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At least 19 recordsLinked to original sources
PubMed2003

Acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, and is characterised by multifocal white matter involvement. Diffuse neurological signs along with multifocal lesions in brain and spinal cord characterise the disease. Possibly, a T cell mediated autoimmune response to myelin basic protein, triggered by an infection or vaccination, underlies its pathogenesis. ADEM is a monophasic illness with favourable long term prognosis. The differentiation of ADEM from a first attack of multiple sclerosis has prognostic and therapeutic implications; this distinction is often difficult. Most patients with ADEM improve with methylprednisolone. If that fails immunoglobulins, plasmapheresis, or cytotoxic drugs can be given. Recent literature suggests that a significant proportion of patients with ADEM will later develop multiple sclerosis; however, follow up experience from developing countries does not support this view.

Adult↗
PubMed1998

Myelin basic protein reactive Th2 T cells are found in acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM), a postinfectious illness of the central nervous system (CNS), is thought to be an autoimmune disease. Here, we characterized the cytokines secreted by myelin-reactive T cells generated from patients with ADEM. The frequency of MBP-reactive T cell lines was ten-fold higher in patients with ADEM compared to patients with encephalitis and normal subjects. Whereas there was no significant IFN-gamma secretion, the predominant cytokine secreted by MBP-reactive T cell lines was IL-4 in patients with ADEM. In contrast, IL-4 secretion was only rarely detected in the controls. The presence of high frequencies of MBP-reactive IL-4 secreting T cells in subjects with ADEM during their recovery phase may be similar to myelin reactive IL-4 secreting T cells observed during the spontaneous recovery of animals with EAE.

Antibodies, Viral↗
PubMed2003

Acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a monophasic inflammatory disorder of the central nervous system (CNS). Unlike viral encephalitis, microorganisms do not invade the CNS. Instead, ADEM is a postinfectious disease mediated by auto-reactive cells or molecules. Clinical characteristics of ADEM are consistent with disseminated involvement of the CNS, including encephalopathy and pyramidal, cerebellar, and brainstem signs. Bilateral optic neuritis and transverse myelitis are particularly suggestive of demyelinating diseases such as ADEM. Unlike viral encephalitis, seizures rarely are a prominent symptom. The most useful diagnostic investigation is magnetic resonance neuroimaging that commonly shows multifocal lesions throughout the brain and spinal cord. As ADEM is an immune-mediated disorder, treatment includes immunomodulatory therapies (particularly steroids), although no clinical trials have been performed to define the most efficacious agent. In view of the treatment differences between ADEM and viral encephalitis, being familiar with ADEM is essential for pediatricians managing acute neurological disorders.

Central Nervous System↗
PubMed1999

Pathogenesis, diagnosis, and treatment of acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis is considered a monophasic, inflammatory demyelinating disorder of the central nervous system. A temporal relationship usually exists between the onset of neurologic symptoms and an infection or a vaccination. A viral exanthem facilitates the diagnosis. Some heterogeneity exists with regard to etiology and clinical course of this disease. Immunosuppression is considered the treatment of choice.

Combined Modality Therapy↗
PubMedDate not supplied

Multiple complete ring-shaped enhanced MRI lesions in acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) lesions may or may not enhance with contrast material on contrast T1-weighted magnetic resonance images (T1WI). Enhancing ADEM lesions may show varying patterns of contrast enhancement, which include nodular, diffuse nodular, amorphous, gyral, spotty and incomplete ring-like. Multiple, complete ring-shaped enhanced lesions on contrast T1WI is a rare finding in ADEM. We report such a magnetic resonance imaging (MRI) finding in a 36-year-old female patient with this disease.

Adult↗
PubMed2006

Combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the treatment of acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system that is associated with significant morbidity and mortality. Early recognition of the disease is of paramount importance; however, treatment options are limited because only case reports and small series are available in the literature. We report a case of a 42 year-old previously healthy man, whom we treated successfully with a combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the course of the disease.

