Factors predicting early stroke fatality in Poland. Preliminary report of the Polish National Stroke Registry.

Poland has one of the highest rates of death due to stroke in Europe, which, in contrast to many industrialized countries, has not changed since at least 1984. To improve this unfavorable situation, the entire approach to stroke management needs to be recognized. For this purpose, an analysis of stroke epidemiology regarding regional differences was one of the the strategic points of the Polish National Project of Stroke Prevention and Treatment. The Polish National Stroke Registry was maintained from 1 January to 31 December 2000 in 59 Neurological Department in all 16 districts of Poland. In total 11,107 patients were included: 11% with intracerebral hemorrhage, 63.4% with ischemic stroke, and 25.6% with unclassified stroke. Computed tomography (CT) was performed in 73.6% of patients. Analysis of in-hospital deaths showed great differences between the centers (from 8% to 36%). According to multifactorial analysis, not only well-known predictors of early death (decrease in consciousness at the onset of stroke, decrease in functional state prior to stroke, and severity of stroke) influence the prognosis. In centers with high risk of death, CT, especially CT on admission, was performed significantly less often (4.2% vs. 62.6%), early rehabilitation was delayed (38.3% vs. 73.4%), and secondary prevention treatment was prescribed to fewer patients (antiplatelettherapy 36.4% vs. 77.4%; antithrombotic therapy 4.9% vs. 13%).

[Clinical picture of spinocerebellar ataxia type I (SCA1)]. / Obraz kliniczny bezladu rdzeniowo-mózdzkowego typu I (SCA1).

Spinocerebellar ataxia is a group of diseases with autosomal dominant inheritance heterogenous both clinically and genetically. So called dynamic mutations underlie most these nosological units. The clinical patterns of various SCA types have not yet been defined completely. The purpose of the present report was description of the typical symptoms and signs of type 1 SCA. Seventeen patients from 13 families (M-2, F-15) were studied clinically in detail. The diagnosis was confirmed by DNA analysis. The assessment included neurological status, cognitive functions, the results of EEG, EMG, SEP, VEP, BAER and MRI examinations. The pedigrees indicated autosomal dominant inheritance pattern. The mean age at onset was 35.5 +/- 6.8 years (range 23-45 years) and it suggested negative correlation with the number of CAG repetitions. Cerebellar syndrome limb and truncal, ataxia and dysarthria was present in all cases. Six patients had nystagmus, 3 had slow saccades, 2 had gaze limitation upward, and lateral and 6 had dysphagia. Signs of pyramidal system involvement were found in 10 cases, one had athetotic movements, one had orthostatic hypotension. Two patients had dementia features, 9 had some decline of intellectual functions, mainly with difficulties of memorization, learning and concentration. In 16 cases MRI demonstrated vermis atrophy and atrophy of cerebellar hemispheres, 14 had fourth ventricle dilatation, 8 had flattening of pons base, 8 had narrowing of cervical spinal cord, 8 had dilated CSF spaces over frontal lobes and in 6 cases lateral ventricles were dilated. Electrophysiological peripheral nervous system investigations showed in 16 cases long-standing damage to the motor and sensory peripheral neurons at the level of nerve trunks, more pronounced in sensory nerves. In 13 cases peripheral neuron damage was subclinical. SEP showed in all patients disturbed function of ascending sensory pathways at peripheral and spinocortical levels.

[Autosomal dominant spinocerebellar ataxia]. / Autosomalny dominujacy bezlad rdzeniowo-mózdzkowy.

The modern classification is presented based on genetic criteria of the group of degenerative nervous system diseases inherited as autosomal dominant trait and called collectively spinocerebellar ataxia (SCA). They belong mostly to the class of diseases of similar mutation mechanism in which amplification is present of the trinucleotide sequence (CAG)n. Clinical picture and neuropathological changes in various SCA types are compared.

[Epileptic seizures in relation to the age of SSPE onset]. / Napady padaczkowe w zaleznosci od wieku zachorowania na SSPE.

