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At least 1,297 records · Page 72Linked to original sources
PubMed1978

Renal failure secondary to angiomyolipoma. Case of Forme-Fruste tuberous sclerosis.

A fifty-four-year-old woman without the clinical features of tuberous sclerosis underwent nephrectomy at age thirty-three years for angiomyolipoma, and twenty-one years later severe renal failure developed. At necropsy the remaining kidney had extensive angiomyolipomatous involvement; not until the brain was examined was the diagnosis of tuberous sclerosis made. To date, this would appear to be the third case without clinical tuberous sclerosis in which renal involvement was the sole clinical expression of tuberous sclerosis, and the seventh reported instance of renal failure due to renal angiomyolipomatous hamartomatous transformation.

Female↗
PubMed1990

Antibodies to cerebellar soluble lectin CSL in multiple sclerosis.

Cerebrospinal fluid samples from 239 patients with various neurological disorders were tested for the presence of autoantibodies to an endogenous mannose-binding protein, the cerebellar soluble lectin CSL, by means of an immunoblotting test with rat CSL as antigen. 47 of 51 patients with multiple sclerosis were positive for anti-CSL compared with 30 of 188 patients with other neurological disorders. 14 of the 30 false-positive patients were over 60 years old, an age group not typical of multiple sclerosis patients. The specificity of the test for multiple sclerosis was 85% and the sensitivity 93.5%. The possibility that CSL is an important immunological target in multiple sclerosis allows new insights into the possible causes and development of this disorder.

Adult↗
PubMed1993

Hippocampal sclerosis in epilepsy and childhood febrile seizures.

The connection between hippocampal sclerosis and childhood febrile seizures (CFS) is a contentious issue in the study of epilepsy. We investigated 107 patients with drug-resistant epilepsy by high-resolution volumetric magnetic resonance imaging (MRI). 20 had a history of CFS, 45 had focal (26) or diffuse (19) hippocampal volume loss (HVL). The frequency of CFS was significantly (p < 0.001) higher in the patients with HVL, especially of the diffuse pattern, compared to other epileptic patients without HVL and to the general population. Furthermore, the severity of HVL was greatest in those with a history of CFS. No other clinical or demographic features were associated with either a history of CFS or HVL. The frequency of CFS in patients with other structural congenital causes of epilepsy did not differ from that in a general population. Although these findings show that hippocampal sclerosis is strongly associated with a history of CFS, they do not indicate whether this is a causal relationship. If CFS do cause some cases of hippocampal sclerosis, this can not be the only mechanism, as 64% of those with HVL gave no history of CFS. As diffuse HVL is more strongly associated with a history of CFS than focal HVL, it is also possible that CFS convert pre-existing congenital focal abnormalities into diffuse hippocampal sclerosis. Given the possibility that CFS may cause hippocampal damage and epilepsy, they require urgent medical intervention.

Adolescent↗
PubMed1987

Psychiatric aspects of multiple sclerosis.

A patient who presented with depressive and hysterical symptoms of several years' duration and who proved to have multiple sclerosis is presented. Two further cases whose initial symptoms of dissociation and major depression were prodromes of multiple sclerosis are also presented. A discussion of the factors leading to the correct diagnosis in these cases and reference to relevant literature on psychiatric presentations of multiple sclerosis are presented to illustrate the need for psychiatrists' awareness of multiple sclerosis as a causative factor in patients with a wide variety of psychiatric syndromes.

Adult↗
PubMed1989

Virus-specific cytotoxic T lymphocytes in multiple sclerosis: a normal mumps virus response adds support for a distinct impairment in the measles virus response.

An impairment of the measles virus-specific cytotoxic T lymphocyte response in multiple sclerosis was previously reported. This response is predominantly mediated by HLA class II-restricted CD4+ cells. In the present report, virus-specific cytotoxic T lymphocyte responses in multiple sclerosis were further studied by examining the response to mumps virus. No significant difference was detected in the generation of mumps virus-specific cytotoxic T lymphocyte responses between normal individuals and multiple sclerosis patients with impaired measles virus-specific cytotoxicity. A portion of the mumps virus-specific cytotoxic T lymphocyte response could be mediated by HLA class II-restricted CD4+ cells generated from both normal controls and MS patients. This CD4+ cell-mediated portion of the response was similar in both groups. These findings support the view that there is a distinct measles virus-specific impairment in cell-mediated cytotoxicity in multiple sclerosis.

