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At least 1,315 records · Page 73Linked to original sources
PubMed2006

[Renal manifestations in tuberous sclerosis].

BACKGROUND: Tuberous sclerosis is a rare inherited disease which can involve several organs. Renal involvement is one of the most severe manifestations of the disease. We analysed in this study renal involvement of tuberous sclerosis. METHODS: We studied retrospectively nine cases of tuberous sclerosis with renal involvement diagnosed between 1994 and 2005. The diagnosis was based on the presence of typical extra-renal manifestations and familial history of tuberous sclerosis. RESULTS: There were eight females and one male. Their mean age was 40.8+/-16.4 years (range: 15-62). At first presentation, we noted hypertension in three cases, proteinuria in seven cases, hematuria in eight cases and renal failure in five cases (end stage renal failure in four cases). By ultrasonography, we found angiomyolipoma in one case, cysts in four cases and angiomyolipoma associated to cysts in four cases. Nephrectomy was performed in two patients, in one case for hydronephrosis secondary to urolithiasis and in the other for hemorrhagic complication. In the later, pathological examination revealed renal carcinoma. At last evaluation, two patients were lost to follow-up, one had normal serum creatinine, five were on renal replacement therapy and the latter died from sepsis following kidney removal. CONCLUSIONS: In our patients, renal disease was diagnosed late at chronic renal failure stage. Patients with end-stage renal failure require dialysis and renal transplantation, but we recommend binephrectomy after starting dialysis and before transplantation due to the risk of cancer and bleeding related to angiomyolipomas.

Adolescent↗
PubMed2001

A case of multiple sclerosis with granulomatous uveitis in Japan--use of the antilipoarabinomannan (LAM)-B test in differential diagnosis.

PURPOSE: To report a patient with multiple sclerosis and associated with granulomatous uveitis, and how anti-lipoarabinomannan (LAM)-B antibody can play a key role in differential diagnosis. METHODS: Case report. RESULTS: A 35-year-old Japanese woman with multiple sclerosis, diagnosed 3 years ago, presented with blurred vision in her left eye. Ophthalmological examinations revealed granulomatous iridocyclitis in her left eye and retinal periphlebitis in both eyes. The diagnosis of tuberculosis was suspected because of a positive tuberculin skin test. However, a further examination by an anti-LAM-B antibody test excluded active tuberculosis. Her clinical findings were thought most likely to be caused by multiple sclerosis and treated with corticosteroids. CONCLUSION: We should consider the possibility of multiple sclerosis as the underlying origin in patients with granulomatous uveitis. A measurement of anti-LAM-B antibody titer may be useful in the differential diagnosis of granulomatous uveitis.

Administration, Topical↗
PubMed2001

Assessment of nuclear sclerosis after nonvitrectomizing vitreous surgery.

PURPOSE: Nuclear sclerosis develops frequently after successful pars plana vitrectomy. We evaluated changes in the degree of nuclear sclerosis after nonvitrectomizing vitreous surgery for idiopathic epimacular proliferation. METHODS: Forty-one consecutive patients (41 eyes) underwent removal of idiopathic epimacular proliferation by nonvitrectomizing vitreous surgery and were followed postoperatively for at least 12 months. Visual acuity, refractive error, slit-lamp biomicroscopy, and Scheimpflug photographs were assessed preoperatively and postoperatively to evaluate changes in the degree of lenticular opacification. Quantitative analysis of the nuclear sclerosis was performed by densitometry with Scheimpflug photographs performed on only the last 21 patients. We evaluated these measurements by comparing statistically the preoperative and postoperative difference between both eyes (operative eye minus nonoperative ocular data). RESULTS: There was no significant difference in the progression of nuclear sclerosis or degree of myopic shift between the operated and fellow eyes during postoperative follow-up (mean +/- SD, 22 +/- 8 months; median, 22 months; range, 12 to 48 months). The average preoperative and postoperative refractive errors in operated eyes were 0.0 +/- 2.4 diopters and 0.1 +/- 2.5 diopters, respectively; the average difference in the refractive errors between both eyes was -0.2 +/- 0.7 diopter preoperatively and -0.2 +/- 0.9 diopter postoperatively (P =.961, paired t test). The average preoperative and postoperative nuclear density values by Scheimpflug photography in 21 operated eyes were, respectively, 72 +/- 18 nuclear density units and 75 +/- 17 nuclear density units; the average difference in nuclear density values between both eyes was -1 +/- 4 nuclear density units preoperatively and 0 +/- 6 nuclear density units postoperatively (P =.631, paired t test). CONCLUSION: Progression of nuclear sclerotic cataract based on changes in refractive error and Scheimpflug photography was not observed after nonvitrectomizing vitreous surgery.

