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PubMed1990

Mononuclear leucocytes, granulocytes and thrombocytes in the calcium ionophore-induced leucocyte adherence inhibition in multiple sclerosis patients and controls.

The calcium ionophore A23187-induced adherence inhibition of mononuclear leucocytes and granulocytes in multiple sclerosis patients and in controls was investigated. Furthermore, the role of thrombocytes in the ionophore-induced adherence inhibition of both the mononuclears and the granulocytes was evaluated. In the control group and in patients treated with corticosteroids the ionophore consistently induced the adherence inhibition of mononuclear leucocytes. In multiple sclerosis patients without corticosteroid treatment rather heterogenous results were obtained, which appears to reflect the fluctuation of the disease activity. Addition of thrombocytes to the mononuclear leucocyte suspension led to a decrease of leucocyte adherence in both multiple sclerosis groups regardless of the presence of the ionophore. The ionophore stimulation of granulocytes preincubated with the thrombocyte suspension resulted in an increase of the granulocyte nonadherence in healthy controls. An analogous response in multiple sclerosis patients without corticosteroid treatment was significantly less expressed and was not found in patients treated with corticosteroids. These results suggest a thrombocyte-dependent alteration of the membrane properties in the early phase of nonspecific granulocyte activation in patients with multiple sclerosis.

Adrenal Cortex Hormones↗
PubMed1990

[Retinal periphlebitis and multiple sclerosis. Incidence, cerebrospinal fluid and results of visual evoked potentials].

In a retrospective study 323 patients with multiple sclerosis were screened for the incidence of retinal periphlebitis. The results of lumbar puncture and the visual evoked potentials were related to the eye disease. Retinal periphlebitis was seen in 19 patients (5.9%). Of the patients with retinal periphlebitis, 79% developed the eye pathology within the first 10 years of multiple sclerosis. The cerebrospinal fluid and visual evoked potentials were no different than the results in a control group of multiple sclerosis patients without periphlebitis. Multiple sclerosis should always be considered as one of the potential underlying diseases in retinal periphlebitis since it is found in 30% before or at the same time as the first neurological symptoms of multiple sclerosis.

Adult↗
PubMed1990

[Brainstem auditory evoked potentials in patients with multiple sclerosis--use of 2 stimulation frequencies].

The authors examined Brainstem acoustic evoked potentials at different frequencies of repeated clicks (10, 15, 20, 30 and 50 Hz) in 30 healthy subjects (18 men, 12 women, age 12-50 years) and in a group of 89 patients with the diagnosis of multiple sclerosis (28 men, 61 women, age 16-55 years). Examination of the control group revealed that an increase of the stimulation frequency from 10 to 50 Hz leads to a significant prolongation of the absolute latencies of BAEP waves (in particular V wave), as well as inter-peak latencies (in particular I-V) and to a reduction of the amplitude of waves I and V, which was, however, significant only in wave I. The differentiability of some waves, in particular late ones was reduced. For these changes a zone of normal values was assessed. In patients with multiple sclerosis at a stimulation frequency of 10 Hz the most frequent abnormality of BAEP was a prolongation of the latency of the V wave and of I-V resp. At a stimulation frequency of 50 Hz the ratio of responses with undifferentiable waves V or III and V increased. The increase of the stimulation frequency from 10 Hz to 50 Hz led to a higher detection rate of abnormal BAEP findings in the whole group from 31% to 51% (p less than 0.001), in the sub-group of patients with a clinical lesion of the stem from 43% to 65% (p less than 0.01), in the sub-group without a clinically obvious lesion from 23% to 40% (p less than 0.01). In the sub-group of patients with a suspect diagnosis of multiple sclerosis, but without a clinically obvious symptomatology of the stem the detection rate of abnormal findings increased from 27% to 35% (NS). Based on their results, the authors are in favour of those views reported in the literature which consider the application of two stimulation frequencies during BAEP examinations useful for diagnostic purposes and recommend its inclusion in routine BAEP examinations of patients with multiple sclerosis, or suspect multiple sclerosis.

