dysfunction and impotence. Neurological investigation firstly shows hand paresis and muscle atrophy, later on the paralysis of the lower limbs and the neurogenic bladder dysfunction. Often, it is concluded the presence of depression. Some patients required surgical treatment. In this review article the most commonly used scales for the clinimetric evaluation of patients with CSM has been described. There is a wide range scales for the basic clinical assessment of patients with CSM. The most commonly used are: Neck Disability Index (Modified Oswestry Low Back Pain Index), Ranawat grade, Nurick scale, Cooper scale, Myelopathy Disability Index (MDI), Japanese Orthopaedic Association (JOA) score, and European Myelopathy Score (EMS). For evaluation of pain most often used is Visual Analogue Scale (VAS; Carlsson, 1983). Basic depression scale used in CSM is Hospital Anxiety and Depression Scale (Zigmond and Snaith, 1983). In assessing the patient’s autonomy in activities of daily living (called activities of daily living, ADL) still widely used is Barthel Index. The most accurate estimation of everyday functional capacity is Functional Independence Measure (FIM). For assessment of hand function test most commonly used are: Nine Peg & Hole test (Kellor, 1971) and Jebsen-Taylor Test (1969). Prabhu et al. in 2005 assessed the results of surgical treatment of CSM’s using the Rapid Hand Flick Time (RHFT). For walking quantitative tests are most commonly used transition distance of 6 meters, 10 meters, 20 meters. Nurick in the year 1972 proposed six-level scale of qualitative walking assessment in patients with CSM which is commonly used so far. Ranawat assessed gait in patients with SCI in the CSM using neurological four-level scale (1979). Since more than 50 years for patient eligibility for surgery and the outcome measure the criteria Odom’s (1958) are still used. Myelopathy Disability Index (MDI) was created in 1996 by Casey et al. as modification of Stanford Health Assessment Questionnaire (HAQ). Modified by Keller, 1993 Japanese Orthopaedic Association Cervical Spinal Myelopathy Functional Assessment Scale (mJOACSMFAS) assesses four features: hand, gait, sensory and sphincter urinary bladder.

Keywords: cervical spondylotic myelopathy (CSM), clinimetrics, outcome measure, clinical evaluation, prognosis
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Fundamentals of anatomy and physiology of the olfactory tract and possibilities of its topodiagnostic damages by means of olfactory evoked potentials
AKTUALN NEUROL 2010, 10 (2), p. 85-88
ABSTRACT

Introduction: The recognition of the influence of olfactory stimuli on the olfactory organ aims at identifying damages within this area, diagnosing many diseases and smell impairments such as parosmia, hyposmia, anosmia and cacosmia. This work is focused on presenting current knowledge on fundamentals of anatomy and physiology of the olfactory tract and possibilities of its topodiagnostic damages by means of olfactory evoked potentials. Definition of smell: In the surrounding world the sense of smell operates through a series of sensations, which are described as smell. The smell of a compound depends both on the structure of a carbon chain and a ring, and on the presence and type of functional groups and their arrangement in a molecule. Defining a smell on the basis of its structure can be particularly difficult. In future the answer should be found in the electron theory of organic compounds structure. Anatomy and physiology of the sense of smell: The receptors, which are responsible for receiving olfactory sensations, are located in the nasal cavity, the upper part of the nasal septum, the root and anterior portion of the superior nasal concha. In adults the olfactory epithelium covers 1-3 cm2 of the mucosa in each nasal passage. The olfactory tract comprises three neurons, whereas the cortical centre is situated in the hippocampal gyrus and amygdalic nucleus of the temporal lobe. Olfactory sensations are perceived via the olfactory epithelium, which is often accompanied by additional stimulation of endings of the trigeminal, facial, glossopharyngeal and vagus nerve. Diagnostics of the sense of smell: Registering olfactory evoked potentials is an objective method of testing the sense of smell by detecting changes in bioelectrical functions of the brain. In Poland this method is quite modern and, in fact, is seldom used because of the lack of an appropriate batcher. Conclusion: Uniform standards and methodology of the study should lead to further implementation of this objective testing method in topodiagnostics of smell disorders.

Keywords: anatomy, physiology, olfactory tract, topodiagnostics, olfactory evoked potentials
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The influence of antiepileptic drugs on the indications of osteoporosis
AKTUALN NEUROL 2010, 10 (2), p. 105-111
ABSTRACT

Osteoporosis is a generalized disease of the skeleton, characterized by low bone mineral density and disturbance of microarchitecture, leading to a lower mechanical endurance of bones and enlargements of susceptibility to fractures. One of the most significant features leading to osteoporosis is treatment with AED. Epilepsy usually reveals at young and needs long treatment. For the early diagnosis of osteoporosis the most important is estimation of real reduction of bone mineral density (BMD). Nowadays the most popular diagnostic method is dual energy X-ray absorptiometry (DEXA). Yet it reveals advanced level of osteoporosis, which cannot be reversed. Thus there is a need for diagnosis development of new markers of bone metabolism: markers of bone formation and bone resorption. The studies were conducted in group 100 patients aged 20-50 treated over 2 years with AED and in the control group – 40 persons properly chosen in respect to age and sex. The patients were examined by DEXA and bone markers: CTX (collagen type and cross-linked C-telopeptide) and oste-ocalcin. The persons suffering from other diseases or intaking different speciments affecting bone metabolism were eliminate from both groups. Results: Long-term intake AED considerably affects the decrease of BMD. It takes only few years of AED intake for the patients to develop osteopenia or osteoporosis. CTX is the most sensitive marker in patients treated below 6 years, while DEXA is most sensitive in patients treated longer than 6 years. The biggest changes in bone metabolism were found in patients treated with phenytoin.

