Metachromatic leukodystrophy in adult patient initially diagnosed as multiple sclerosis

The article presents a case of metachromatic leucodystrophy (MLD) of the adult onset type, in which atypical initial course could suggest multiple sclerosis. MLD is an inherited, metabolic, degenerative disease of the nervous system. It is caused by a deficiency of a lysosomal enzyme--arylsulphatase A, with the storage of cerebroside sulphate and demyelination affecting mainly the central and peripheral nervous system. In the reported case of a female aged 21 the clinical onset of the disease was at the age 20. The first neurological symptoms included a mild spastic paraparesis, ataxia of lower limbs, and imbalance. The neurological deterioration has been progressing since the onset. There was no family history of neurological or metabolic diseases, but the patient's parents were near relatives (first cousins). T-2 weighted MRI showed high intensity areas in the white matter, mainly periventricular. The clinical symptoms could suggest multiple sclerosis (of the primary progressive type). The patient was admitted to our Dept. in Oct. 2000, i.e. about a year since the onset. Her increasing spastic triparesis, cerebellar ataxia, neuropathy in lower limbs and cognitive deterioration were confirmed. Re-analysis of the patient's clinical picture and family history, her MRI scans and repeated neurophysiological examinations which demonstrated a damage of the central and peripheral nervous system, suggested metachromatic leucodystrophy. Therefore, it was decided to assay arylsulphatase A activity. The result confirmed the diagnosis of the late onset MLD. Additionally, molecular genetic examination was performed of a DNA sample obtained from the patient.     

Catecholaminergic activity of the baroreceptor areas of the brain in response to bilateral dorsolateral transection of medulla oblongata in rats

Central catecholaminergic activity was studied by measurement of norepinephrine (NE), epinephrine (EPI), their metabolites: total 3-methoxy-4-hydroxyphenylglycol (MHPG) and 3,4-dihydroxyphenylglycol in brain nuclei in response to bilateral dorsomedullary knife-cut (DMK-cut) in rats. In saline-pretreated conscious rats DMK-cut caused a marked hypertension, tachycardia and increases in plasma NE, EPI and vasopressin. Chlorisondamine (CHL) prevented the rise in plasma catecholamines and the tachycardia but failed to prevent the hypertension and the increment in plasma vasopressin. DMK-cut decreased NE and EPI in the nucleus tractus solitari (NTS) and A2 area; there were no catecholamine changes in A1 area or the periventricular nucleus but an increase in the paraventricular nucleus (PVN), the latter effect reversed by CHL, CHL alone or combined with DMK-cut had no effect on catecholamine concentrations in NTS-A2 area but lowered MHPG content. It is suggested that DMK-cut decreases the activity of the catecholaminergic system originating in A1 and terminating in PVN, where it causes catecholamine accumulation and may be involved in vasopressin release and thereby contribute to hypertension. In NTS-A2 area, however, the DMK-cut appears to increase catecholaminergic activity since catecholamines are depleted. Central effects of DMK-cut differ from those of ganglionic blockade-induced inhibition of the baroflex presumably due to sectioning of other pathways in addition to the primary baroreceptor input.     

Comparison of the FIGO and TNM staging systems for uterine cervix cancer based on classification of 6193 cases

A total of 6193 patients treated for uterine cervix cancer was studied by a collaborative study, aiming to compare the applicability of the TNM versus FIGO clinical staging systems. Retrospective assays of gynecological status were performed. It was found that the FIGO classification system is superior to the TNM system concerning uterine cervix cancer in the aspects of simplicity, recording procedures, and prognostic value.     

Inhibition of plant and mammalian diamine oxidase by substrate analogues

Imidazoles, aliphatic substrate analogues and the natural dipeptides, carnosine and anserine, were investigated as inhibitors of diamine oxidase from the pig kidney, human pregnancy plasma and pea seedlings. Imidazole, methyli-midazoles,N-acetylimidazole, histamine andN -methyl-histamine are relatively potent inhibitors of mammalian diamine oxidase showing no influence on plant enzymes. Anserine and carnosine are inhibitors of pig kidney and pea seedling enzymes.K ₁ values are 2 M and 10 M respectively. Investigated natural derivatives of putrescine and cadaverine have no influence on diamine oxidase of different origin.In conclusion, we present some evidence to suggest that mammalian diamine oxidase, despite a high reaction rate with putrescine, is better adapted to histamine oxidation, whereas for plant enzymes the diamines are preferred substrates.     

