1/21. 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 = 1keywords = sclerosis (Clic here for more details about this article) |
2/21. Childhood chronic inflammatory demyelinating polyneuropathy with central nervous system demyelination resembling multiple sclerosis.central nervous system demyelination has been described in adults but not in children with chronic inflammatory demyelinating polyneuropathy. We describe a patient with clinical and electrophysiological features consistent with chronic inflammatory demyelinating polyneuropathy who presented at age 5 with an intramedullary spinal cord tumor-like lesion and at age 8, represented with cerebral and spinal demyelinating lesions. Her clinical course and magnetic resonance imaging features were atypical for multiphasic disseminated encephalomyelitis and indistinguishable from multiple sclerosis. To our knowledge, this association has not been previously described in the English literature in childhood.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/21. Acute demyelinating disease. classification and non-invasive diagnosis.Five young patients are described with biopsy-proven acute demyelinating disease. Two cases are classified as Schilder's disease, a particular childhood form of multiple sclerosis (MS) with atypical clinical manifestation, normal or atypical CSF-findings and large bilateral lesions in magnetic resonance imaging (MRI). Two further cases presented with a fulminant bout of MS with typical clinical picture and CSF-findings; they are classified as Marburg's disease. The last case was an acute second bout of classical MS. The biopsy seemed to be justified in 4 cases, but unnecessary in the last case. Localized proton magnetic resonance spectroscopy (MRS) performed in one case allowed us to examine the focal cerebral abnormalities directly and non-invasively. The spectra revealed a pattern typical of acute demyelination suggesting potential for a replacement of biopsy in the future.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
4/21. Modern MRI tools for the characterization of acute demyelinating lesions: value of chemical shift and diffusion-weighted imaging.Acute demyelinating lesions occur in various inflammatory disorders of the CNS. Apart from multiple sclerosis, most cases can be attributed to an overshooting immunological response to infectious agents called acute disseminated encephalomyelitis (ADEM). ADEM, which is mostly characterized by a monophasic course, has a multiphasic variant (MDEM). The early application of corticosteroids has been shown to be beneficial for the outcome; thus, an early diagnosis is highly desirable. Furthermore, the differential diagnosis ruling out neoplastic disorders may be difficult using conventional MRI alone. The potential diagnostic value of advanced MR techniques such as chemical shift imaging (CSI) and diffusion-weighted imaging (DWI) was investigated in a patient with MDEM, who had a new lesion in continuity with the initial disease manifestation. CSI was performed at 1.5 T with a long echo time of 135 ms for the evaluation of N-acetyl-aspartate (NAA) and choline (Cho) and with short TE of 30 ms for macromolecules (mm) and myo-inositol (mI). DWI was performed using a single-shot isotropic EPI sequence. Whereas acute and chronic areas of demyelination were neither distinguishable on T2- nor on contrast-enhanced T1-weighted images, CSI and DWI revealed different metabolite concentrations and diffusion characteristics within the composite lesion, clearly separating acute from chronic areas of demyelination. In conclusion, the addition of CSI and DWI may add to the diagnostic power of MRI in the setting of demyelinating disorders by identifying areas of acute and chronic demyelination, even in the absence of contrast enhancement.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
5/21. Destructive lesions in demyelinating disease.Three cases are presented in which clinical and radiological features suggested the diagnosis of glioma but surgical biopsy revealed a demyelinating process, with tissue destruction and cyst formation in two. One patient had clinically definite multiple sclerosis. Two had probable acute disseminated encephalomyelitis. Treatment with high dose steroids is appropriate when there is clinical or investigative evidence to suggest the presence of demyelinating disease, before deciding on biopsy.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