Acoustic Stimulation↗
PubMed1989

[Renal cell carcinoma associated with tuberous sclerosis: a case report].

Renal angiomyolipoma associated with tuberous sclerosis is well known. On the other hand, few cases of renal cell carcinoma in connection with tuberous sclerosis have been reported. We report a patient with tuberous sclerosis whose kidney was involved with renal cell carcinoma. A 18-year-old woman was first admitted in August 1987 for evaluation of left renal tumor. Diagnosis of tuberous sclerosis was made when she was 11 years old on the basis of mental retardation, papules on her face, seizures, white leaf-shaped macules and periventricular calcifications. Computerized tomographic scan demonstrated a large mass arising from the left kidney and small masses in the right kidney. Angiography confirmed bilateral hypervascular renal tumors. On these bases, a clinical diagnosis of bilateral renal angiomyolipomas was made and surgical treatment of the left kidney was recommended because of its large size. However, her parents did not permit treatment until March, 1988. Finally, left nephrectomy of 4,750 g was performed and histological examination revealed renal cell carcinoma with clusters of spindle cells. In the literature available to us, we found twelve reports of malignant renal tumors associated with tuberous sclerosis including five renal cell carcinomas in Japan.

Adolescent↗
PubMedDate not supplied

[ACTH in the plasma and cerebrospinal fluid in patients with multiple sclerosis].

In 1984 and at the beginning of 1985 the authors carried out radioimmunoassays (SORIN-CIS kit) the plasma levels of ACTH in 116 multiple sclerosis patients (m-52, f-64) and in 10 cases this radioimmunoassay was done in the cerebrospinal fluid (m-5, f-5). The control group comprised 90 patients with ischialgia and neuroses. The normal value in the plasma was from 0 to 80.86 pg/ml, and in the fluid it was from 0 to 77.08 pg/ml. In multiple sclerosis patients the plasma ACTH level was from 0 to 286.9 pg/ml, in the cerebrospinal fluid from 0 to 89 pg/ml. The values of ACTH were significantly higher in multiple sclerosis patients, mainly in males. In the fluid the level of ACTH was significantly higher in the studied patients. No significant differences in ACTH levels were found between males and females with multiple sclerosis, and in the control group this level was higher in females. Raised ACTH level was found mainly in multiple sclerosis with lung duration of the disease (10 years) at the time of exacerbations. The authors continue studies on the axis hypothalamus-hypophysis-adrenals, on various hormones, prostaglandins, beta-endorphin, biochemical markers, cAMP, cCMP, arylosulphatase A and B MBC etc.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

[Value of polyacrylamide gel electrophoresis of non-concentrated cerebrospinal fluid in the diagnosis of multiple sclerosis].

Using SDS-PAGE for non-concentrated cerebrospinal fluid investigations connected with immunofixation and silver impregnation of gels the authors found oligoclonal IgG in 39 cases (95%) of clinically reliably diagnosed multiple sclerosis, and in 92.3% of probable multiple sclerosis cases. The number of IgG lines reached 13, while in the control group of neuroses it never exceeded 6-8. Comparative analysis of the IgG spectrum in cerebrospinal fluid and serum in the same multiple sclerosis cases demonstrated different IgG patterns which were only partly similar. Moreover, it was found that the electrophoretic pattern of cerebrospinal fluid proteins in each case of multiple sclerosis had an individual peculiar pattern of Ig spectrum. The use of SDS-PAGE in combination with silver impregnation made possible obtaining of qualitatively very good separation in 10-20 microliters samples of non-concentrated cerebrospinal fluid. The silver impregnation method is 50-100 times more sensitive than the traditional staining of gel with Coomasie Blue R-250. In multiple sclerosis SDS-PAGE of non-concentrated cerebrospinal fluid combined with silver impregnation of gel is a very god diagnostic method.

Adolescent↗
PubMed1977

Early perivenular sclerosis in alcoholic fatty liver: an index of progressive liver injury.