The aim of this study is a clinical and electroencelographic analysis of those SSPE patients who suffered epileptic seizures in course of the disease. The material is based on an analysis of a computed database including 1180 case histories from multiple hospitalizations of 248 SSPE patients (141 males, 107 females) in years 1978-1995. The analysis was made using computer system EPI-INFO 6. The average age of SSPE onset was 12.3 +/- 4.5 (range 4-27.5). 74.2% of patients developed the disease before the age of 15. Epileptic seizures occurred in 43.5% (N = 108) of all SSPE patients in different stages of the disease. In 47 patients (M-21; F-26) they were observed among first SSPE symptoms, in 41 of those the seizures had the form of GTCS. Epileptic seizures were more frequent in patients with SSPE onset before the age of 15 (50.5%) than in patients with later onset (23.4%). More frequent occurrence of epileptic seizures and epileptiform changes in EEG in children under 15 can be associated with specificity of developmental age: immaturity of nervous and endocrine system and decreased convulsant threshold.

[Computerized analysis of the database of SSPE patients treated at the 1st Neurological Clinic of the Institute of Psychiatry and Neurology in Warsaw in 1978-1995]. / Analiza komputerowej bazy danych chorych na SSPE, leczonych w I Klinice Neurologicznej Instytutu Psychiatrii i Neurologii w Warszawie w latach 1978-1995.

Computer-assisted analysis was done of database containing 1180 records of 249 patients with SSPE hospitalized repeatedly at the I Dept. of Neurology using the EPI INFO 6 computer system recommended by the WHO. Boys were slightly prevalent in these cases (58%). The results of the analysis showed a falling incidence of SSPE among children born after 1976 (increased mean age of onset and death with successive years and hospitalization period, but without correlation between the mean age at onset and the birth year, decreased number of patients born after 1978 and absence of new admissions of those born after 1984). The falling incidence of SSPE can be a result of the introduction in 1976 of routine measles vaccination. 80.5% of the patients had measles, and 70.5% had it in the first two years of life (mean age to onset 2.34 +/- 2.32). Only 29 patients (11.6%) had had measles vaccination, and 15 of them (6%) had had measles.

[The application of graphic (visual) databases in neurology and neuropathology]. / Zastosowanie graficznych (wizualnych) baz danych w neurologii i neuropatologii.

The possibilities and principles of creation in Windows environment of visual databases are described which could be used for elaboration of multimedial encyclopedia of selected nosological entities. Such databases are particularly important in education making possible finding of data and their relative comparison. The method of organization of "files" using standard programmes of Windows packs and the method of application of the technique of image coding for forms of symbolic icons are presented. Examples of graphic bases of neurological and neuropathological data evolved in Windows environment using professional application programmes (framing and retouching of images in graphic editors, coding of icons of macroinstruction of reading of information introduced into the relating visual database of the analysed cases.

[Changes in NMR and CT images in SSPE]. / Zmiany w obrazach CT i MRI w przebiegu SSPE.

In 25 patients with subacute sclerosing panencephalitis in various phases of the disease CT and NMR imaging of the brain were done at the same times and the findings were related to clinical symptomatology. NMR imaging, in contrast to CT imaging, demonstrated even very small brain changes in the initial stage. The inflammatory-demyelinizing process begins in SSPE as a rule in the white matter of the occipital lobes, and only later in appears in the vicinity of the anterior horns of the lateral ventricles. Spreading of subcortical changes in the occipital lobes and their later penetration into the parietal lobes causes the development of ideatory apraxia frequent in these cases, and involvement of the cortex of the occipital lobes leads to visual agnosia. The paresis of extremities in later stages not always are correlated with greater intensity of changes in the contralateral cerebral with greater intensity of changes in the contralateral cerebral hemisphere. Hydrocephalus developing after longer duration of the disease is an expression of postinflammatory brain atrophy and not of disturbances in cerebrospinal fluid absorption.

[Selective problems from catamnestic studies of SSPE patients (1980-1989)]. / Wybrane zagadnienia z badan katamnestycznych pacjentów z SSPE (1980-1989).

The analysis of disease courses and survival times of 132 cases of SSPE treated by various methods in the I Department of Neurology, Institute of Psychiatry and Neurology, in years 1980-1989 is reported. Follow-up data were obtained during control examinations of patients or from an inquiry answered by parents or doctors at the place of residence of the patients. In the treatment all patients were given immunomodulating drugs. About 25% of the patients were treated with isoprinosine, in the remaining groups other drugs active on the immune system were added: TFX, alpha interferon (intraventricularly through a Rickham chamber), beta interferon (through lumbar tap) or Propionibacterium granulosum strain K 14 vaccine as an inductor of endogenous interferon. The reference group comprised 22 patients who had not been systematically treated for various reasons. The statistical analysis of the clinical courses showed that early begun immunomodulatory treatment increases the number of cases with remissions in the group of patients with the subacute form of the disease. In primarily acute cases no effect of the treatment was noted. The best results were obtained using Propionibacterium granulosum vaccine and treating with interferons. The survival times were shortest in the group not treated systematically.