Antigens, Differentiation, T-Lymphocyte↗
PubMed1991

HLA-DPB1 gene polymorphism and multiple sclerosis: a large case-control study in the southwest of France.

The polymorphism at the HLA-DPB1 locus has been characterized in a large number of patients with multiple sclerosis (n = 112) and in healthy controls (n = 115). Both patients and controls lived in the southwest of France (in the Pyrénées Atlantiques) and had similar ethnic background. The typing procedure involved the selective amplification of the second exon of the DPB1 locus by polymerase chain reaction, followed by hybridization of the amplified DNA with 14 sequence-specific oligonucleotide probes. Individual alleles were identified by the pattern of hybridization of the different probes. The distribution of the DPB1 alleles was not significantly different in multiple sclerosis patients and controls (p = 0.11). This does not corroborate the reported association of multiple sclerosis with the primed lymphocyte typing (PLT)-defined DPw4 specificity and is not in favour of a role played by polymorphic residues of the DP molecule in susceptibility to multiple sclerosis.

Alleles↗
PubMed1994

Expression of Fc epsilon R2/CD23 and p55 IL-2R/CD25 on peripheral blood macrophages/monocytes in multiple sclerosis.

Macrophages have been found histologically to be activated in multiple sclerosis. We analyzed the expression of CD23 and CD25 on monocytes/macrophages in peripheral blood obtained from patients with multiple sclerosis (MS) to investigate their role in the demyelinating process. Peripheral blood mononuclear cells were obtained from 30 patients with MS including four Baló's diseases (24 with acute relapsing type disease, six with chronic progressive type disease) and 12 healthy controls. The percentage of CD14+CD23+ monocytes/macrophages and CD14+CD25+ monocytes/macrophages were determined by two-color flow cytometry. The percentage of CD14+CD23+ monocytes was significantly higher in patients with MS in the active phase as compared with controls (P < 0.01). Six patients with acute relapsing MS, who had received no therapy, had higher CD14+CD23+ cells than did controls (P < 0.0001). CD14+CD25+ monocytes/macrophages were not detected in peripheral blood monocytes/macrophages of patients with MS except Baló's concentric sclerosis. The four patients with Baló's concentric sclerosis had markedly elevated levels of CD14+CD25+ monocytes/macrophages. Our findings suggest that monocytes/macrophages are activated during an exacerbation of MS, and that they may play an important role in the process of demyelination.

Adult↗
PubMed1996

The immune response to mycobacterial 70-kDa heat shock proteins frequently involves autoreactive T cells and is quantitatively disregulated in multiple sclerosis.

Heat shock proteins (HSP) are the most conserved molecules known to date that may also function as immune targets during infection. Hence, theoretically there is a high chance of cross-reactive responses to epitopes shared by host and microbe HSP. If not properly regulated, these responses may contribute to the pathogenesis of autoimmune disease. To determine if immune responses to HSP could contribute to the pathogenesis of multiple sclerosis, we raised T lymphocyte lines specific for the purified protein derivative of Mycobacterium tuberculosis (PPD) from patients with multiple sclerosis, patients with tuberculosis and from healthy individuals. These lines were then screened for their proliferative response to a M. tuberculosis 70-kDa heat shock protein (M.tb.HSP70). The relative frequency of the recognition of highly conserved sequences of M.tb.HSP70 compared to variable ones was also assessed by mapping experiments on those PPD specific T lymphocyte lines which also recognized the mycobacterial 70-kDa heat shock protein. In patients with multiple sclerosis, we observed a significantly higher estimated frequency of PPD-specific T lines responding to M.tb.HSP70 compared to healthy individuals and patients with tuberculosis. Furthermore, mapping experiments using recombinant proteins representing mycobacterial and human HSP70 sequences and a panel of synthetic peptides encompassing the whole sequence of Mycobacterium leprae HSP70, showed that the response to conserved epitopes of HSP70 is a frequent event in each of the three conditions studied, often leading to the cross-recognition of microbial and human sequences. These findings implicate the 70-kDa heat shock proteins as potential autoantigens in multiple sclerosis.

