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1/35. 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.
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2/35. 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.
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3/35. 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.
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4/35. 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.
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5/35. 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.
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6/35. Presence of oligoclonal T cells in cerebrospinal fluid of a child with multiphasic disseminated encephalomyelitis following hepatitis a virus infection.

    We have investigated the clonality of beta-chain T-cell receptor (TCR) transcripts from the cerebrospinal fluid (CSF) and peripheral blood from a 7-year old child who developed a multiphasic disseminated encephalomyelitis following an infection with hepatitis a virus. We amplified beta-chain TCR transcripts by nonpalindromic adaptor (NPA)-PCR-Vbeta-specific PCR. TCR transcripts from only five Vbeta families (Vbeta13, Vbeta3, Vbeta17, Vbeta8, and Vbeta20) were detected in CSF. The amplified products were combined, cloned, and sequenced. sequence analysis revealed in the CSF substantial proportions of identical beta-chain of TCR transcripts, demonstrating oligoclonal populations of T cells. Seventeen of 35 (48%) transcripts were 100% identical, demonstrating a major Vbeta13.3 Dbeta2.1 Jbeta1.3 clonal expansion. Six of 35 (17%) transcripts were also 100% identical, revealing a second Vbeta13 clonal expansion (Vbeta13.1 Dbeta2.1 Jbeta1.2). Clonal expansions were also found within the Vbeta3 family (transcript Vbeta3.1 Dbeta2.1 Jbeta1.5 accounted for 5 of 35 transcripts [14%]) and within the Vbeta20 family (transcript Vbeta20.1 Dbeta1.1 Jbeta2.4 accounted for 3 of 35 transcripts [8%]). These results demonstrate the presence of T-cell oligoclonal expansions in the CSF of this patient following infection with hepatitis a virus. Analysis of the CDR3 motifs revealed that two of the clonally expanded T-cell clones exhibited substantial homology to myelin basic protein-reactive T-cell clones. In contrast, all Vbeta TCR families were expressed in peripheral blood lymphocytes. Oligoclonal expansions of T cells were not detected in the peripheral blood of this patient. It remains to be determined whether these clonally expanded T cells are specific for hepatitis A viral antigen(s) or host central nervous system antigen(s) and whether molecular mimicry between hepatitis A viral protein and a host protein is responsible for demyelinating disease in this patient.
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7/35. Acute disseminated encephalomyelitis (ADEM) due to mycoplasma pneumoniae infection in an adolescent.

    A 17-year-old boy presented with a severe form of an acute disseminated encephalomyelitis (ADEM) with hemiparesis and coma after initial symptoms of a flu-like febrile infection 1 week previously. Titers against mycoplasma pneumoniae were significantly increased in serum and cerebrospinal fluid (CSF). Detection of M. pneumoniae was achieved in the initial CSF sample using M. pneumoniae-specific PCR. The patient improved significantly on antimicrobial therapy with erythromycin and immunosupressive therapy with immunoglobulins and corticosteroids. This case report demonstrates a well-documented course of a central nervous system (CNS) infection resulting in the ADEM syndrome.
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8/35. Acute disseminated encephalomyelitis associated with hepatitis c virus infection.

    BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system that is frequently preceded by an acute viral infection. This is the first reported case of ADEM associated with hepatitis c virus (HCV) infection. CASE DESCRIPTION: A 46-year-old woman underwent a surgical procedure and received multiple blood transfusions, at which time serologic testing for HCV was negative. Fifty days later, she suddenly developed seizures, alteration of consciousness, right hemiparesis, hemianopsia, and urinary retention. magnetic resonance imaging revealed symmetric multifocal changes on T2-weighted images in the cerebral gray and white matter and in the cerebellar white matter with some lesion enhancement after gadolinium administration. blood testing showed a recent HCV infection with high titer of IgM early antigens and a strongly positive reaction for HCV rna. All other microbiological and virological test results were negative both in serum and in cerebrospinal fluid. Treatment with high-dose dexamethasone was followed by a dramatic improvement of the clinical and magnetic resonance picture. Within a few months the patient recovered completely and there were no relapses during 2 years of follow-up. CONCLUSIONS: infection with HCV is associated with several autoimmune neurological manifestations. It is recommended the patients with ADEM be screened for HCV.
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9/35. Severe depression as an initial symptom in an elderly patient with acute disseminated encephalomyelitis.

    We report a 63-year-old man with acute disseminated encephalomyelitis (ADEM), initially showing depression for one and a half months but subsequently meningoencephalitis followed by acute-onset myelopathy. Neuroradiological examinations of the brain demonstrated no focal lesion causative for his depression, while cerebrospinal fluid revealed elevated levels of inflammatory cytokines in parallel with disease activity. Because depression is usually a rare initial symptom for patients with ADEM, an increased production of inflammatory cytokines in the central nervous system as well as age-related alterations of immune response might have played an important role in the development of depression in this elderly patient.
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10/35. Acute disseminated encephalomyelitis following nephropathia epidemica.

    Acute disseminated encephalomyelitis (ADEM) is an acute monophasic inflammatory and demyelinating disease of the central nervous system (CNS) occurring days to weeks after a virus infection or vaccination. Nephropathia epidemica (NE) is a haemorrhagic fever with renal syndrome caused by puumala virus, with endemic regions in europe, especially scandinavia and Western russia. We describe a case of severe nephropathia epidemica requiring dialysis, followed by severe CNS symptoms caused by ADEM. To our best knowledge this is the first case in the literature in which NE caused ADEM.
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