Acyclovir↗
PubMed1994

[Acute disseminated encephalomyelitis].

Acute disseminated encephalomyelitis (ADEM) follows certain viral infection or vaccinations, which has a close etiological relationship to experimental autoimmune encephalomyelitis (EAE). Immunological examinations have not been sufficiently carried out on human ADEM except postmeasles encephalomyelitis. The most important pathogenic role in ADEM might be changes of immune regulatory mechanisms by virus infection. Therefore, it would be very useful to investigate cytokine, adhesion molecules and receptors on neural cells and endothelial cells in the central nervous system. Recently, the incidence of postvaccinated encephalomyelitis has reduced. Neurologic complications of immunization have been still reported, however, there is no scientific method to prove that the association is causal or coincidental in an individual case.

Amino Acid Sequence↗
PubMed1997

[Acute disseminated encephalomyelitis].

Acute disseminated encephalomyelitis (ADEM) occurs late in the course of viral infection, mainly exanthematous diseases such as measles, chicken pox, and rubella. Postinfectious encephalomyelitis is characterized by immune-mediated demyelination, which is found in experimental autoimmune encephalomyelitis (EAE), and virus cannot be isolated from the central nervous system. The investigations using animal models infected with. Theiler's virus or measles virus would be very useful for clarifying the mechanisms of demyelination induced by viral infection. Although the incidence of postvaccinal encephalomyelitis has fallen markedly due to development of vaccines, the neurological complications following immunization are still reported. The vaccines prepared from viruses other than live-attenuated viruses and whole-killed organisms have never been proved to induce ADEM.

Animals↗
PubMed2002

Acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating disease of the central nervous system. The true incidence of the disease in India is undetermined and is likely to be more frequent than reported, as the common antecedent events, exanthematous fevers and Semple antirabies vaccination, which predispose to ADEM, are still prevalent. The existing evidence suggests that ADEM results from a transient autoimmune response towards myelin or other self-antigens, possibly via molecular mimicry, or by non-specific activation of auto-reactive T cell clones. ADEM is a monophasic illness with favourable long-term outcome. Involvement of neuroaxis is variable and can be diffuse or multifocal and site restricted. Magnetic resonance imaging (MRI) is highly sensitive in detecting white matter lesions and the lesions described are rather extensive and subcortical in location. Involvement of the deep gray matter, particularly basal ganglia, is more frequent. Oligoclonal bands in CSF are usually absent. No therapy has been established by controlled trials in ADEM. Use of high-dose methylprednisolone, plasma exchange, and IVIG are based on the analogy of the pathogenesis of ADEM with that of multiple sclerosis (MS). Differentiation of ADEM from the first attack of MS is important from prognostic as well as therapeutic point of view. However, in the absence of biological marker, at times differentiation of ADEM from the initial presentation of MS may not be possible even by combination of clinical, CSF analysis, and MRI. This differentiation is more relevant to India where the incidence of MS is low.

Encephalomyelitis, Acute Disseminated↗
PubMed2006

[Acute urinary retention in a child with acute disseminated encephalomyelitis].

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory demyelinating encephalomyelitis with often monosymptomatic abrupt onset, followed by multifocal neurologic symptomatology depending on lesion-site. Diagnosis is made on the basis of characteristic magnetic resonance imaging (MRI) signal alterations. ADEM is sensitive to steroid therapy, immunoglobulins and plasmapheresis, presents usually a monophasic course and disappears completely after 2 or 3 weeks. Resolution of MRI lesions appears usually within 6 months of presentation. We report on a 14-year-old male, admitted to our Emergency Unit because of fever and acute urinary retention with a normal neurological examination. Urinary tract ultrasonography and mictional cystography were normal; electrophysiology showed a mild involvement of the peripheral nervous system and brain and spine MRI revealed disseminated areas of increased signal on T2-weighted sequences suggestive of ADEM. Steroid therapy brought about clinical recovery in a few days. Resolution of lesions on MRI after 4 months and absence of relapses during four-year clinical follow-up confirmed definitive diagnosis. Our case is interesting because, to our knowledge, this is the first literature report with acute urinary retention as predominant symptom in monosymptomatic forms. Another peculiar feature is the absence of associated neurologic symptomatology despite MRI evidence of important brain and spine alterations.