Aged↗
PubMed2003

Intermediate uveitis in childhood preceding the diagnosis of multiple sclerosis: a 13-year follow-up.

PURPOSE: An association between multiple sclerosis during childhood and uveitis is exceptionally rare. This is a report of a female patient who presented at the age of 8 years with bilateral intermediate uveitis and whose final diagnosis of multiple sclerosis was made at age 21 years. DESIGN: Case report. METHOD: Retrospective chart review of a 13-year follow-up history. RESULTS: Over 10 years our patient was treated systemically and underwent bilateral vitrectomy to reduce permanent side effects. Owing to good visual function and low inflammatory signs, systemic therapy was stopped. Multiple sclerosis was diagnosed at the age of 21, after a 13-year history of uveitis and after 3 years without medication. CONCLUSIONS: In the constellation of uveitis in childhood and later diagnosis of multiple sclerosis, the outlined therapy provided good functional results. Moreover, it may have delayed the manifestation of the underlying disease for 13 years.

Azathioprine↗
PubMed2000

Multiple sclerosis and chronic autoimmune encephalomyelitis: a comparative quantitative study of axonal injury in active, inactive, and remyelinated lesions.

Recent magnetic resonance (MR) studies of multiple sclerosis lesions indicate that axonal injury is a major correlate of permanent clinical deficit. In the present study we systematically quantified acute axonal injury, defined by immunoreactivity for beta-amyloid-precursor-protein in dystrophic neurites, in the central nervous system of 22 multiple sclerosis patients and 18 rats with myelin-oligodendrocyte glycoprotein (MOG)-induced chronic autoimmune encephalomyelitis (EAE). The highest incidence of acute axonal injury was found during active demyelination, which was associated with axonal damage in periplaque and in the normal appearing white matter of actively demyelinating cases. In addition, low but significant axonal injury was also observed in inactive demyelinated plaques. In contrast, no significant axonal damage was found in remyelinated shadow plaques. The patterns of axonal pathology in chronic active EAE were qualitatively and quantitatively similar to those found in multiple sclerosis. Our studies confirm previous observations of axonal destruction in multiple sclerosis lesions during active demyelination, but also indicate that ongoing axonal damage in inactive lesions may significantly contribute to the clinical progression of the disease. The results further emphasize that MOG-induced EAE may serve as a suitable model for testing axon-protective therapies in inflammatory demyelinating conditions.

Acute Disease↗
PubMed1999

WT1 and PAX-2 podocyte expression in Denys-Drash syndrome and isolated diffuse mesangial sclerosis.

Denys-Drash syndrome is a rare disorder of urogenital development characterized by the association of early onset glomerulopathy caused by diffuse mesangial sclerosis, gonadal dysgenesis leading to pseudohermaphroditism in males, and a high risk of developing Wilms' tumor. The syndrome is caused by dominant negative point mutations in the WT1 gene that encodes a tumor suppressor transcription factor normally expressed in podocytes. Mutations usually affect the zinc fingers of the WT1 protein. The basic defect is unknown in most cases of isolated diffuse mesangial sclerosis, a disease characterized by the same glomerular changes as in Denys-Drash syndrome but possibly transmitted as an autosomal recessive trait. Here we show that the distribution of WT1 is abnormal in most patients with Denys-Drash syndrome : WT1 nuclear staining of podocytes is decreased or absent. This finding is consistent with the decreased DNA binding capacity of the mutated protein. One target gene of WT1 is PAX2, the expression of which is down-regulated in podocytes during early stages of nephrogenesis. We demonstrate that WT1 mislocalization is associated with abnormal podocyte expression of PAX2 protein and RNA. We suggest that persistent expression of PAX2 is likely to result from the loss of WT1 dependent transcriptional repression and may participate in the pathological mechanisms leading to glomerular dysfunction. Abnormal distribution of WT1 and PAX2 was also observed in isolated diffuse mesangial sclerosis suggesting that a defect in WT1 could also be operative in isolated diffuse mesangial sclerosis. Primary involvement of PAX2 is an alternative hypothesis because persistent expression of PAX2 in transgenic mice is associated with the occurrence of early and severe glomerulopathy.

Animals↗
PubMed2000

Verbal learning strategies in patients with multiple sclerosis.