6/21. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis.Brain MRI was performed on 12 patients with acute disseminated encephalomyelitis (ADEM). Multifocal white matter lesions indistinguishable from those seen in multiple sclerosis (MS) were found in 10. In 5 there were rather extensive symmetric abnormalities in the cerebral (2) or cerebellar white matter (2), or basal ganglia (1). Follow-up MRI after intervals of 2 wks to 18 months demonstrated partial resolution of the abnormalities, but some persisting lesions. New MRI lesions were found at follow-up in only 1 case after an interval of 2 wks. Serial MRI makes a useful contribution to the distinction between MS and ADEM.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/21. connective tissue disease mimicking multiple sclerosis.Systemic lupus erythematosus, primary sjogren's syndrome and systemic sclerosis may be associated with acute transverse myelitis and chronic relapsing neurological syndromes mimicking multiple sclerosis in the same individuals and/or their relatives. We now present three cases which suggest that there is a wide spectrum of connective tissue disorders mimicking multiple sclerosis and acute disseminated encephalomyelitis. These cases demonstrate that the diagnosis of multiple sclerosis should be kept under constant review by searching for the development of connective tissue disorders in the patients or their relatives.- - - - - - - - - - ranking = 1.6keywords = sclerosis (Clic here for more details about this article) |
8/21. magnetic resonance imaging in asymptomatic disseminated vasculomyelinopathy.Two cases of disseminated vasculomyelinopathy (one of acute disseminated encephalomyelitis (ADEM), the other of acute transverse myelitis), are reported because of the persistence, 3 years and 5 months respectively, of abnormalities of magnetic resonance imaging (MRI). These abnormalities remained although in the first case the disease had been essentially asymptomatic from the onset except for one seizure, the patient remaining neurologically intact, whereas in the second case, the patient had made a complete recovery from very serious neurologic dysfunction. The first case illustrates the fact that ADEM may rarely occur without any symptoms, even in the presence of severe imaging abnormalities in both CT and MRI. Neither the persistence of a blood-brain barrier permeability alteration nor gliosis can satisfactorily explain the MRI changes, and thus the pathological significance of areas of increased signal intensity in MRI remains poorly understood and a matter of uncertainty. This report emphasizes the futility of attempting to correlate any kind of clinical observation, laboratory parameter, or effect of therapeutic regimens with changes, or lack thereof, in the MRI in multiple sclerosis and disseminated vasculomyelinopathy.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
9/21. Subtle encephalomyelitis in children: a variant of acute disseminated encephalomyelitis.Four children with chronic, mild, nonspecific symptoms are described in whom magnetic resonance imaging (MRI) showed the presence of multifocal white-matter lesions suggestive of acute disseminated encephalomyelitis. The children ranged in age from 14 months to 15 years. The clinical picture was vague and inconclusive and consisted of several months of headaches, irritability, clumsiness, and personality change. Physical examinations were noncontributory. Laboratory investigation revealed no other cause of the demyelination. All of the patients have done well without any treatment, with a disappearance of symptomatology. The white-matter lesions on MRI scan in these children may indicate subtle exposure to a myelinolytic antigen. It has been suggested that such an exposure may create a state of complete or partial resistance to the encephalitogenic potential of the next infection or immunization. With complete resistance, the patient remains healthy and with partial resistance progressive demyelination results. Verification of these findings by others would suggest a possible benefit of a multicenter study of such patients, with virological, HLA testing, and long-term follow-up, in understanding the etiopathogenesis of multiple sclerosis.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
10/21. Parainflammatory leukoencephalomyelitis: clinical and magnetic resonance imaging findings.Parainflammatory leukoencephalomyelitis is a broad term used to include the spectrum of disorders that affect the central nervous system following infection, immunization, or other noxious stimuli. There is a wide range of clinical and pathologic severity, ranging from acute cerebellar ataxia to acute hemorrhagic leukoencephalopathy. With the improved survival of these patients, magnetic resonance imaging provides a window to the pathologic process, which can aid in the long-term management of these patients. Although lesions of the brainstem and spinal cord correlate well to clinical symptoms, multiple cortical lesions may be present without specific localizing signs. The distribution of magnetic resonance lesions is different from that commonly seen in multiple sclerosis. In some cases, prolonged immunosuppression may be required to prevent recrudescence of the inflammatory response.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
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