Acute Disease↗
PubMedDate not supplied

Gadolinium enhancement in acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a widespread CNS inflammation that usually follows an infection or vaccination. We present a case of ADEM, which in addition to the typical MR findings, demonstrates gadolinium enhancement of several of the lesions. Since ADEM is usually a monophasic illness, it has been postulated that all lesions might enhance with paramagnetic contrast medium since they would all be expected to be active. Our case demonstrates enhancement of some of the lesions, without enhancement of others.

Acute Disease↗
PubMed1989

Cranial computed tomography in acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory disorder of the nervous system. The appearance of ADEM on cranial CT scans has rarely been reported. The author reports seven cases in two institutions during a period of seven years. Only four of the seven patients had hypodense lesions in the white matter and six patients showed spotty, nodular, or gyral enhancement after contrast injections. The enhancement resolved with steroid therapy, leaving some persistent hypoattenuated areas even after 10 months follow-up, these findings support the dual components of the pathogenesis of ADEM. The vasculitic component may be responsive to steroids, nevertheless the demyelinating or necrotic areas may not, and the latter may be responsible for the sequelae of ADEM.

Acute Disease↗
PubMed2005

Fulminant acute disseminated encephalomyelitis mimicking acute bacterial menigoencephalitis.

Most patients with acute disseminated encephalomyelitis (ADEM) recover quickly under corticosteroid treatment and have a favourable long-term prognosis. We report on a young woman with acute onset of an extensive and solitary white-matter lesion in the left hemisphere. Fever, high pleocytosis and elevated protein in cerebrospinal fluid initially suggested bacterial meningoencephalitis. The patient died from brain herniation despite maximal conservative therapy. Histological changes in necropsy were consistent with the diagnosis ADEM. Treatment options of fulminant ADEM are discussed.

Adult↗
PubMed2006

Scrub typhus-associated acute disseminated encephalomyelitis.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a monophasic demyelinating disease of the central nervous system, typically occurring after infections or vaccinations. To our knowledge, scrub typhus has not been described in association with ADEM. CASE REPORT: A 77-year-old man was admitted with fever, convulsions and an altered level of consciousness. On neurological examination, the patient was stuporous and had nuchal rigidity and left hemiparesis. A generalized tonic-clonic seizure was observed. Serum and cerebrospinal fluid samples were positive for anti-Orientia tsutsugamushi antibody. Despite a 10-day course of parenteral minocycline, his clinical condition deteriorated. Serial cranial magnetic resonance images demonstrated progressively extensive areas of signal hyperintensity on conventional T2-weighted and fluid attenuated inversion recovery sequence images, mainly affecting the periventricular white matter. After administration of intravenous high-dose corticosteroids, the patient had limited improvement. CONCLUSIONS: This is the first identifiable case of ADEM temporally associated with scrub typhus alone.

Aged↗
PubMed2005

Ophthalmologic manifestations in pediatric patients with acute disseminated encephalomyelitis.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an inflammatory syndrome affecting the central nervous system that often is associated with para-infectious causes. Optic neuritis is a common feature of this demyelinating disease, and a paucity of data regarding this entity exists in the ophthalmologic literature. METHODS: We describe 10 pediatric patients with ADEM who presented at our institution between 1995 and 2003. RESULTS: Six of these patients had optic neuritis, with visual acuities in the affected eyes ranging from 20/30 to light perception. Five of those with optic neuritis had bilateral disease. All patients were treated with high-dose methylprednisolone, and 4 of these patients had recovered with a visual acuity of 20/40 or better in the affected eyes. Two patients were lost to follow-up, and final visual acuities were not available. Nine of 10 patients had an identifiable preceding viral illness or immunization. CONCLUSIONS: ADEM is a known cause of optic neuritis in children and should be considered by the ophthalmologist treating this population of patients. All patients in this series were treated with high-dose intravenous steroids followed by an oral steroid taper and/or intravenous immunoglobulin. Final visual outcomes were favorable in all patients.