[Hospitalization of patients with central nervous system neoplasms in 1979-1981 and 1986-1988]. / Hospitalizacja z powodu nowotworów ukladu nerwowego w Polsce w latach.

The hospitalizations and hospital deaths due to nervous system tumours were analysed on the basis of statistical cards filled in hospitals in a random sample of 10% of patients hospitalized in all Polish hospitals in the years 1979-1981 and 1986-1988. An evident rise was observed in the frequency of these hospitalizations and a less evident rise in the number of hospital deaths with a decrease of the hospital deaths caused by nervous system tumours. In the analysed time periods the greatest number of hospitalizations was in the age group 40-59 years, and deaths in the age group over 60 years. Hospitalization and hospital deaths indices were higher for urban population and males.

[Hospitalization of patients with cerebrovascular diseases in Poland in 1979-1981 and 1986-88]. / Hospitalizacja z powodu chorób naczyn mózgowych w Polsce w latach 1979-1981 i 1986-1988.

Hospitalization and death indices of patients admitted with cerebrovascular diseases were analysed on the basis of statistical cards filled by hospitals in a sample of 10% in patients in all Polish hospitals in the years 1979-1981 and 1986-1988. In these years a 40% rise was noted of the hospitalization indices and a 22% rise in the death rated caused by these diseases. On the other hand, the hospital death rate decreased from 32.2% to 27.9%. Higher hospitalization indices were found for males and for the urban population. The death rates and intrahospital mortality were higher for women but the standardized death rates, with the exception of oldest age group, were higher for men. A cause for concern is the rise of hospitalization indices for men and women and death rates in men aged 40-59 years which may indicate lowering of the age of threatening stroke.

[Comparison of the results of the treatment of patients with SSPE using various immunomodulating preparations]. / Porównanie wyników leczenia chorych na SSPE róznymi preparatami immunomodulujacymi.

The authors analysed the results of controlled treatment by three methods of patients in the 1st or 2nd phase of SSPE in a randomized group. The groups received: I. Propionibacterium granulosum KP-45 (interferon inducer) + isoprinosine, II--TFX (thymus extract) + isoprinosine, III--only isoprinosine. In all groups the treatment was continued during 6 months. The analysis of the clinical results of these methods failed to demonstrate a statistically significant superiority of any of these methods (among others, due to small number of cases) but an evident statistical tendency was revealed suggesting a better effectiveness of combined treatment (immunostimulator + isoprinosine) in relation to treatment with isoprinosine only. The final evaluation of the results requires further observations of these patients.

[Indicators of brain biogenic amine metabolism in various extrapyramidal disorders]. / Przemiana mózgowa amin biogennych w niektórych chorobach ukladu pozapiramidowego.

The authors describe the results of determinations of the main metabolites of dopamine (DA), serotonin (S) and noradrenaline (NA) in the cerebrospinal fluid (ventricular and lumbar) in patients with various extrapyramidal system diseases. A profound decrease was demonstrated in the concentration of homovanillic acid (HVA)--the end metabolite of DA in parkinsonism, reflecting damage to DA--containing pathways and reduced DA synthesis in basal ganglia. Treatment with L-DOPA raises considerably the HVA level in the cerebrospinal fluid evidencing increased DA metabolism in the brain during administration of its precursor L-DOPA. In torsion dystrophy a statistically significant difference was found in HVA concentrations in the ventricular fluid depending on the clinical manifestations of the disease. In the patients with local muscular rigidity HVA level was much lower than in patients with the hyperkinetic form of the disease. It is concluded that the character of changes in the cerebral dopaminergic systems differs phenotypically in the form of torsion dystonia. In hepatolenticular degeneration (Wilson's disease) the level of all studied metabolites was decreased, which could be an evidence of deficient cerebral metabolism of their precursors--amines. In cases of Huntington's chorea a low level of HVA was found in the ventricular fluid, reflecting decreased total amount of DA in the brain due to damage to the corresponding neurons. Absence of detectable changes in MHPG concentration (the main cerebral metabolite of NA) indicates that this amine plays a lower role than DA and S in the biochemical mechanisms of the pathogenesis of extrapyramidal motor disturbances. The obtained data are important for the understanding of the pathogenesis and for evolving therapeutic methods in extrapyramidal diseases.