Word list learning was studied in patients with a definite diagnosis of Multiple Sclerosis and in Normal Control subjects by means of the selective reminding procedure of Buschke and Fuld in two learning conditions: (1) using unrelated items and (2) paired-associate items. The Multiple Sclerosis patients displayed poor learning in both conditions. To identify the functional locus of their deficit, stochastic Markov chain analyses were performed, which allowed individual measurements of encoding, automatic and intentional retrieval abilities. On both tasks, encoding on the first trial and automatic retrieval on the subsequent trials were impaired in Multiple Sclerosis patients, whereas intentional retrieval, both with and without reminding by the examiner, appeared to be preserved. As all of the impaired abilities require a normal speed of information processing, the salient learning deficit of the Multiple Sclerosis patients could be tentatively traced back to the slowing down of their mental activity.

Adult↗
PubMed1996

Renal cell carcinoma in association with tuberous sclerosis in children.

Tuberous sclerosis, a genetically transmitted multisystem neurocutaneous disorder, is associated with renal lesions in 50% of cases. The expected lesions are angiomyolipomas or renal cysts; renal cell carcinoma has been encountered in adults who have tuberous sclerosis, but is very rare in children. The authors report the case of a 5-year-old girl with tuberous sclerosis for whom atypical computed tomography findings led to the diagnosis of renal carcinoma at an early age. This experience suggests that children with tuberous sclerosis may need earlier screening.

Adult↗
PubMed1998

The nature of fatigue in multiple sclerosis.

The aim of this cross-sectional descriptive study was to determine whether people with multiple sclerosis differentially experience physical and mental fatigue, and whether the pattern of fatigue is influenced by mood, disease duration, or disease course. Seventy-eight consecutive out-patients with multiple sclerosis were asked to complete the Fatigue Rating Scale (FRS) and the Hospital Anxiety and Depression Scale (HAD). Sixty-eight (87%) subjects completed the questionnaires. Fifty-eight (85%) subjects in this group scored above the recommended cut-off for fatigue on the FRS scale. Both the mental fatigue score and the total fatigue score were positively correlated with the depression and anxiety scores on the HAD scale. There was no significant correlation between the physical fatigue score and either of the HAD subscale scores. There was no significant association between duration of disease or disease course and the total scores or subscale scores of the FRS and HAD. This is the first reported study to differentiate between mental and physical fatigue in multiple sclerosis and to demonstrate a significant correlation between fatigue and mood level. This has important implications for the effective treatment of fatigue in multiple sclerosis.

Adult↗
PubMed2001

Sexual dysfunction in multiple sclerosis: a 2-year follow-up study.

BACKGROUND AND OBJECTIVE: Sexual dysfunction severely affects the quality of life of patients, but longitudinal studies of sexual function in multiple sclerosis are lacking. We performed a study on a group of patients with multiple sclerosis to evaluate the change in sexual function and to examine the relationship between sexual dysfunction and other clinical variables over time. METHODS: A 2-year follow-up study on 99 patients with definite multiple sclerosis. Information on sexual and sphincteric disturbances have been collected through face-to-face structured interviews. Disability, independence, cognitive performances and psychological functioning have also been assessed. Spearman rank correlation analysis corrected for multiple comparisons, and linear regression analysis have been performed to test variables relationship and remove the effect of potential confounding covariates. RESULTS: The proportion of patients with sexual dysfunction remained over 70% and did not change during the 2-year follow-up, but the extent and number of symptoms increased significantly The number of symptoms of sexual dysfunction did not change significantly after an exacerbation. Significantly, more patients than before the study resorted to counseling and discussed with doctors of sexual matters. In the univariate analysis, changes in sexual function over time correlated with changes in bladder function (r=0.47, p<0.0001) and EDSS score (r=0.41, p<0.0001), but the multivariate analysis demonstrated that only bladder dysfunction was independently related to sexual dysfunction (R=0.36, p=0.003) when the effect of psychological factors were removed. CONCLUSIONS: Symptoms of sexual dysfunction increase in significance and number over time in patients with multiple sclerosis. Relapses did not influence the number of symptoms of sexual dysfunction, but a worsening of pre-existing symptoms cannot be excluded. The change of sexual function appears to be independently associated to bladder dysfunction.

Adult↗
PubMed1998

Fatigue in multiple sclerosis: interrelations between fatigue complaints, cerebral MRI abnormalities and neurological disability.