Keywords: osteoporosis, antiepileptic drugs, bone markers, dual energy X-ray absorptiometry
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The contemporary look on physiotherapy in Parkinson’s disease – some chosen questions
AKTUALN NEUROL 2010, 10 (2), p. 94-99
ABSTRACT

Morbus Parkinsoni (Parkinson’s disease, PD) is the neurodegeneration illness of the central nervous system, which characterizes tricky beginning and slow progress of the symptoms, meanwhile pathomorphology – the degeneration of the cells of part compact black matter including the dye neuromelanin. Clinical symptoms appear after destruction about 50% cells of the black matter and the fall down of production dopamine. This disease belong to the extrapyramidal system diseases, in which applies symptomatological treatment (pharmacotherapy, rehabilitation) what permits lengthen and bear current efficiency and physical condition. In the treatment comply also the operating treatment – neurosurgical, which the aim is the improvement patient’s quality of life. Complex rehabilitation should be initiate on every stage of the disease and hold in continuous way, not just in hospital term, as well as ambulatory too. The use of the different possibilities and the therapeutic methods in significant modus accelerate return to optimum functional patient’s efficiency in dependence from the degree of advanced disease, and this is the succession of improving. Rehabilitation in neurological diseases state very important and wide question. At that time it is the most important elements in the treatment of those patients. In this review article the authors introduced some chosen forms and methods of motor exercises, physical procedures and some conduct physiotherapy principles patients with Parkinson’s disease, which should be realized as a main part of comprehensive treatment.

Keywords: morbus Parkinsoni, therapeutic treatment, rehabilitation, physiotherapy, quality of life
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Choreoacanthocytosis – case report
AKTUALN NEUROL 2010, 10 (2), p. 118-122
ABSTRACT

Choreoacanthocytosis or Levine-Critchley syndrome (MIM 200150) is a progressive multisystem disease with autosomal recessive transmission and a wide range of symptoms including: involuntary movements, lesion of peripheral nervous system, myopathy, behavioural and intellectual abnormalities, epilepsy, acanthocytosis and absence of any lipid abnormality. This disease is caused by mutation in VPS13A gene (CHAC) which is located on ch9q21. VPS13A gene spanning a 250 kbp region and consists of 73 exons. This gene encodes a large protein of unknown function, named chorein. Treatment for choreoacanthocytosis is aimed at the problematic symptoms of the sufferer but may not result in modification of natural history of this disease. Herein we report the case of a patient with positive familial history, choreiform movements in the arms, involuntary movements of the face and tongue, which was associated with vocalizations, dysarthria and dysphagia, progressing intellectual abnormalities, depression, clinical sign of peripheral nervous system lesion or/and myopathy, epilepsy with tonic-clonic fits and acanthocytosis. Creatine kinase was raised at – 387 U/l (N: 0-145 U/l) with LDH – 244 U/l (N: 80-240 U/l). Direct bilirubin and indirect bilirubin was slightly elevated. The MRI of the brain showed typical atrophy of the caudate nucleus (left) and seldom observed atrophy of hippocampus (right). As far as we know, it is the first observation of a patient with familial choreoacanthocytosis in Poland.

Keywords: choreoacanthocytosis, Levine-Critchley syndrome, acanthocytosis, involuntary movements, epilepsy, mental deficits
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Spontaneous intracranial hypotension – case report
AKTUALN NEUROL 2010, 10 (2), p. 100-104
ABSTRACT

The syndrome of spontaneous intracranial hypotension (SIH) was first described in 1938 and was considered a rare disorder. Because all of SIH occur secondary to spontaneous spinal cerebrospinal fluid (CSF) leak, the term: spontaneous spinal CSF leak is the preferred descriptive term. Recent evidence suggest that SIH is not rare but underdiagnosed. The spectrum of clinical and radiological manifestations is varied. The orthostatic headache and opening pressure of cerebrospinal fluid (CSF) under 60 mm H2O are cardinal diagnostic features. Treatment consist in bed rest, oral hydration, caffeine or theophylline intake, epidural blood patching, percutaneous fibrin glue injection or surgical CSF leak repair. The effectiveness of those treatment is limited and poorly studied. Author present case of 19-year-old woman with 2 days history of headache, tinnitus and photophobia. On neurologic examination patient presented only vertical diplopia. CT of head showed swelling of brain and contractions of cerebral ventricles. A lumbar puncture on the day after admission excluded neuroinfection and subarachnoid haemorrhage. The CSF opening pressure was 15 mm H2O. An MRI of cervical, thoracic and lumbar spine did not show any potential structures connected with CSF leak. Myelo-CT showed in lumbar region four places of CSF leak. This case demonstrates some of the potential diagnostic difficulties and unusual cause for sudden onset headache.

Keywords: spontaneous intracranial hypotension, spontaneous spinal cerebrospinal fluid leak, orthostatic headache, diagnosis, treatment
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