Adult↗
PubMed1995

Eye problems in children with multiple sclerosis.

In a retrospective review, the eye symptoms of 17 children (mean age: 13 1/2 years) who had definite multiple sclerosis (Poser's criteria) and 15 who had probable multiple sclerosis over the last 18 years were evaluated. Follow-up varied from 3 weeks to 6 years. Of 94% of children (16 of 17) with ophthalmologic symptoms, 47% (8 of 17) presented with an initial disturbance of vision. Twelve children had optic neuritis, 1 progressive uveitis, and 4 brainstem symptoms (i.e., VIth nerve palsy, 1 1/2 syndrome, internuclear ophthalmoplegia). Four children had cerebellar signs (nystagmus, saccadic pursuit). In 4 children, clinical localization was less specific. Recovery was generally good in most of the children; cerebellar problems were most persistent. Multimodal potentials were more helpful for investigation of optic neuritis and cerebellar lesions than for brainstem lesions. In the cohort of probable multiple sclerosis of 15 children, 11 had eye symptoms (5 with neuromyelitis optica, 4 optic neuritis, 1 internuclear ophthalmoplegia, and 1 cerebellar symptoms). Ophthalmologic symptoms are slightly more frequent in children with multiple sclerosis than in adults and should be specifically investigated to establish the diagnosis.

Adolescent↗
PubMed1995

Optic neuritis in children: recurrence and subsequent development of multiple sclerosis.

Recurrent optic neuritis and the subsequent development of multiple sclerosis in children, particularly in Asian countries, are not well known. We report on recurrent optic neuritis and subsequent multiple sclerosis in 22 Thai children who were younger than 15 years of age at the onset of optic neuritis, had no previous neurologic illness, and were monitored for 6-20 years. Improvement of vision was observed in 8, 10, and 2 patients after the first, second, and third week of onset, respectively. Two patients had recurrent optic neuritis and the other 2 patients subsequently developed clinical definite multiple sclerosis characterized by hemiparesis at 6 months and myelopathy at 2 years later in 1 patient and myelopathy and brainstem dysfunction in the other at 4 and 6 years later. The frequency of subsequent development of multiple sclerosis after optic neuritis may be similar to that reported from the United States and European countries.

Age of Onset↗
PubMed1996

A comparison of health-related quality of life in patients with epilepsy, diabetes and multiple sclerosis.

The purpose of this investigation was to compare self-reported health-related quality of life (HRQOL) in epilepsy compared to another neurological condition or a non-neurological chronic illness. Patients with epilepsy (N = 271), multiple sclerosis (N = 85) and diabetes (N = 555) completed a generic measure of HRQOL (RAND 36-Item Health Survey 1.0 (SF-36)), and the eight SF-36 scale scores were compared across groups, adjusting for differences in sociodemographic characteristics and co-morbid medical conditions. Patients with multiple sclerosis reported significantly worse HRQOL compared to both the epilepsy and diabetes groups (who did not differ from one another) on the Physical Functioning, Role Limitations-Physical, Energy, and Social Function scales. Patients with epilepsy and multiple sclerosis did not differ from one another but reported significantly lower HRQOL scores than the diabetes group on the Emotional Well-Being and Role Limitations-Emotional scales. However, the epilepsy group reported better health perceptions compared to the diabetes and multiple sclerosis patients. Generic measures of HRQOL appear useful in identifying some effects of neurological disease, but disease-targeted supplements may be required to more clearly identify the impact of epilepsy on quality of life.

Adult↗
PubMedDate not supplied

The skeletal effects of colony-stimulating factor-1 in toothless (osteopetrotic) rats: persistent metaphyseal sclerosis and the failure to restore subepiphyseal osteoclasts.

