[Amanita pantherina and Amanita muscaria poisonings--pathogenesis, symptoms and treatment]. / Zatrucia muchomorami plamistym i czerwonym--patogeneza, objawy leczenie.

Amanita pantherina and Amanita muscaria are commonly occurring mushrooms in Polish forests. They contain ibotenic acid and muscimol: the substances reacting with neurotransmitter receptors in central nervous system. The ingestion of these mushrooms produces a distinctive syndrome consisting of alternating phase of drowsiness and agitation with hallucinations, and sometimes with convulsions. The diagnosis of Amanita pantherina or Amanita muscaria poisoning is established by means of mycologic investigation of gastric lavage. The treatment is only symptomatic, and the prognosis is usually good.

Ataxia telangiectasia heterozygotes and patients display increased fluidity and decrease in contents of sulfhydryl groups in red blood cell membranes.

In red blood cell membranes of ataxia telangiectasia mutated (ATM) homozygotes and heterozygotes, decreased values of the corrected fluorescence anisotropy and the anisotropy parameter were found, indicating increased fluidity and decreased microviscosity, respectively. These changes in membranes were accompanied by a decrease in SH-groups and an increase in malondialdehyde (MDA) contents. The content of MDA both in homozygotes and in heterozygotes exceeded roughly threefold the respective control values. Decreased content of GSH in red blood cells was found only in ATM homozygotes. The change most specific for the ATM gene appears to be the increase in fluidity, since only this parameter displays the proportionally greater changes in ATM homozygotes compared to ATM heterozygotes. The observations presented here may indicate that the ATM gene is expressed in precursors of red blood cells and deficiency of normal AT gene function may produce the changes which persist in circulating cells.

[Poisoning with spotted and red mushrooms--pathogenesis, symptoms, treatment]. / Zatrucia muchomorami plamistym i czerwonym--patogeneza, objawy, leczenie.

Amanita pantherina and Amanita muscaria are commonly occurring mushrooms in Polish forests. They contain ibotenic acid and muscimol: the substances reacting with neurotransmitter receptors in central nervous system. The ingestion of these mushrooms produces a distinctive syndrome consisting of alternating phases of drowsiness and agitation with hallucinations, and sometimes with convulsions. The diagnosis of Amanita pantherina or Amanita muscaria poisoning is established by means of mycologic investigation of gastric lavage. The treatment is only symptomatic, and the prognosis is usually good.

[Evaluation of acceptance rate and outcome of renal replacement therapy in patients with diabetic nephropathy--multicenter study]. / Ocena dostepnosci i wyników leczenia nerkozastepczego u chorych z nefropatia cukrzycowa--obserwacje wieloosrodkowe.

Development of dialysis methods and progress in kidney and pancreas transplantation allowed to treat an increasing number of patients suffering from diabetic nephropathy (D.N.). This report evaluates availability and results of treatment in these patients. 31.12.93 in Gdansk and Bydgoszcz area there were treated 519 patients, including 43 (8.2%) with D.N. It is impossible to evaluate the demand for renal replacement therapy in patients with D.N., because there is no exact data concerning diabetic patients with progressing renal failure. Up to now 88 patients with D.M. (68 with IDDM, 20 with NIDDM) were treat in this area. Most of them (92%) were treated with hemodialysis is and only a few with CAPD, 13 patients received a kidney graft. The average patient survival on dialysis treatment in NIDDM patients was 15 months and in IDDM patients was 11 months. Deaths were mainly caused by cardiovascular complications. The results of renal replacement therapy in these patients cannot be compared with data from other re ports, because the treatment was introduced at advanced stage of D.N. in patients with systemic complications (serum creatinine in IDDM was 9.7 md% and in NIDDM was 6.2% mg%). Following conclusions can be drawn from our observations: 1. There is a need for close cooperation between diabetologist and nephrologist in repeat of evaluation of the demand for renal replacement therapy and time for its institution in a particular patient. 2. The choice of method of renal replacement therapy depends on clinical findings in a particular patient but also on methods available in a particular center. 3. Improvement of therapy outcome can be achieved primarily by earlier institution of dialysis (serum creatinine below 5 m5%).

[Extrapyramidal diseases in children--clinical problems regarding etiopathogenesis and epidemiology]. / Choroby ukladu pozapiramidowego u dzieci--problemy kliniczne, etiopatogenetyczne i epidemiologiczne.