Alcoholic steatosis was associated with sclerosis around the terminal hepatic venules in liver biopsies of 40% of chronic alcoholics but not in those of moderate drinkers. To determine whether this sclerosis could be a precursor lesion of cirrhosis, controlled studies were performed in animal models. In the alcohol-fed baboons that developed fibrosis or cirrhosis, progressive perivenular sclerosis invariably started at the fatty liver stage before or even more commonly in the absence of alcoholic hepatitis. No sclerosis occurred in controls or in alcohol-fed baboons and rats that did not progress beyond the fatty liver stage. The clinical and experimental data indicate that sclerosis around the terminal venules, a common but often overlooked complication of alcoholic fatty liver, reflects heavy prolonged drinking, and may identify those patients who are susceptible to develop the more advanced lesions of alcoholic liver injury upon continued drinking.

Adult↗
PubMed1981

Myelin basic protein, oligoclonal bands, and IgG in cerebrospinal fluid as indicators of multiple sclerosis.

There currently are three clinical laboratory procedures for use with cerebrospinal fluid that assist in the diagnosis of multiple sclerosis: measurement of myelin basic protein and IgG, and demonstration of an oligoclonal band. We compared characteristics of these procedures, using CSF samples from 166 patients identified as having (54 patients) or not having (112 patients) multiple sclerosis. We find that oligoclonal band demonstration is the most useful single test in helping to establish the presence of multiple sclerosis; IgG quantitation is the least helpful. Myelin basic protein should be quantitated for following the activity of multiple sclerosis; it may be applied only selectively in the context of screening. The incidence of false-positive results reinforces the view that the diagnosis of multiple sclerosis must be made in clinical context. These laboratory procedures are not suitable for use as screening tests.

Electrophoresis, Agar Gel↗
PubMedDate not supplied

[Somatic types of patients with multiple sclerosis].

In 129 patients with multiple sclerosis (61 men and 68 women) anthropometric measurements were taken and 5 somatic indices calculated. The types of somatic constitution and the so-called somatic composition were determined using the method of reference points of Wanke. The results were compared with those in a control group representing the Polish population and with a group of patients with psoriasis. The usual male patient with multiple sclerosis was of athletic-pyknic constitution with very strongly expressed (in relation to the control group) features of dysplastic type (A). The typical male individual from the control group had pyknic-athletic constitution. The typical female patient with multiple sclerosis had leptosomic-dysplastic constitution, while the typical control female was leptosomic-pyknic. Male and female patients with multiple sclerosis had the hyperbrachycephalic skull configuration while the control group was branchycephalic. There was no difference between controls and patients with multiple sclerosis with regard to blood groups A1, A2, B, O and Rh.

Adolescent↗
PubMedDate not supplied

[Multiple sclerosis and the history of infectious diseases in childhood].

A case-control study was carried out comparing 300 patients with multiple sclerosis with 300 patients with ischialgia of various aetiology matched for age analysing history data concerning past infectious diseases and their complications in childhood. The following conclusions have been reached: In patients with multiple sclerosis exanthematous diseases (measles, rubella, varicella) and mumps in childhood were more frequent than in controls. No history of measles in late childhood occurring with higher frequency was found in the multiple sclerosis group as compared with controls. The age of measles had no greater pathogenetic importance for the development of multiple sclerosis. A high susceptibility to viral infections in childhood contributes to the susceptibility to neuroallergic reactions such as multiple sclerosis at later age.

Adolescent↗
PubMed1983

[Radionuclide assessment of urodynamics and kidney function in prostatic sclerosis patients (before and after surgery)].

