1/367. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/367. Cranial polyneuropathies in multiple sclerosis: case report and literature review.Although cranial neuropathies are common disorders in multiple sclerosis patients, multiple cranial nerve involvement is an unusual occurrence. Correlation of clinical symptoms with magnetic resonance imaging evidence of demyelinating central nervous system lesions can confirm the diagnosis. The authors report on the case of a 43-year-old woman who initially was thought to have suffered a brainstem infarct but, in fact, had developed multiple cranial nerve functional deficits. Treatment of multiple sclerosis remains primarily supportive in nature, with corticosteroids used for acute exacerbations and chronic progression.- - - - - - - - - - ranking = 4.2254950243701keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
3/367. 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.- - - - - - - - - - ranking = 1.5455505384908keywords = brain (Clic here for more details about this article) |
4/367. 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.- - - - - - - - - - ranking = 3.8638763462269keywords = brain (Clic here for more details about this article) |
5/367. trigeminal neuralgia triggered by auditory stimuli in multiple sclerosis.OBJECTIVES: To describe a patient with a demyelinating brainstem lesion who developed right-sided trigeminal neuralgia triggered by auditory stimuli and to discuss the pathophysiological mechanisms underlying this unusual phenomenon. DESIGN: Case report. SETTING: Referral center. PATIENT: A 27-year-old man who presented with clinical signs of a brainstem lesion developed right-sided trigeminal neuralgia triggered by auditory stimuli to the right ear. magnetic resonance imaging and electrophysiological studies demonstrated a demyelinating lesion in the pons affecting the right lateral lemniscus and the right trigeminal pathway. This phenomenon completely subsided within 4 days. After a relapse, the diagnosis of clinically definite multiple sclerosis was made. CONCLUSION: Lateral spread of impulse activity within the demyelinating pontine lesion is the likely explanation for the unusual phenomenon of trigeminal neuralgia triggered by auditory stimuli.- - - - - - - - - - ranking = 1.5455505384908keywords = brain (Clic here for more details about this article) |
6/367. Lesion development in Marburg's type of acute multiple sclerosis: from inflammation to demyelination.We report a patient who suffered from acute inflammatory CNS demyelination and underwent two consecutive diagnostic stereotactic brain biopsies during the early disease course. The first lesion was drawn 33 days after the onset of disseminated neurological symptoms. macrophages and T lymphocytes diffusely infiltrated small vessel walls and the white matter. mRNA for tumor necrosis factor alpha (TNFalpha) and inducible nitric oxide synthase (iNOS) was abundantly expressed. Myelin sheaths were entirely preserved. The second biopsy 76 days later showed confluent demyelinating lesions with a diffuse infiltration of macrophages that were positive for myelin debris, activation markers and TNFalpha and iNOS mRNA. IgG and C9neo deposits were found along myelin sheaths. The patient had received intravenous immunoglobulins (IVIG) prior to biopsy. Findings from this single patient affirm that demyelination follows the migration of inflammatory cells from the circulation into the white matter with subsequent inflammation and demyelination. inflammation alone may be sufficient to cause significant clinical deficits without demyelination. Inflammatory mediators such as TNFalpha and NO are involved at very early stages in the pathogenetic process. IVIG treatment may lead to the deposition of immunoglobulins and to the activation of the complement cascade, but the clinical relevance of this particular finding remains uncertain.- - - - - - - - - - ranking = 0.77277526924539keywords = brain (Clic here for more details about this article) |
7/367. tissue plasminogen activator gene expression in multiple sclerosis brain tissue.Recent studies have implicated tissue-type plasminogen activator (tPA) in neurodegeneration. We studied multiple sclerosis (MS) brain tissue for tPA gene and protein expression in comparison with reference tissue, by in situ hybridisation and immunohistochemistry. MS is characterised by demyelination in the central nervous system. In this study, neuronal cell bodies in MS brain showed high expression of tPA mRNA and protein, while in reference brains, staining for protein and mRNA expression were very low in neurons and mostly restricted to blood vessel walls. In MS, there was an additional staining of mononuclear cells within perivascular cuffs and foamy macrophages within demyelinating plaques. In view of evidence that the final process of demyelination in MS is thought to be enzyme-mediated, our work suggests the involvement of tPA and by inference plasmin, in the demyelinating process. Blocking tPA or plasmin activity may be a potentially beneficial therapeutic approach in MS.- - - - - - - - - - ranking = 8.8621466398424keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
8/367. Disappearance of hypointense multiple sclerotic lesions on FLAIR MRI.A child is presented who displayed hypointense multiple sclerotic lesions on fluid-attenuated inversion recovery sequences by magnetic resonance imaging, with the possible pathologic tissue changes of these hypointense lesions evaluated. The magnetic resonance imaging results in this patient demonstrated the disappearance of low-signal lesions on fluid-attenuated inversion recovery in multiple sclerosis, and the improvement of this patient's condition was likely compatible with sequential magnetic resonance imaging findings. Some hypointense lesions in the supratentorial white matter that appear on fluid-attenuated inversion recovery images in multiple sclerosis patients may include reversible brain lesions, suggesting extracellular fluid collection not accompanied by axonal loss or gliosis.- - - - - - - - - - ranking = 0.77277526924539keywords = brain (Clic here for more details about this article) |
9/367. Optic-spinal form of multiple sclerosis and anti-thyroid autoantibodies.The optic-spinal form of multiple sclerosis (OSMS), characterized by recurrent involvement of optic nerve and spinal cord with rare brain magnetic resonance imaging lesions, is relatively common among Asians. While individual cases of OSMS with anti-thyroid autoantibodies (ATABs) have been reported, the frequency of ATAbs in OSMS and classical multiple sclerosis has not been studied. We studied serum ATAbs and anti-nuclear antibodies (ANA) in 46 Japanese patients with multiple sclerosis: 14 with OSMS, and 32 with non-OSMS. Six patients were positive for ATAbs: five women with OSMS and one man with non-OSMS. The frequency of ATAbs in OSMS (5/14) was significantly higher than that in non-OSMS (1/32; P = 0.007), but the frequency of ANA did not differ between OSMS (3/14) and non-OSMS (6/32; P = 0.99). There may be a pathogenetic link between anti-thyroid autoimmunity and a subgroup of OSMS in Japanese.- - - - - - - - - - ranking = 0.77277526924539keywords = brain (Clic here for more details about this article) |
10/367. 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.- - - - - - - - - - ranking = 3.4527197551247keywords = central nervous system, nervous system (Clic here for more details about this article) |
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