[Progress in the studies on the pathogenesis, clinical course and treatment of extrapyramidal disorders].
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Biomedical subjects
Publications and source records attributed to J Kulczycki.
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The natural outcome of SSPE which is as a rule unfavourable independently of the treatment given, and this indicates the necessity of studies on new methods of treatment. After failures of steroids, immunosuppressants and transfer factor the treatment with virostatic drugs seems to be most safe and justified. The paper contains preliminary results of clinical observations of 34 patients with SSPE treated with amantadine, ITF (a derivative of isothiazole) and isoprinosine. In some cases it was tried to correlate the results of treatment with those of immunological and electrophysiological investigations. The presented data are insufficient for a reliable conclusion to be drawn concerning the effect of various drugs on the course of SSPE, nevertheless, it seems that the best results with slowing down of the progression of the disease were observed in the group treated with isoprinosine.
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In the light of pneumoencephalographic investigations and radioisotope scinticysternography carried out in 22 patients with clinical manifestations suggesting the syndrome of Hakim the authors analyse the diagnostic usefulness of both methods. In all cases PEG demonstrated dilatation of the ventricular system, in 10 cases radioisotope cisternography showed abnormal passage of the cerebrospinal fluid, and in 8 of these cases ventricular reflux was found lasting at least 24 hours, while in 2 other cases absorption of the cerebrospinal fluid was impaired. On the basis of this material the authors believe that in cases of presence of ventricular reflux this investigation is very important for undertaking the decision of surgical treatment, besides that, the authors think that despite normal scintigraphic pattern in early stage of the disease the investigation should be repeated after from 10 to 20 months, since clinical symptoms may precede disturbances of cerebrospinal fluid passage detectable in cisternographic investigation.
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Laser spectrography was used for analysis of cations in vascular and tissue deposits in a case of Fahr's syndrome. The following cations were found: calcium, iron, silica, magnesium, aluminium, copper, zinc, phosphorus. In the tissue outside the deposits only magnesium, iron and aluminum were found, and the degree of blackening of spectral lines was much weaker. The authors think that these observations could contribute to a better knowledge of the aetiology and pathogenesis of Fahr's syndrome.
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A case of multisymptomatic brain stem injury, possibly during subacute viral infection, in a 27-year-old man is reported. Two MR imaging at the time of regression of disease symptoms demonstrated in the medulla stabilized lesions of the type of central myelinolysis without damage to the corticospinal pathways. During four years of follow-up the neurological status was improving gradually, but involuntary movements of the mandible and face muscles make speech difficult.
A case of symmetric "calcifications" in the white matter of cerebral hemispheres, basal ganglia and cerebellum is reported in a patient without parathyroid function changes and calcium-phosphorus metabolism disturbances (Fahr syndrome). Attention is called to differences in the extensiveness of these deposits in CT and MRI examinations. The cause of false magnification of the outline of calcifications in CT images is discussed.
The report is a trial of tracing of the evolution of traumatic spinal cord lesions in patients with trauma to the cervical spine and cord. The results of the initial MRI studies (immediately after injury) were compared with those carried out later.
The authors present the results of an epidemiological study of SSPE in Poland conducted since 15 years. Since the years covered by the study (1977-1991) included the first period after the introduction of obligatory vaccinations against measles in our country, the most important aim of this analysis was to find out the possible influence of the effect of these vaccinations on SSPE incidence. A comparison of the data obtained in successive stages of the study shows that a slow gradual decrease of the annual incidence of SSPE has been taking place in our country (from at least 1.6 per million before 1983 to 0.69 in 1991). The effect of the vaccinations is visible also in a significant increase of the mean age of SSPE onset which is a result of ever more widespread vaccination of the youngest children. These observations indicate unequivocally that the aetiological factor in SSPE is a wild strain of measles virus and not that used for vaccinations.
The effect of Ergolaktyna (bromocriptine) produced by Grodzisk Pharmaceutical Works POLFA on the intensity of parkinsonism manifestations was studied in 27 patients in four clinical centres. All patients had moderately advanced parkinsonism and prior to Ergolaktyna they received L-Dopa preparations which were also given concomitantly with that preparation. The final assessment was done after 4 months of treatment in 18 cases. A good effect of the drug was found in 55% of cases (reduction of the intensity of parkinsonian signs, increased fitness and life activity). Adverse effects developed in 4 cases and required drug withdrawal.
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In three neurological centres the therapeutic effects and adverse effects were compared of two levodopa preparations combined with carbidopa (25/250): 1. A foreign drug widely prescribed in Poland, and 2. Its Polish analogue produced by Polfa Stargard Works. The drugs were given to 30 patients (in two matched groups) with Parkinson disease or syndrome during 6 weeks. The trial was completed by 28 patients (one patient dropped out from each group for reasons not directly connected with the treatment). The therapeutic results were assessed on the basis of effects obtained in the NUDS and NSP scales and by measuring of the "on" phase at the beginning and at the end of the trial. In every case the global assessment of patient's condition by the patient and by the doctor was considered. The analysis of the results showed no significant statistically differences between both drugs respect to antiparkinsonian action and incidence of adverse effects.
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.