A study was made of 137 patients with prostatic sclerosis ranging in age from 14 to 79. Basing on the results of clinical, x-ray and radionuclide examination of patients with prostatic sclerosis 3 degrees of disturbed urodynamics of the lower urinary tract have been singled out for this disease entity: compensated, subcompensated and decompensated. In accordance with the above degrees of the disturbed urodynamics groups of patients have been analysed before and after surgery (partial and subtotal resection of the prostate and prostatectomy). Prostatic sclerosis has been shown to cause changes in the urine output rate, the time of urination and residual urine. The UFM index is lowered as compared to the normal one more than 6 times. Residual urine (RU) reached 234.0 +/- 62.2 ml, the time of urination is extended by more than 2.4 times. It has been established that disturbed permeability of the posterior urethra results in the disturbance of the urodynamics of the upper urinary tract, separate and summary renal function. Radionuclide investigation helped to study the long-term results of operation efficacy by the reparation of the urodynamics in 91 patients with prostatic sclerosis. In most of the patients, the urodynamic indices returned to normal or improved: in 78.2% of the patients with prostatic sclerosis, the time of urination returned to normal, in 21.2% the time reduced. The UFM index returned to normal in 36.2% of the patients, in 40.6% it was moderately reduced, and in 23.0%, it remained sharply reduced. RU over 100 ml was determined in 6.5% of the patients only. After reparation of the permeability of the posterior urethra renal function either returned to normal or greatly improved in 37.9% of the patients. In 52.5% of the patients, renal function did not reach normal however, gradually improving it got stable at a certain level which was sufficient for the improved conditions of a patient and his future life.

Adolescent↗
PubMed1980

[Electronystagmograhy in disseminated sclerosis. Uses and limits (author's transl)].

A review of the literature shows the extreme frequency of nystagmus and changes in vestibular reflexes in disseminated sclerosis. During the course of this disease, it is possible to detect nystagmus in at least two-thirds of cases and in a similar proportion there are changes in vestibular reflexes, usually a hyperreflexia, especially in the early phases of disseminated sclerosis. Subjectively the patients rarely complain of vertigo and such symptoms are in any case part of the early form of the disease. On the other hand, disorders of balance are much more frequent but the factors involved are numerous, and the role of the vestibular system is often debatable. Electronystagmography and oculography demonstrate the extreme frequency of spontaneous or fixed gaze nystagmus in this disease, as well as the extreme frequency of changes in conjugated eye movements; changes in reflex, voluntary and automatic conjugated eye movements. As a matter of fact, there are many kinds of nystagmus which are not related to any changes in the vestibular system, but which are rather more part of the overal changes of conjugated eye movements. Seen in this light, electronystagmography and oculography, by exploring the different kinds of conjugated eye movement, can help decisively in the diagnosis of disseminated sclerosis by revealing changes which reflect damage to the brain stem, lesions which are clinically very often silent. In this way the diagnosis of disseminated sclerosis may be confirmed. Furthermore, electronystagmography can easily suggest another diagnosis, other than the early form of disseminated sclerosis of the vestibular type.

Electronystagmography↗
PubMed1982

The cerebrospinal fluid immunoglobulin G index as a diagnostic aid in multiple sclerosis: a Bayesian approach.

Having determined immunoglobulin G (IgG) and albumin concentrations in 1100 cerebrospinal fluid and serum samples, we calculated the IgG index. Likelihood ratios for multiple sclerosis were calculated by using a training set consisting of 100 patients with definite multiple sclerosis and one consisting of 97 patients suffering from diseases from which multiple sclerosis must be differentiated. Predictive values for multiple sclerosis, given different values for the IgG index, are given in a graphical representation of Bayes' theorem. We conclude that this approach increases the diagnostic usefulness of the IgG index for the diagnosis of multiple sclerosis.

Adolescent↗
PubMedDate not supplied

[Complement 3 level in the cerebrospinal fluid in multiple sclerosis].

Using the test of radial diffusion the absolute (mg%) and relative (% of albumin, % of total protein in cerebrospinal fluid) levels of the C3 component of the complement were determined in the cerebrospinal fluid and serum in a group of 61 cases of multiple sclerosis, and similar determinations were carried out in the control groups of patients with neurosis and organic diseases of the central nervous system. The absolute and relative levels of the C3 component in the cerebrospinal fluid in cases of multiple sclerosis were significantly lower (p < 0,001) and were independent of the levels of total protein and albumin in the cerebrospinal fluid. The low level of C3 in the cerebrospinal fluid was due to its low level in the serum in the same cases of multiple sclerosis, and the correlation coefficient of C3 levels in the cerebrospinal fluid and serum in this disease was r = 0.816. The C3 index studied in this work was statistically insignificantly lower in the group of multiple sclerosis with low C3 level in the cerebrospinal fluid as compared with the cases of this disease with normal C3 level in the cerebrospinal fluid. This shows that no data were obtained indicating a local consumption of complement components within the central nervous system in cases of multiple sclerosis. The possibility of a temporarily reduced complement consumption in the areas of demyelination cannot be excluded.