Clinical picture, etiopathogenesis and epidemiological analysis were performed on the extrapyramidal diseases of the children. Despite of advances in neurology and on the own observations etiopathogenesis of these syndromes remains unexplained. The abnormalities neurotransmitters of the brain is the main cause abnormal function of the basal ganglia. The various categories extrapyramidal syndromes are known, but a variety of intermediate forms have been described. The diagnosis extrapyramidal diseases in children despite recent advances in neurology and genetic remains very difficult.

[Analysis of adverse factors affecting the result of therapy for West syndrome in children]. / Analiza czynników niekorzystnie wplywajacych na wyniki leczenia w zespole Westa u dzieci.

West syndrome is a from of epileptic attacks of infants. Diagnosis of West syndrome includes: presence of the sudden violent flexion of the trunk and limbs, psychomotoric development retardation, especially after the onset of attacks, abnormal EEG records, and therapeutical problems. Clinical course and results of therapy were analysed in 66 children with West syndrome (39 boys and 27 girls). Children were divided into four groups, depending on etiology of the disease. Group 1 included 39 children with lesions to CNS during pregnancy; group 2-8 children with developmental CNS disorders, group 3-6 children with a history of encephalitis or meningitis, and group 4-13 children in whom etiology of West syndrome was unclear. Patients were treated with Synacthen-Depot in a daily dose of 0.03 mg/kg combined with other anti-epileptic agents. The most difficult to treat were those children in whom West syndrome occurred below 6 months of life, were psychomotor retarded before the onset of symptoms, hormonal treatment was introduced with delay, there were additional seizures of different etiology, and there were frequently recurrent infections.

[Lennox-Gastaut syndrome--clinical course and therapy]. / Zespól Lennoxa-Gastauta--obraz kliniczny i postepowanie terapeutyczne.

Clinical course and results of therapy were analysed in the group of 92 children, aged between 3 and 9 years, with diagnosed Lennox-Gastaut syndrome. The obtained results of an analysis have shown that Lennox-Gastaut syndrome origin is not clear--causative factor can not be established in 1/3 of patients whereas in 1/2 of them abnormal course of pregnancy and perinatal period is noted. Together with seizures of various origin, other focal neurological symptoms, mental retardation and abnormalities in CT scans of the brain are frequently seen in patients with Lennox-Gastaut syndrome. Clinical course, prognosis and results of therapy are largely dependent on the degree of mental development before the onset of epileptic seizures, course of pregnancy and perinatal period, and the time of therapy. Children with Lennox-Gastaut syndrome require relative polytherapy in which valproic acid derivatives are predominating together with benzodiazepines, and temporary corticosteroids. An improvement was achieved in about 30% of the treated children. Prognosis in the remaining 70% of children is rather poor. Irregular administration of drugs, frequent changes of anti-epileptic agents, too low doses and abnormal environmental effects (abnormal parental attitudes) affect the results of therapy. An emphasis is on the poor prognosis in Lennox-Gastaut syndrome proceeded with West syndrome.

[The examination of mental status of children with multiple sclerosis]. / Ocena stanu psychicznego u dzieci ze stwardnieniem rozsianym.

Assessment was conducted on 50 children with multiple sclerosis ranging in age from 3 to 16 years. The aim of the study was to the discover how they deviate from the norm in their mental status. It was concluded that the most frequent symptoms in this group of children, were various disorders in the impulsive--emotional sphere. It was noted that with time the chance of organic pathology increases.

Unaltered cell proliferation rate of the ataxia telangiectasia lymphocytes dividing in vitro.

Lower cell density was found in cultures of ataxia telangiectasia lymphocytes as compared to control lymphocyte cultures. This fits with the earlier observations of decreased incorporation of 3H-Thymidine into lymphocytes of ataxia telangiectasia (AT) patients. However, the finding that there was no significant difference in proliferation rate index between AT and control cultures was unexpected. This may indicate that both cell count and measurements of thymidine incorporation in lymphocyte cultures characterize cell populations distinct from that of proliferating cells.

Progressive peripheral neuron degeneration in ataxia-telangiectasia: an electrophysiological study in children.

Electrophysiological examinations were performed on 32 children aged three to 17 years who had typical clinical manifestations of ataxia-telangiectasia (AT). EMG findings demonstrated neurogenic lesions, more pronounced in the distal leg muscles of older children where they resembled the picture characteristic of motor neuron disease. Electrophysiological and nerve conduction results showed that generalised, progressive, sensory nervous system degeneration, with neurogenic amyotrophy affecting the distal part of the lower limbs, is an established feature of this disease and can be considered one of the diagnostic characteristics of AT. This allows the syndrome to be classified as an hereditary spinocerebellar degeneration.

