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1/18. apoptosis of T lymphocytes in acute disseminated encephalomyelitis.

    apoptosis has been shown to be an efficient mechanism involved in clearance of T lymphocytes from the brains of animals with acute experimental autoimmune encephalomyelitis (EAE), an animal model for human multiple sclerosis. In this report we describe a case of acute disseminated encephalomyelitis following general measles infection. In this disease, which closely mimics the pathology of acute EAE we found a high percentage (30%) of apoptotic T cells. This indicates that in both rodent and human brain clearance of T cell-mediated inflammation follows similar mechanisms.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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2/18. An 11-year-old girl with syndrome of inappropriate antidiuretic hormone secretion.

    An 11-year-old girl presented with a syndrome of inappropriate antidiuretic hormone secretion, which was transitory and, initially, of obscure origin. Subsequently, the patient's hypothalamic disorder emerged as a component of a steroid-responsive relapsing encephalomyelitis with cerebral pathology restricted to the basal ganglia and brainstem. Where such a disorder fits in the spectrum from acute disseminating encephalomeylitis to multiple sclerosis is discussed.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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3/18. Acute disseminated encephalomyelitis associated with poliomyelitis vaccine.

    A 6-year-old female patient with acute disseminated encephalomyelitis associated with poliomyelitis vaccine virus is reported. She had a history of high fever, headache, and gait disturbance. neurologic examination confirmed spastic triparesis, urinary incontinence, diminution of tactile sensation, and vision deterioration. Hemography, serum laboratory findings, and urinalysis were normal. The cerebrospinal fluid was clear, with normal pressure, 9 leukocytes/mm(3), and 27 mg/dL protein, but the myelin basic protein was elevated to 10.7 ng/mL. T(2)-weighted magnetic resonance imaging disclosed multifocal high-intensity lesions of the spinal cord. The serum polio virus type 2 antibody titer was raised in the acute phase, and polio vaccine virus type 2 was detected in viral cultures of the cerebrospinal fluid and pharynx swab and had undergone an A-G neurovirulence mutation at nucleotide 481. Finally, she had human leukocyte antigen (HLA)-Cw3 and HLA-DR2, to which multiple sclerosis is related in japan. Thus the cause of ADEM may have been related to her HLA type.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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4/18. Sequential MR studies of a patient with white matter disease presenting psychotic symptoms: ADEM versus single-episode MS.

    Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) are both demyelinating white matter disorders. It is difficult to differentiate ADEM from single episode MS because of the similar clinical presentation, cerebral spinal fluid (CSF) analysis, histological finding and magnetic resonance image (MRI) appearance. We report an ADEM case with unusual clinical presentations of predominant psychiatric symptoms, and relatively long disease course. Initially, we were not able to distinguish it from single episode MS. By means of sequential MR images followed up 2 years and spectroscopy studies, and the dramatic clinical improvement after corticosteroid therapy, ADEM was diagnosed as the disease entity of this patient. In this case report we will present the MR findings of this patient and discuss the differentiation between the ADEM and MS.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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5/18. aphasia as a rare presentation of monosymptomatic demyelinating disease: case report and review of the literature.

    We present a case of sudden-onset aphasia due to a single pathological lesion, which at neuroradiological imaging studies was suggestive of glioma, while on biopsy proved be of demyelinating nature. Every cause of demyelinating lesions of the central nervous system was considered in the differential diagnosis, concluding for a primary demyelinating disease. The clinical and radiological differences between multiple sclerosis and acute disseminated encephalomyelitis are discussed. Although aphasia has already been described in demyelinating diseases, we underline its rarity as onset symptom.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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6/18. Schilder's disease: case study with serial neuroimaging.

    Schilder's myelinoclastic diffuse sclerosis is a rare sporadic demyelinating disease that usually affects children between 5 and 14 years old. The disease often mimics intracranial neoplasm or abscess. We report a 9-year-old girl with Schilder's disease who presented with left hemiparesis. Cranial computed tomography and magnetic resonance imaging (MRI) showed large lesions in the subcortical white matter of the occipital and parietal lobes of both hemispheres that were indistinguishable from an abscess. A cerebrospinal fluid oligoclonal band test was found positive. A diagnosis of acute disseminated encephalomyelitis was then suspected. Serial MRI examinations revealed regression of parenchymal lesions, but there were new developing corpus callosum lesions. After 2 months, the patient presented with right-sided visual loss. MRI examination revealed a right optic nerve lesion. The patient had a dramatic clinical response to corticosteroid therapy. Subsequent control MRI examination revealed regression of both lesions. At 24 months of observation, the patient continued to do well without any complaints or neurologic sequelae.
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ranking = 0.029555819505007
keywords = sclerosis
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7/18. In situ cytokine immune responses in acute disseminated encephalomyelitis: insights into pathophysiologic mechanisms.

    Acute disseminated encephalomyelitis (ADEM) is thought to be an autoimmune disorder of the central nervous system in which myelin is targeted. Pathological studies on closely related human diseases (eg, multiple sclerosis) and on animal models for these demyelinating disorders have suggested the involvement of cytokines. Studies on peripheral immunocytes and on cerebrospinal fluid revealed the presence of cytokine-mediated responses in ADEM. We carried out this neuroimmunopathologic exploration and report for the first time the in situ expression of "inflammatory" cytokines in ADEM. Moreover, we note a particular spatial and molecular pattern whereby tumor necrosis factor-alpha and interleukin (IL)-1beta are intensely expressed, whereas IL-6 is absent. Differential expression at different levels of the neuraxis was also noticed. Our findings suggest that these cytokines, reported to be toxic to myelin, are implicated in the molecular cascade, resulting in the neural damage. These observations might provide insights into molecular pathways involved in the immunopathogenesis of ADEM and might open new horizons in neuroimmunomodulation and anticytokine treatment.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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8/18. Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis.

    We present a case in which human coronavirus was detected in the cerebrospinal fluid of a child presumed to have acute disseminated encephalomyelitis. In murine models, coronavirus has been found to cause a chronic demyelinating condition that resembles multiple sclerosis. Additionally, there is in vitro evidence of human coronavirus's ability to infect neural cells. This case report provides additional support for the hypothesis that coronavirus may be an important etiologic factor in the pathogenesis of demyelinating disease in humans.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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9/18. 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.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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10/18. Sudden onset aphasic hemiplegia: an unusual manifestation of disseminated encephalomyelitis.

    The association of the sudden onset of aphasia with hemiplegia, hemisenosry defect, and facial palsy, with MRI evidence of white matter lesions, requires differentiation between multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). We have observed eight patients with such a syndrome, all of whom were originally diagnosed as multiple sclerosis, but who, on closer examination, turned out to be instances of disseminated encephalomyelitis. The patterns of demyelination seen in T2-weighted MRI are quite different in both conditions. In two of our patients, MRI reverted to normal after the treatment; in others, the images remained unchanged. A review of the reported cases of multiple sclerosis presenting with the acute onset of aphasia, reveals that the majority of them are, in reality, instances of acute disseminated encephalomyelitis with a much better prognosis. Most of these cases are monophasic and immunomodulatory treatment is inappropriate.
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ranking = 3
keywords = multiple sclerosis, sclerosis
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