The protective action of alpha-tocopherol on the white matter lipids during moderate hypoxia in rats

Hypoxia and ischemia acting on the brain cause alterations of the level of lipids and sterols. Famile 3.0-3.5-month-old rats were used for the experiment. They were given alpha-tocopherol in the dose of 11.43 mg/kg of body weight through seven days, then underwent hypoxia (7% of oxygen in the breathing mixture) and myelin was isolated in four times after experiment: 4, 24 hours, 14 days and 2 months after experiment. Three lipids groups were isolated that are neutral lipids, galactolipids and phospholipids. They were quantitatively analyzed with spectrophotocolorimetry. The obtained results indicate that vitamin E administration to animals does not cause significant changes of brain lipids levels. However, alpha-tocopherol administred before moderate hypoxia balances the concentrations of lisophosphatidylcholine and phosphatidylinositide and cerebrosides with control level 2 months after experiment. Vitamin E changes in concentration of the myelin neutral lipids. Vitamin E administered before experimental moderate hypoxia stabilizes some membrane lipids and could be used in brain hypoxia.     

Quantitative assessment of mRNA TGF-beta1 in liver tissue in connection with serum mean daily level of TGF-1 in chronic hepatitis B patient

Monitoring of fibrosis process with the use of histopathologic studies on liver's bioptates is limited, so it is attempted to find reliable, obtained with less invasive methods, sensitive and reflecting fibrosis dynamics markers of this process. The aim of the study was simultaneously to assess liver's expression as well as circadian and mean daily TGF-betal concentration in serum of patients with chronic hepatitis type B in comparison to control group. Studies were performed on 50 patients (9 women, 41 men) aged 45.9 +/- 2.3 years with chronic hepatitis type B. Control group consisted of 20 patients (mean age 38.6 +/- 3.7 years), in which so called minimal changes without fibrosis were observed in histophatologic bioptate of liver. Blood for studies was collected every 4 houres during the day. Serum concentration of TGF-betal was assessed with the use of ELISA method, and expression of mRNA TGF-betal in liver with QRT-PCR method. No significant difference between circadian as well as mean daily serum TGF-betal concentration beetwen control group and the group with chronic hepatitis type B was shown. Increased expression of mRNA, TGF-betal in bioptate of liver of patients with chronic hepatitis type B in comparison to control group was noted. In "minimal changes" control group presence of significant positive correlation between expression of mRNA TGF-beta1 in liver and concentration of this cytokine in serum was shown, in the group of patients with chronic hepatitis B this connection was not noted.     

Range and ecology of Dermacentor reticulatus (Fabricius, 1794) in Mazuria focus. III. The influence of temperature and day length on the activity of hungry adult ticks in laboratory conditions

The influence of day length and temperature on the activity of adult Dermacentor reticulatus was studied in two experiments. In the first one, changes in the activity of ticks kept in three aquariums were observed where the differentiating factor was day length (14, 15.5 and 17 h). In all of the three variants the activity of ticks decreased over time. The activity of ticks kept in the conditions of a 17 h day decreased the fastest; the decrease was a little slower in the conditions of a 15 h day, and in the conditions of a 14 h day it was the slowest. In the second experiment, changes in the activity of ticks kept in the conditions of a long (20 h) and short (10 h) day was observed, with temperatures differentiated between 18 degrees and 25 degrees C. The activity of hungry adult ticks was most limited in the conditions of a long (20 h) day and high temperatures (25 degrees C), while it decreased somewhat more slowly in the conditions of a long day and moderate temperatures (18 degrees C). An even slower decrease in their activity was observed when day length was reduced to 10 h; it was more marked in temperatures around 25 degrees C than 18 degrees C.     

Poly(vinyl alcohol) membranes for adhesion prevention

The abnormal joining of anatomic structures after abdominal and pelvic surgery can lead to such major complications as bowel obstruction or infertility. Poly(vinyl alcohol) (PVA) membranes and hydrogels were placed over the injured tissue to act as a physical barrier and prevent such adhesions from occurring in a rabbit sidewall model. The membranes were sutured into place to prevent their slipping or curling on the moist tissue. Various in vitro experiments (including testing for swelling and mechanical strength) were conducted in order to better understand the behavior of these membranes in the wound. The results showed that both the PVA membranes and PVA hydrogels significantly reduced the number and severity of adhesions in the rabbit sidewall model, and even indicated a distinct improvement over SEPRAFILM as antiadhesion barriers. Contact-angle measurements were taken in order to evaluate the surface properties of the membranes and hydrogels. Three approaches were taken to render the membranes more bioadhesive, and forego the need for future additional suturing: imprinting a texture onto the membrane, coating the membrane with carboxy methyl cellulose (CMC), and producing bi-layered, porous PVA membranes through a process of lyophilization. Though the surface of the PVA hydrogels is more hydrophilic than the surface of the PVA membranes, neither would adhere untreated to moist tissue. However, all three approaches aimed at improving their bioadhesion yielded excellent results and demonstrated that PVA could indeed be considered a viable method of adhesion prevention.