1/47. Acute disseminated encephalomyelitis (ADEM) after autologous peripheral blood stem cell transplant for non-Hodgkin's lymphoma.Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the central nervous system with an acute clinical onset and a wide variability in severity and outcome. It usually follows a viral infection or an immunization and is thought to be immuno- mediated. We report a case of ADEM with a dramatic clinical onset in an autologous peripheral blood stem cell transplant (PBSCT) recipient for non-Hodgkin's lymphoma who developed the neurologic syndrome 12 days after PBSC reinfusion. This is the first report of ADEM in the setting of autologous PBSCT, a therapeutic procedure performed with increasing frequency in a wide variety of hematologic and solid malignancies.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
2/47. ADEM: literature review and case report of acute psychosis presentation.Acute disseminated encephalomyelitis is a monophasic, immune-mediated disorder that produces multifocal demyelinating lesions within the central nervous system. It is characterized clinically by the acute onset of neurologic abnormalities, including varying degrees of mental state changes ranging from drowsiness to coma. It is unusual for the illness to present as an isolated acute psychosis. The case of a 14-year-old female with biopsy-confirmed acute disseminated encephalomyelitis, who was initially diagnosed with an acute psychiatric disorder, is presented, and published reports on this unusual manifestation are reviewed. A medline database search was performed from 1965 to 1999, using the terms acute disseminated encephalomyelitis, postvaccinal encephalomyelitis, postinfectious encephalomyelitis, and measles encephalomyelitis, combined with the terms psychosis, psychiatric disorder, and behavioral disorder. Selected cross-referenced reports were also reviewed. Nine patients were identified who presented with acute psychosis. We conclude that, although rare, acute disseminated encephalomyelitis can present as an acute psychosis. This immune-mediated condition should be included in the differential diagnosis of neurologic disorders presenting as a psychiatric illness.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
3/47. Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin.Acute disseminated encephalomyelitis (ADEM) is a presumed immune-mediated, demyelinating disease of the CNS for which the standard treatment is high-dose steroids. We describe two patients with ADEM in whom treatment with IV methylprednisolone coincided with deterioration in their clinical status. They were subsequently treated with IV immunoglobulin and exhibited dramatic clinical improvement, with return to their previous level of functioning.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
4/47. encephalomyelitis-associated antimyelin autoreactivity induced by streptococcal exotoxins.OBJECTIVE: After implicating streptococcus pyogenes as causing acute disseminated encephalomyelitis (ADEM) in a child, we wanted to prove that in vivo activation of autoreactive T lymphocytes by superantigens of this Streptococcus contributed to the dramatic demyelination. BACKGROUND: ADEM is a demyelinating disorder of the CNS sharing many similarities with MS. Demyelination in MS is considered to be the result of an autoimmune process mediated by autoreactive T lymphocytes with specificity for myelin antigens. methods: Phenotypic analysis and proliferation assays on blood monocytes, as well as isolation of myelin basic protein (MBP)-reactive T-cell lines/clones; and TCR repertorium analysis by PCR-ELISA and cytokine production. RESULTS: 1) The blood T-cell receptor (TCR) repertoire was compatible with in vivo expansion induced by S. pyogenes exotoxins. 2) TCR expression analysis indicated clonal expansion of CD8 MBP-reactive T cells, suggesting in vivo activation. MBP-reactive T cells showed crossreactivity to S. pyogenes supernatant and exotoxins. 3) Cytokine mRNA quantification of the mononuclear cells revealed a Th2-biased profile. CONCLUSION: In vivo exposure to S. pyogenes may have induced activation of pathogenic myelin reactive T cells, contributing to the dramatic inflammatory demyelination.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
5/47. Acute disseminated encephalomyelitis in a female with hereditary neuropathy with susceptibility to pressure palsy.A 7-year-old female presented with fever, urinary incontinence, mental regression, gait disturbance, and lethargy after diarrhea. magnetic resonance imaging revealed multifocal T(2)-weighted hypersignal lesions supportive of acute disseminated encephalomyelitis. Her mother had been diagnosed with hereditary neuropathy with susceptibility to pressure palsy. The girl was also determined to have hereditary neuropathy with liability to pressure palsy, with a 1.5-Mb deletion in chromosome 17p11.2 encompassing the gene for peripheral myelin protein 22 detected by fluorescent in situ hybridization. Hereditary peripheral neuropathies may be a factor in triggering the autoimmune demyelinating disorder of the central nervous system.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
