Cases reported "Multiple Sclerosis"

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1/104. Quantitative follow-up of patients with multiple sclerosis using MRI: technical aspects.

    A highly reproducible automated procedure for quantitative analysis of serial brain magnetic resonance (MR) images was developed for use in patients with multiple sclerosis (MS). The intracranial cavity (ICC) was identified on standard dual-echo spin-echo brain MR images using a supervised automated procedure. MR images obtained from one MS patient at 24 time points in the course of a 1-year follow-up were aligned with the images of one of the time points. Next, the contents of the ICC in each MR exam were segmented into four tissues, using a self-adaptive statistical algorithm. Misclassifications due to partial voluming were corrected using a combination of morphologic operators and connectivity criteria. Finally, a connectivity detection algorithm was used to separate the tissue classified as lesions into individual entities. Registration, classification of the contents of the ICC, and identification of individual lesions are fully automatic. Only identification of the ICC requires operator interaction. In each MR exam, the program estimated volumes for the ICC, gray matter (GM), white matter (WM), white matter lesions (WML), and cerebrospinal fluid (CSF). The reproducibility of the system was superior to that of supervised segmentation, as evidenced by the coefficient of variation: CSF supervised 45.9% vs. automated 7.7%, GM 16.0% vs. 1.4%, WM 15.7% vs. 1.3%, and WML 39.5% vs 52.0%. Our results demonstrate that this computerized procedure allows routine reproducible quantitative analysis of large serial MRI data sets.
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2/104. Dissociation of 'on-line' and 'off-line' visuomotor control of the arm by focal lesions in the cerebellum and brainstem.

    Visuomotor control of the arm was assessed in a single case study of a subject with focal lesions in the cerebellum and brainstem. A dissociation between 'on-line' and 'off-line' visuomotor control was revealed: impairments in 'on-line' visuomotor control included inaccuracy of tracking velocity, increase in spatial pointing variability and a delay in simple reaction time; whereas the patient was able to adapt to a gain change in 'off-line' visual feedback during a pointing task, and his adaptation was less affected than that of control subjects by trial-to-trial random fluctuations in 'off-line' visual feedback. We conclude that focal damage in the cerebellar peduncles may be principally responsible for this dissociation.
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3/104. Demyelination in primate autoimmune encephalomyelitis and acute multiple sclerosis lesions: a case for antigen-specific antibody mediation.

    Neuropathological and ultrastructural features of central nervous system demyelination were compared in marmoset experimental autoimmune encephalomyelitis (EAE) induced with myelin/oligodendrocyte glycoprotein (MOG), and in 3 cases of multiple sclerosis (MS) displaying recent lesions. At the edges of EAE and MS lesions, a zone of myelin vacuolation was common, whereas in the lesion proper, myelin sheaths were consistently transformed into vesiculated membranous networks. These networks became dissociated from axons by cell processes from macrophages. Oligodendrocytes were remarkably spared and evidence of myelin repair was present but not prominent. Axonal pathology was more common in the MS material than in marmoset EAE. Immunocytochemistry, using gold-labeled encephalitogenic peptides of MOG and silver enhancement to detect MOG autoantibodies, revealed the presence of MOG-specific autoantibodies over vesiculated myelin networks. gold-labeled antibody to IgG also gave a positive reaction. gold-labeled peptide of myelin basic protein did not react with MOG/EAE tissue, but the same conjugate gave positive staining in MS (and in marmoset EAE induced by whole white matter), perhaps indicating broader spectrum immunoreactivity or sensitization to myelin antigens. Thus, vesicular disruption of myelin was a constant feature in these evolving, highly active lesions in primate EAE and MS and appeared causally related to the deposition of antigen-specific autoantibodies.
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4/104. Psychological factors in the etiology of ulcerative colitis: objectlessness and rage.

    In this second investigation of psychological factors in the etiology of ulcerative colitis, the author again utilizes unusual cases characterized by limited variables preceding illness. The first investigation involved several patients whose partial deafness antedated their illness. A significant point was that prior to developing their illness, all these patients had arrived at a state of objectlessness which was abetted by the deafness. This report presents four ulcerative colitis patients with pre-existing organic problems affecting their mental status. Two of the patients are severely mentally retarded. A third patient has rapidly advancing multiple sclerosis, and a fourth--actually an addition to the earlier study--has long-standing partial deafness. Aside from the objectlessness which was also prominent in the earlier group, all four patients demonstrate a consistent pattern of vicious self-directed rage. This paper takes into account the interaction between the rage and the state of objectlessness in the production of illness.
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5/104. A nursing practice model for chronic illness.