Administration, Oral↗
PubMed2006

Diffusion-weighted imaging and proton MR spectroscopy in the characterization of acute disseminated encephalomyelitis.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is usually a monophasic illness characterized by multiple lesions involving gray and white matter. Quantitative MR techniques were used to characterize and stage these lesions. METHODS: Eight patients (seven males and one female; mean age 19 years, range 5 to 36 years) were studied using conventional MRI (T2- and T1-weighted and FLAIR sequences), diffusion-weighted imaging (DWI) and proton magnetic resonance spectroscopy (MRS). Apparent diffusion coefficient (ADC) values and MRS ratios were calculated for the lesion and for normal-appearing white matter (NAWM). Three patients were imaged in the acute stage (within 7 days of the onset of neurological symptoms) and five in the subacute stage (after 7 days from the onset of symptoms). RESULTS: ADC values in NAWM were in the range 0.7-1.24 x 10(-3) mm/s2 (mean 0.937 +/- 0.17 mm/s2). ADC values of ADEM lesions in the acute stage were in the range 0.37-0.68 x 10(-3) mm/s2 (mean 0.56 +/- 0.16 mm/s2) and 1.01-1.31 x 10(-3) mm/s2 (mean 1.24 +/- 0.13 mm/s2) in the subacute stage. MRS ratios were obtained for all patients. NAA/Cho ratios were in the range 1.1-3.5 (mean 1.93 +/- 0.86) in the NAWM. NAA/Cho ratios within ADEM lesions in the acute stage were in the range 0.63-1.48 (mean 1.18 +/- 0.48) and 0.29-0.84 (mean 0.49 +/- 0.22) in the subacute stage. The ADC values, NAA/Cho and Cho/Cr ratios were significantly different between lesions in the acute and subacute stages (P < 0.001, P < 0.027, P < 0.047, respectively). ADC values were significantly different between lesions in the acute (P < 0.009) and subacute stages (P < 0.005) with NAWM. In addition, NAA/Cho and Cho/Cr ratios were significantly different between lesions in the subacute stage and NAWM (P < 0.006, P < 0.007, respectively). CONCLUSION: ADEM lesions were characterized in the acute stage by restricted diffusion and in the subacute stage by free diffusion and a decrease in NAA/Cho ratios. Restricted diffusion and progressive decrease in NAA/Cho ratios may help in staging the disease.

Adolescent↗
PubMed2005

MRI and proton MR spectroscopy in acute disseminated encephalomyelitis.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is one of a group of demyelinating disorders of the central nervous system (CNS). It is said to be attributed to an overshooting immunologic response following an infection or vaccination. The clinical course and type of manifestation is heterogeneous. The early application of corticosteroids has been shown to be beneficial to outcome; thus, an early diagnosis is highly desirable. METHODS: The potential diagnostic value of advanced MR techniques such as proton MR spectroscopy and diffusion-weighted imaging (DWI) was investigated in two paediatric patients with ADEM, one of whom had a remitting and relapsing clinical course and presented with additional cranial nerve involvement. Proton MR spectroscopy revealed typical signs of acute demyelination, such as increased macromolecules, not found in other forms of non-necrotising pathology. CONCLUSION: The addition of proton MR spectroscopy and DWI adds to the diagnostic power of MRI in the setting of post-infectious demyelinating disorders of the CNS or ADEM and may obviate the need for biopsy.

Adolescent↗