6/47. chlamydia pneumoniae-associated ADEM.Inflammatory demyelinating diseases are a common cause of neurologic disability in young adults, and usually the cause is unknown. We describe a case of acute disseminated encephalomyelitis (ADEM) associated with chlamydia pneumoniae infection. An 18-year-old previously healthy women, with a one-week history of coryzal illness, was admitted because of progressive headache, dizziness, and a left-sided hemiparesis. MR imaging of the brain and brainstem showed typical signs of ADEM. The diagnosis was established by PCR chlamydia pneumoniae dna positivity in a tracheal swab and by increasing titres of chlamydia IgM antibody. The patient was treated with doxycycline and steroids and recovered completely. Apart from therapeutic implications, this case may contribute to our understanding of demyelinating diseases of the central nervous system.- - - - - - - - - - ranking = 2keywords = demyelinating (Clic here for more details about this article) |
7/47. Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins.Acute disseminated encephalomyelitis is a demyelinating syndrome that occurs infrequently in children. Various treatment modalities, such as plasmapheresis or steroids or intravenous immunoglobulins (IVIG), have been prescribed. The article describes the results of combined IVIG and high-dose steroids given for 3 days in the treatment of a patient with atypical encephalomyelitis. The results suggest that this approach may be more beneficial than the application of either drug alone.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
8/47. recurrence of acute disseminated encephalomyelitis at the previously affected brain site.BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a usually monophasic demyelinating disorder of the central nervous system. Recurrences pose a diagnostic challenge because they can be overlooked or suggest an alternative diagnosis. OBJECTIVE: To examine the frequency, nature, and outcome of recurrent ADEM. DESIGN: review of the medical records of patients diagnosed in our institution as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were defined as appearance of new symptoms and signs at least 1 month after the previous episode. RESULTS: Five (24%) of 21 patients with ADEM developed recurrent disease episodes. In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease episodes, 2 had 3, and the other 2 each had 2. recurrence appeared 1.5 to 32 months after initial presentation and involved the same brain territory in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included neuropsychiatric signs. A good response to corticosteroid therapy was observed in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events and in 7 of 9 recurrences. CONCLUSIONS: Recurrent ADEM may be more prevalent than previously recognized. patients who relapse tend to have more than 1 recurrence that usually involves, clinically and radiologically, a brain territory that was affected before and can simulate a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric features may be the main presentation of a relapse. Since recurrent ADEM is a corticosteroid-responsive condition, awareness and early diagnosis are mandatory.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
9/47. ophthalmoplegic migraine: a recurrent demyelinating neuropathy?The demonstration by magnetic resonance imaging (MRI) scanning of thickening and enhancement of the cisternal part of the oculomotor nerve in patients diagnosed as "ophthalmoplegic migraine" prompts reconsideration of this uncommon disorder. The case histories of five patients, three male and two female, varying in age from 6 to 30 years, are presented here. Recurrent painful ophthalmoplegia started in infancy in two cases, childhood in two instances and adult life in one. One child had his first attacks at 3, 5 and 12 months of age, on each occasion 10 days after an injection of triple vaccine. The possibility of this condition being a recurrent demyelinating neuropathy is considered and its possible relationship to migraine explored.- - - - - - - - - - ranking = 5keywords = demyelinating (Clic here for more details about this article) |
10/47. 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.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
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