Toothless (tl), one of four osteopetrotic mutations in the rat, is characterized by few osteoclasts, undetectable bone resorption, and failure of correction by bone marrow transplantation. We recently reported that CSF-1 treatment improves these skeletal problems but that metaphyseal sclerosis persists. In the present study we demonstrate that optimal reduction of the skeletal sclerosis in tl rats following CSF-1 treatment has lower and upper dosage thresholds and that skeletal sclerosis returns after CSF-1 withdrawal. Osteoclasts increase significantly in tl rats after CSF-1 treatment, but compared to untreated normal littermates, histochemical staining for characteristic enzymes and osteoclast number is reduced and no osteoclasts appear in the subepiphyseal areas of long bones. These data are interpreted to mean that there are dosage limits to the beneficial skeletal effects of CSF-1, that persistent sclerosis is related to the failure to restore subepiphyseal osteoclasts, that osteoclast or progenitor populations may be deficient or differ in their responses to CSF-1, and that the defect in tl rats is not merely lack of a circulating, biologically active form of CSF-1.

Animals↗
PubMed2005

[Eccentric isokinetic strengthening in hamstrings of patients with multiple sclerosis].

OBJECTIVE: To assess the benefit of a program of rehabilitation involving eccentric isokinetic muscle strengthening for hamstrings of patient with multiple sclerosis. PATIENTS AND METHODS: A total of 28 patients with multiple sclerosis and abnormalities when walking who underwent a program of rehabilitation involving eccentric isokinetic muscle strengthening associated with classical neurological rehabilitation. Assessment was by use of an isokinetic test, clinical examination and score on a visual analog scale (VAS) about quality of walking at the beginning and end of the rehabilitation program and three months later. The rehabilitation program involved eccentric isokinetic exercises during 12 sessions. RESULTS: Patients with multiple sclerosis and difficulty walking showed weak hamstrings on manual muscular testing. After 12 sessions of isokinetic eccentric rehabilitation, hamstring and quadriceps strength increased, VAS score increased, and recurvatum of the knee improved in 26 cases. Three months later, the score for quality of walking remained the same, but that for strength returned to a previous level. CONCLUSION: Hamstring strength in patients with multiple sclerosis can be improved without complication with use of eccentric isokinetic rehabilitation. This is an interesting way to improve the quality of walking in these patients.

Adult↗
PubMed2005

Adaptive cortical changes and the functional correlates of visuo-motor integration in relapsing-remitting multiple sclerosis.

Cortical reorganization has been demonstrated during performance of a motor task in patients with multiple sclerosis. Converging evidence suggests that changes in gray matter volume represent an early hallmark of the disease. We used functional MRI to investigate the role of cortical adaptive mechanisms in maintaining visuo-motor function in the face of structural damage. Two cohorts of patients with clinically definite relapsing-remitting multiple sclerosis were compared with healthy controls matched for demographic, motor and cognitive characteristics during the performance of a visuo-motor integration task. Direct comparison between the two groups demonstrated a greater response of the contralateral dorsal premotor cortex and of the ipsilateral superior parietal cortex in relapsing-remitting multiple sclerosis patients. The functional MRI changes in these areas were strongly correlated with decreased gray matter volumes and increased lesion burden, respectively. Our study demonstrated a selective involvement of the parieto-premotor circuitry in a relatively early stage of the disease, which was not influenced by clinical, motor or cognitive variables. Moreover these results confirm the potential for functional recovery and the adaptive role of these areas in the motor reorganization of multiple sclerosis patients.

Adolescent↗
PubMed2006

The effects of the ketogenic diet in refractory partial seizures with reference to tuberous sclerosis.

PURPOSE: Tuberous sclerosis complex (OMIM 191100) is a multiorgan disease commonly associated with epilepsy refractory to anticonvulsants. Individual reports indicate that seizures in children with tuberous sclerosis might benefit from a ketogenic diet. We studied the effects of the diet introduced at 3.5 years of age in three boys with tuberous sclerosis and refractory partial seizures. METHODS: On admission a classical LCT ketogenic diet was initiated and patients were followed for 12 months. Antiepileptic drugs were maintained unless adverse effects required reduction. RESULTS: Two patients became seizure-free within 2 months on the diet. In the third patient drop attacks decreased significantly. On follow-up the diet was well accepted and without adverse effects. CONCLUSION: The ketogenic diet should be considered as a treatment option for children with tuberous sclerosis and partial seizures refractory to anticonvulsants. Our data support the need for further studies in larger cohorts to confirm the effectiveness of the ketogenic diet in this entity.