Albumins↗
PubMed1994

[Magnetic Resonance Imaging study of the role of the blood-brain barrier in the pathogenesis of experimental allergic encephalomyelitis: application to multiple sclerosis].

Nuclear magnetic resonance imaging (MRI) proved to be, from the first, a very sensitive method, allowing the visualisation of multiple sclerosis lesions, yet which never permitted to establish a non equivocal relationship between the semeiology of such lesions and the clinical signs. The multifocal aspect of disseminated multiple sclerosis lesions is probably one of several factors accounting for this discrepancy. The study of an autoimmune disease, experimental allergic encephalomyelitis (EAE), regarded as a suitable model for multiple sclerosis in humans, has been performed using MRI in order to unravel the pathogenesis of the disease and apprehend the mechanisms responsible for the formation of multiple sclerosis lesions. The study focused on the part played by the blood-brain barrier (BBB) in the induction process of an autoimmune disease, since the central nervous system is normally screened from immunological supervision, by this barrier. Models both of acute EAE, induced by active or passive transfer of the antigen (myelin basic protein-MBP)--and chronic EAE, induced by passive transfer of MBP-specific T cells and myelin glycoproteins or MOG-specific monoclonal antibodies, have been reproduced, and their evolution followed up using high field MRI. Every time, the crucial role of the BBB was evidenced by the synchronism existing between the clinical signs, the appearance of lesions, preferentially in the most sensitive or permeable areas, and the BBB breakdown encouraged by the action of adjuvants. The physiopathological study of EAE using MRI is suggestive of the concept of systemic disease for multiple sclerosis, according to a two-step process, involving, in a first stage some primary viral or bacterial infection, causing T-cells to be sensitized to the host's own proteins by molecular mimicry, and in a second stage some bacterial infection or accidental circumstances which, resulting in a BBB breakdown, would provide immunocompetent cells with an opportunity to reach their target.

Animals↗
PubMed1994

Multiple sclerosis.

Differences in the risk of multiple sclerosis depending on racial background, and the high clinical concordance rates in monozygotic compared with dizygotic twins, have stimulated attempts to identify and locate genes that confer susceptibility to the disease. The risk of multiple sclerosis is increased from 1 in 800 in northern European Caucasians to 1 in 3 in the monozygotic co-twins of affected individuals, with intermediate rates for siblings, offspring and more distant relatives. Concordance rates in monozygotic and dizygotic co-twins of affected individuals rise to 35% and 15%, respectively when magnetic resonance imaging is used to supplement clinical evidence for disease status. The increased recurrence risk in relatives of patients with multiple sclerosis is consistent with a model in which more than one gene contributes to susceptibility. Population studies have demonstrated an association with the class 2 major histocompatibility complex (MHC) phenotypes DR15 and DQw6 and their corresponding genotypes DRB1.1501, DRB5.0101 and DQA1.0102, DQB2.0602. An extensive search, using population studies, for other polymorphic alleles involved in restriction of the immune response may have yielded an additional candidate gene in the VH2-5 immunoglobulin heavy-chain variable region. Identity by descent analysis of candidate genes encoded within the alpha-chain of the T-cell receptor and the gene for myelin basic protein has failed to demonstrate linkage; paradoxically, this is also true for the MHC class 2 region, despite the population association. However, studies involving a large number of sibling pairs have reported a bias in the distribution of T-cell receptor beta-chain variable region haplotype sharing, favouring linkage. This becomes more marked when stratification is made for the presence of DR2 in both affected siblings, suggesting an interaction between genetic polymorphisms encoded within the MHC and T-cell receptor genes, as expected from their known functional co-operation in antigen presentation. The same is true for the immunoglobulin heavy chain, providing provisional evidence for linkage to a gene encoded within the immunoglobulin heavy-chain variable region in families reported from the UK. Taken together, these findings demonstrate the importance of family studies in elucidating the genetic basis of multiple sclerosis, and confirm that several genes are involved, one or more of which regulates genetic restriction of the immune response. The contribution made by the susceptibility genes that have provisionally been identified, occurring in isolation or together, can account for only a proportion of the increased risk of multiple sclerosis implicated by family studies.(ABSTRACT TRUNCATED AT 250 WORDS)