    As the 21st century approaches, one very important issue for nursing is the increased prevalence of chronic conditions such as arthritis. Self-management is one way that individuals can cope with the uncertainty and many changes chronic illness brings. The purpose of this article is to describe a nursing model that addresses the need for enhanced self-management skills and the rehabilitation nurse's role in this process. The model is based on the concept of intentional action and uses Orem's (1995) self-care deficit nursing theory as its conceptual structure. The expected outcomes of care for clients are effective self-management skills and subsequently improved health status and quality of life. The model will be useful in the design of rehabilitation nursing research as well as in the planning of care for individuals with chronic illness.
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6/104. Detailed analysis of the oligodendrocyte myelin glycoprotein gene in four patients with neurofibromatosis 1 and primary progressive multiple sclerosis.

    neurofibromatosis 1 (NF1) is a common autosomal disorder with a wide range of neurological manifestations. The case histories of five patients, including two siblings, are reported who have both neurofibromatosis 1 and primary progressive multiple sclerosis (PPMS). A further patient with both NF1 and PPMS has since been identified. More recently, a systematic clinical review of 138 unselected adult patients with NF1 identified one patient with a slowly progressive spastic paraparesis and multiple high signal hyperintensities on T2 weighted MRI. Molecular genetic studies suggest a mechanism by which the clinical association of progressive white matter disease and NF1 might arise. The gene for NF1 is located on chromosome 17q, spans 350 kb of genomic dna, and contains 60 exons. The gene for oligodendrocyte myelin glycoprotein (OMgp) is embedded within intron 27b of the NF1 gene. OMgp is a membrane glycoprotein that appears in the human CNS at the time of myelination. It can be detected immunohistochemically on CNS myelin and on the surface of cultured oligodendrocytes. Structurally, OMgp has the potential to function as an adhesion molecule and could contribute to the interactions between the plasma membranes of oligodendrocytes and axons required for myelination and/or axon survival. This study considers the specific hypothesis that PPMS in patients with NF1 results from concurrent mutation of the OMgp gene. The OMgp genes of four unrelated patients with NF1 and PPMS were examined using a combination of Southern blot, dosage polymerase chain reaction, and chemical cleavage of mismatch. The entire OMgp coding sequence, all intronic sequence, the intron-exon boundaries, and 1 kb of flanking sequence were screened. The dna from two patients was found to contain an alteration in the OMgp gene resulting in an amino acid change of glycine to aspartic acid at codon 21. It is concluded that PPMS in patients with NF1 can occur without concurrent mutation of the OMgp gene. The glycine to aspartic acid polymorphic alteration at codon 21 is neither sufficient nor necessary for the development of PPMS.
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7/104. Middle-ear myoclonus.

    tinnitus produced by repetitive contraction of the middle-ear muscles is a rare condition. We present an interesting case of bilateral middle-ear myoclonus causing incapacitating tinnitus in a patient with multiple sclerosis. Otological examination demonstrated rhythmic involuntary movement of the tympanic membrane. These movements correlated with a rhythmic 'rushing wind' noise perceived by the patient. Oropharyngeal examination showed no evidence of palatal myoclonus. Impedance audiometry confirmed rhythmic change in the middle-ear volume. Medical management was unsuccessful. The patient's tinnitus was subsequently cured with bilateral sectioning of the tensor tympani and stapedial tendons.
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8/104. New therapeutic perspectives for demyelinating retrobulbar optic neuritis.

    In patients with demyelinating retrobulbar optic neuritis (RON), a spontaneous or corticosteroid-induced improvement is generally observed within the first month, but this is clinically insignificant in 5%-7% of patients. We report the case histories of four patients who were considered to be "non-responders" to corticosteroids because their visus remained unchanged or had improved by only 1/10 after one month from intravenous corticoid therapy begun 2-7 days after disease onset, and who were therefore subsequently administered high intravenous doses of immunoglobulin. Three of these patients completely recovered in a period of 3-9 months; the fourth showed only a partial improvement, but this was consolidated after long-term continuation of the same therapy. These cases suggest the possible efficacy of early administration of intravenous immunoglobulin in RON patients who fail to respond to cortisone therapy. As recently demonstrated in animal models, it can be hypothesised that the result is due to immuno-mediated mechanisms of action that reduce autoimmune responses in the short- and medium-term, and in the long-term favour remyelination.
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9/104. intention myoclonus of multiple sclerosis, its patho-anatomical basis and its stereotactic relief.

    The typical multiple sclerosis case considered here is especially informative from both the standpoint of its clinical course and on the basis of the autopsy findings. The foci responsible for the severe bilateral intention myoclonus of the trunk and limbs are the nerve cell losses in both red nuclei due to extensive and almost complete demyelination. Thereby the triangle of Mollaret between the red nucleus, inferior olives and dentate nucleus is involved as the patho-physiological circuit responsible for myoclonus. Stereotactic coagulation of dentato-thalamic fibres resulted in complete relief of intention myoclonus. With regard to the triggering of fresh demyelinating foci by stereotactic interventions, our point of view is as follows: Although a stereotactic operation introduces the possibility of triggering new demyelinating foci in less than 10% of the cases, such a possibility does not represent an absolute contra-indication to the stereotactic treatment of action myoclonus in multiple sclerosis, if the patient is informed accordingly.
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10/104. Anaphylactic reactions after therapeutic injection of mistletoe (viscum album L.).

    mistletoe (viscum album) is a plant that is semiparasitic of several trees: apple, oak, pine trees, etc. Because of the probable cytolytic action of one of the leaf's most abundant composites, in some countries mistletoe is used as a complementary medicine. Although only a few adverse reactions have been noted (cephalea, fever), cases of anaphylactic shock have been described. We present three cases of severe reaction after injection of mistletoe extract. Two of the patients had cancer. The third, whose brother had cancer, used the plant for preventive purposes. We discuss the danger of possible severe reactions due to the use of products employed in so-called alternative therapies.
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