Anticonvulsants↗
PubMed2004

Phase-locking characteristics of limbic P3 responses in hippocampal sclerosis.

Amplitudes of the P3 recorded invasively from the medial temporal lobe (MTL-P3) have been reported to be reduced on the side of a mediotemporal epileptogenic focus. This reduction has been attributed to the massive cell loss within the hippocampus associated with hippocampal sclerosis. It has remained unclear how functional connectivity between the hippocampus and rhinal cortex, as well as within the hippocampus, is altered in hippocampal sclerosis. To investigate this issue, we analyzed to what extent stimulus-related phase-locking and power changes within the low-frequency range (2-30 Hz) and within the gamma band (32-48 Hz), as well as rhinal-hippocampal phase synchronization contribute to the averaged MTL-P3 potentials. Event-related responses were recorded via bilateral depth electrodes in epilepsy patients with unilateral hippocampal sclerosis, who performed a visual oddball experiment. On the contralateral (nonsclerotic) side, successful target detection was associated with an increase of power and phase locking of hippocampal activity in both the low-frequency range and in the gamma range. Besides, there were rhinal-hippocampal synchronization enhancements in the theta and gamma range. On the ipsilateral (sclerotic) side, the event-related power increase in the low-frequency range had almost disappeared, a finding likely to be explained by the loss of principle neurons. However, low-frequency phase-locking, rhinal-hippocampal synchronization, as well as event-related power changes in the gamma range persisted ipsilaterally, although there were differences in temporal and spectral characteristics. These findings support the hypothesis that functional connectivity between hippocampus and rhinal cortex, as well as intrahippocampal connectivity, are partially preserved in hippocampal sclerosis.

Adult↗
PubMed2004

Epilepsy surgery for tuberous sclerosis.

Tuberous sclerosis complex is often associated with medically refractory epilepsy secondary to cortical tubers. Previous studies have identified an association between early seizure onset, greater seizure burden, and mental retardation in childhood. Early effective seizure control could therefore significantly reduce the adverse developmental effects of chronic epilepsy in tuberous sclerosis complex. In medically intractable epileptic patients, surgical treatment has been demonstrated to reduce or abolish seizures and the associated burden for the child and its environment. Epilepsy surgery has not been widely used in patients with tuberous sclerosis complex thus far, mostly because of the concern that the multifocal nature of the cortical lesions could be associated with the development of independent epileptogenic zones. Advances in electroencephalographic techniques, functional neuroimaging, and invasive cortical mapping are changing this view and allowing an increased number of tuberous sclerosis complex patients to be evaluated for resective surgery. Additional techniques emerging in the neurosurgical field may add further treatment options to the current state of the art.

Anticonvulsants↗
PubMed2006

New proteins configure a brain drug resistance map in tuberous sclerosis.

Epileptogenic cortical tubers, characterized by dysplastic neurons and balloon cells, is a frequent feature of tuberous sclerosis. In severe tuberous sclerosis-affected individuals, seizures are refractory to medication. Multidrug resistance proteins (multidrug resistance protein-1 [MDR-1] and multidrug resistance-associated protein-1 [MRP-1]) have been found to be highly expressed in epileptogenic cortical tubers. However, two new proteins related to refractoriness in cancer (breast cancer resistance protein and major vault protein) have not been investigated in tuberous sclerosis and refractory epilepsy. On the same brain specimens previously describing the MDR-1 and MRP-1 expression, we investigated retrospectively breast cancer resistance protein and major vault protein by specific monoclonal antibodies and routine immunohistochemistry methods. Breast cancer resistance protein was present in vascular endothelial cells from all the vessels examined in 3 of 3 cases. Major vault protein was detected in only one case, and selectively expressed in several but not all ballooned cells. In epileptogenic cortical tubers, the additional expression of breast cancer resistance protein on vessels and major vault protein in some ballooned cells to the previously demonstrated expression of MDR-1 and MRP-1 (in vessels, astroglia, microglia, neurons, and ballooned cells) configures a brain protein pharmacoresistance map from patients with tuberous sclerosis and refractory epilepsy.

ATP Binding Cassette Transporter, Subfamily B, Mem↗