Increased frequency of chromatid breaks in lymphocytes of heterozygotes of ataxia telangiectasia after in vitro treatment with caffeine.

The frequencies of caffeine-induced chromosomal aberrations (CA), mainly chromatid (CdB) and chromosome (CB) breaks, were studied in lymphocyte cultures derived from 6 obligatory heterozygotes and 1 homozygote of ataxia telangiectasia (AT), and from 4 control adult healthy persons. Caffeine (CF, 1 mM) was added at the beginning of the culture. In control cultures exposed to CF the frequency of CB was 1.9% and of CdB 1.3%. In cells of the AT homozygote, the frequency of CdB was 6.8% in the absence and 8.7% in the presence of caffeine, the frequencies of CB being 3.4 and 10.9%, respectively. In AT heterozygous cells treated with CF, CdB increased 13-fold as compared to a less than 3-fold increase in control cells. Comparing the frequencies of CF-induced chromosomal lesions in control and AT heterozygous cells, potentiation factors (Pf) for the effect of 1 AT gene on cell sensitivity to CF (Pf [AT]) were 3.5 for CB, 6.6 for CdB and 5.5 for CA. These data demonstrate that lymphocytes of AT heterozygotes are significantly more sensitive to caffeine treatment in vitro in terms of increased frequency of CdB than normal cells, which may be useful for the diagnosis of carriers of this defective gene.

Carriers of ataxia-telangiectasia gene display additional protein fraction and changes in the environment of SH groups in erythrocyte membrane.

Additional protein fraction migrating slower than spectrin has been detected in erythrocyte membranes from an ataxia-telangiectasia (A-T) patient and from his mother (A-T heterozygote). In erythrocyte membranes labelled with maleimide spin label changes in signal of the weakly immobilized spin label as related to that of strongly immobilized one (w/s) were noted. In comparison to age-matched control groups the values of w/s were lower in A-T heterozygotes (ten persons) and higher in A-T homozygotes (four persons). In control persons the values of w/s increased with age, whereas in families with A-T no significant differences in this parameter were noted between children and parents. The presence of additional protein fraction in erythrocytes membranes of A-T patient and A-T heterozygote indicates that these phenotypes can be differentiated from the healthy control persons for the first time on the basis of changes detected in the erythrocytes. This change in erythrocyte membrane may explain the decrease in the w/s parameter of electron spin resonance in A-T heterozygotes. On the other hand increased values of w/s in A-T patients may be caused by disease process.

[The Landau-Kleffner syndrome]. / Zespól Landau-Kleffnera.

The variable aetiology and complicated pathogenesis of aphasia during epilepsy in children cause many diagnostic and therapeutic difficulties. The authors describe two children with aphasia and epileptic seizures and with high titre of toxoplasma antibodies. The role of toxoplasmosis in this syndrome is discussed. The small number of publications on the Landau-Kleffner syndrome in the available literature, especially Polish, is stressed.

[Results of using Dorsiflex in various neurologic diseases in children]. / Wyniki stosowania dorsiflexu w niektórych chorobach neurologicznych u dzieci.

Therapeutic results are described obtained with the preparation Dorsiflex produced by the Yugoslav institution Lek Ljubliana. The drug was given to 40 children aged 8 to 14 years with various neurological diseases with muscle hypertonus as the prevailing sign. The preparation was found to exert a good myorelaxant effect, facilitating comprehensive rehabilitation in cases in infantile cerebral palsy, after craniocerebral injuries, encephalomeningitis, degenerative diseases and other conditions. The drug was well tolerated.

Landau-Kleffner syndrome--epileptic aphasia in children--possible role of toxoplasma gondii infection.

The association of unusual types of aphasia and epilepsy (Landau-Kleffner syndrome) has been described in three children. The children were 5 to 11 years of age and all have EEG abnormalities. Speech disorders appeared after epileptic seizures. No organic causes of the disease have been found in axial computed tomography. In all described cases the increased titer of IgG antibodies against Toxoplasma gondii has been observed. The authors suggest that the Toxoplasma gondii infection should be taken into account as a possible cause of Landau-Kleffner syndrome.