Although fatigue is a frequent complaint of patients with multiple sclerosis (MS), little is known about the origins of multiple-sclerosis-associated fatigue. Our primary focus was to study if the extent of cerebral abnormalities, as shown on magnetic resonance imaging (MRI), had any relation with the frequency and intensity of fatigue complaints of patients with a definite diagnosis of MS. Fatigue severity was rated by the patients with the use of a 2-week diary and a fatigue questionnaire, while conventional T1- and T2-weighted MRI provided several measures for cerebral abnormalities. In total, 72% of 45 patients reported to be seriously fatigued at least several times a week over the last 3-month period. Fatigue severity was not related to the total extent of cerebral abnormalities, or to MRI-based atrophy measures. Regional lesion load did not differ between fatigued and non-fatigued subjects. Although neurological disability, as measured by the Expanded Disability Status Scale (EDSS) and Neurological Rating Scale (NRS), did correlate significantly with most MRI measures, it showed no relation with fatigue severity. Neurological progression rates and number of exacerbations in the 2-year period prior to assessment were not significantly associated with the fatigue measures. Therefore, our findings suggest that differences in levels of self-reported fatigue in patients with multiple sclerosis cannot merely be explained by the degree of clinical disease activity, neurological disability or the extent of MRI abnormalities. These results are compared to other research findings and the possible role of alternative factors influencing fatigue in multiple sclerosis are discussed.

Adult↗
PubMed1999

Olfactory loss in multiple sclerosis.

The objectives of the present study were to test odor identification ability in patients with multiple sclerosis (MS) and to examine possible correlations between smell identification test scores and various clinical variables. We performed a case-control study comparing the Cross Cultural Smell Identification Test scores of 40 patients with definite multiple sclerosis with those obtained in 40 age-, sex- and smoking-habit-matched healthy controls. The neurological impairment, the disability, the cognitive performances and the psychological functioning were also assessed. Patients with multiple sclerosis scored significantly poorer than controls on the Cross-Cultural Smell Identification Test (P<0.001). Olfactory function was borderline normal in four (10%) and abnormal in five (12.5%) MS patients, whereas it was normal in all controls (P<0.02). Significant correlations between the smell identification score and symptoms of anxiety (r=-0.43, P=0.006), depression (r=-0.42, P=0. 008) and severity of neurological impairment (r=-0.32, P=0.05) were found. Only two (5%) patients with multiple sclerosis reported having episodes of smell loss, suggesting a low level of awareness of this problem. Although smell changes are rarely reported, olfactory function is impaired in a considerable number of patients with MS. The observed association between decreased odor identification ability and symptoms of anxiety and depression in our patients suggests that mood and anxiety disorders have to be considered in assessing olfaction in MS patients. Clearly, smell disturbances deserve greater attention from health professionals and caregivers dealing with such patients.

Adult↗
PubMed1991

Evidence for early lower urinary tract dysfunction in clinically silent multiple sclerosis.

The occurrence of micturition complaints as late sequelae of multiple sclerosis is well studied and documented. However, no reports exist on urologically asymptomatic patients with a relatively short disease duration. In a prospective study of 40 patients with definite multiple sclerosis (mean disease duration 5 years), urodynamic investigations (cystometry and pressure-flow study) were combined with neuro-urophysiological measurements (cortical evoked potentials and sacral reflex latencies). Patients with (13) and without (27) micturition complaints were investigated. Neurourodynamic abnormalities were seen in 35 of 40 patients (88%). The lower urinary tract proved to be afflicted by multiple sclerosis at an early stage of the disease. Early neurourodynamic investigations had clinical implications. All of the complaining and half of the noncomplaining patients showed urodynamic abnormalities upon which the need for further followup and eventual therapeutic intervention was based. We conclude that neurourodynamic testing of a urinary functional system can be worthwhile as part of the initial diagnostic evaluation in patients with proved multiple sclerosis.

Adult↗
PubMed1989

Update on the epidemiology of multiple sclerosis.

Neuroepidemiology has been important in providing clues about the cause and pathogenesis of multiple sclerosis. In this review, we update the incidence and prevalence rates of multiple sclerosis in Olmsted County, Minnesota, and examine the potential role of viruses, exposure to animals, toxins, trauma, and diet in the development of this disease. Diseases of probable autoimmune nature have also been linked to multiple sclerosis. These descriptive data may contribute to the formulation of testable specific hypotheses about the pathogenesis and treatment of multiple sclerosis and other demyelinating diseases.

Age Factors↗
PubMed1989

Immunosuppressive treatment of multiple sclerosis.