Genes↗
PubMed1993

[Precocious puberty revealing Bourneville tuberous sclerosis].

BACKGROUND: Benign tumors are seen in tuberous sclerosis. They are found in many organs, and the precocious puberty due to hypothalamic hamartoma and tuberous sclerosis has been reported. However, precocious puberty exceptionally reveals the tuberous sclerosis. CASE REPORT: A 2 month-old boy was admitted because of the fortuitous discovery of polycystic renal disease. Precocious puberty developed at 13 months with enlargement of the penis and testes, appearance of pubic hair, acne and deepening of the voice. Linear growth was recently accelerated and the bone maturation was advanced. Plasma testosterone was elevated (460 ng/100 ml) and LH-RH injection induced rapid rises in plasma LH (2.6 to 28 mUl/ml) and FSH (2 to 8 mUl/ml). Brain imaging techniques (CT scan and NMR) showed a hypothalamic hamartoma and periventricular calcified lesions. Examination with the Wood lamp identified two white leaf macules in the dorsal area. Administration of an analogue of LH-RH effectively reduced the manifestations of precocious puberty. CONCLUSION: Tuberous sclerosis is exceptionally revealed by precocious puberty. The association of polycystic disease and precocious puberty has never before been reported in tuberous sclerosis.

Hamartoma↗
PubMedDate not supplied

Amyotrophic lateral sclerosis an enigmatic disease with B-cellular and anti-retroviral immune responses.

OBJECTIVES: The most frequent sporadic adult motor neuron disease, amyotrophic lateral sclerosis, affects more men, follows no epidemiologic pattern, and was long considered a pure spinal cord disorder. It now becomes evident that the disease is characterized by spinal, cerebral and extra-neuromuscular changes including B-cellular responses and ultrastructural skin alterations. Do these parameters identify subgroups or correlate with the male preponderance of the disease? METHODS: We analyzed age at and site of onset, sex, duration of clinical disease, and human foamy retroviral seroreactivity in 47 consecutive patients with a definite diagnosis of amyotrophic lateral sclerosis. The results were compared with antivisna seroreactivity, immunoglobulin isotypes, circulating immune complexes, neopterin and beta 2-microglobulin as well as skin biopsies in respective subsets of the same 47 patients. RESULTS: Seroreactivity to recombinant human spuma retrovirus (HSRV) envelope and/or capsid protein was positive in 20/47 amyotrophic lateral sclerosis patients, and 28/30 competed with specific retroviral antibodies on maedi-visna antigen. Anti HSRV-seronegative patients had lower immunoglobulin IgG3 isotype concentrations, while HSRV-gag plus HSRV-env antibody positives demonstrated highest circulating IgG immune complexes. All 11 amyotrophic lateral sclerosis patients partially reacting to recombinant HSRV-env or HSRV-gag antigen were men, and male amyotrophic lateral sclerosis patients tended to have higher total cerebrospinal fluid protein levels. Neopterin and beta 2-microglobulin as markers of a cellular immune activation remained basically normal in serum and cerebrospinal fluid. CONCLUSION: We suggest a particular B-lymphocytic and retroviral involvement in this enigmatic, relentlessly progressing, at present untreatable and most frequent neurological system degeneration. To our opinion this situation justifies the search for novel anti-retroviral therapeutic strategies.

Adolescent↗