Multiple sclerosis is thought, by many investigators, to be an immunologic disease. Therefore, a rationale exists for treating this disease by immunosuppressive therapy. In exacerbating-remitting multiple sclerosis, corticosteroids and adrenocorticotropic hormone are the most widely used drugs; high doses of intravenously administered methylprednisolone have recently gained favor. Chronic progressive multiple sclerosis has been treated with a number of immunosuppressive regimens, several of which have shown promise to date. Cyclophosphamide and azathioprine have been used most often and are reviewed in this report, as are other agents currently under investigation. No firm guidelines for the treatment of chronic progressive multiple sclerosis can be offered, but an approach to immunosuppressive therapy is suggested in this review.

Adrenal Cortex Hormones↗
PubMed2004

[Treatment of progressive multiple sclerosis with monthly pulsed cyclophosphamide-methylprednisolone: predictive factors of treatment response].

INTRODUCTION: Cyclophospamide is used in the treatment of progressive multiple sclerosis. We were looking for predictive indicators of treatment response. MATERIAL AND METHODS: Forty-seven patients with secondary progressive multiple sclerosis and seven others with primary progressive received monthly infusions of cyclophosphamide (750mg/m2) and methylprednisolone (500mg). During the year before cyclophosphamide the EDSS had worsened one point in all patients with or without surimposed relapses. Evaluation was based on EDSS change at 6, 12, 24 months and 5 years. RESULTS: Among secondary progressive patients, 91 per 100 (43/47) were stable or improved at 12 months, 65 per 100 (26/40) at 24 months and 22 per 100 (5/23) at 5 years. Annual relapse rate decreased from 0.81 before treatment to 0.48 during treatment and 0.12 after treatment (p<0.001). At 24 months, efficacy was correlated to a progressive phase lasting less than 5 years (p<0.01) and to a rapid increase of EDSS of at least 2 points the year before treatment (p<0.05). There were no influences of age, EDSS and surimposed relapses at the beginning of treatment, and other immunoactive drugs administrated before cyclophosphamide. There was no significant difference in quality of response to treatment between patients with primary progressive and secondary progressive multiple sclerosis. CONCLUSION: Cyclophosphamide appears to be more efficient in early stage of progressive multiple sclerosis independently of age, relapses or neurological disability scale.

Adjuvants, Immunologic↗
PubMed2005

[Balò's concentric sclerosis in a North-African patient].

INTRODUCTION: Balo's concentric sclerosis is a rare variant of multiple sclerosis described by Balo in 1928. It is characterized by alternating rings of demyelination and spared myelin. CASE REPORT: We report a case of Balo's concentric sclerosis diagnosed by the typical MRI findings of concentric rings of demyelination. Medullar and brain localisation were found and clinical course was good under intravenous corticosteroids. CONCLUSION: MRI provides the best diagnostic information for Balo's concentric sclerosis, allowing early diagnosis and treatment.

Adult↗
PubMed2003

TNF-related apoptosis inducing ligand (TRAIL) as a potential response marker for interferon-beta treatment in multiple sclerosis.

BACKGROUND: Many patients with multiple sclerosis do not respond to interferon beta, which is widely used as an immunomodulatory treatment in this disease. We aimed to assess the functional relevance of tumour necrosis factor (TNF)-related apoptosis inducing ligand (TRAIL), which is upregulated on incubation with interferon beta, for clinical treatment response. METHODS: We quantified gene expression longitudinally by realtime-PCR of the peripheral immune cells of 82 patients with multiple sclerosis. In a first cohort of 62 patients, 20 were classified as first-year responders since they did not have relapses during treatment with interferon beta 1a; 19 were classified as first-year non-responders; and 23 developed neutralising antibodies to interferon beta. A second cohort, also characterised by MRI, consisted of 11 patients on interferon beta 1a and nine patients who were not treated. Concentrations of soluble TRAIL were determined by ELISA in serum samples of nine non-treated patients, 49 patients before treatment (29 responders, 20 non-responders), as well as longitudinally in a subset of 23 patients. FINDINGS: In both patient cohorts, drug-responders could be distinguished from non-responders by early and sustained induction of TRAIL (p<0.0001, each). In the presence of neutralising antibodies, initial upregulation of TRAIL expression was subsequently abrogated. Raised concentrations of soluble TRAIL in patients' serum samples before the start of treatment allowed prediction of the treatment response in the first year (ROC analysis with area under the curve 0.879 [0.785-0.974]). INTERPRETATION: Our data suggest that TRAIL expression is a candidate for pretreatment assessment and might thus be used as a prognostic marker of treatment response to interferon beta in multiple sclerosis. Furthermore, our observations have implications for the development of future immunoregulatory strategies in multiple sclerosis therapy.

Adjuvants, Immunologic↗