Acute disseminated encephalomyelitis
| Acute disseminated encephalomyelitis | |
| ICD-10 | G04.0 |
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| ICD-9 | 323.61 |
| DiseasesDB | 158 |
| MeSH | D004673 |
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Acute disseminated encephalomyelitis Microchapters |
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Differentiating Acute disseminated encephalomyelitis from other Diseases |
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Risk calculators and risk factors for Acute disseminated encephalomyelitis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: ADEM
History and Symptoms
Presentation
It has an abrupt onset and a monophasic course. Symptoms usually begin 1-3 weeks after infection or vaccination. Major symptoms include fever, headache, drowsiness, seizures and coma. Although initially the symptoms are usually mild, later in the course of the disease patients may even die, if they are not treated properly. Some patients recover completely, while others have permanent neurological impairments.
- The disease presents with progressive, often abrupt, neurologic deterioration. The clinical course is quite variable, and in some cases, rapid progression occurs over hours to days.
- ADEM classically follows a monophasic course, though there are now discussions in the literature about “multiphasic ADEM”
- Features include:
- Somnolence, confusion, lethargy
- Fever – that had previously resolved if there was a precipitating illness
- Headache
- Meningismus
- Motor features may include ataxia, myoclonic movements, and choreoathetosis
- Seizures
- Decerebrite rigidity may develop in severe cases
- Paraplegia, quadraplegia, absent deep tendon reflexes (DTRs), sensory levels, and bladder or bowel involvement suggest prominent spinal cord involvement
- Coma
- Signs of disseminated neurologic disease are usually present
- Motor findings:
- Hemiparesis
- Quadriparesis
- Extensor plantar responses
- DTR’s may be lost initially, and later become hyperactive
- Sensory findings are commonly present
- Brainstem involvement may be present
- Cerebellar involvement is particularly prominent in ADEM that occurs as a consequence of chickenpox
- Motor findings:
- In post-exantham disease, the rash usually appears 2-4 days before the neurologic manifestations, and is typically fading at the onset of neurologic disease.
- Though most cases demonstrate widespread disease (brain, spinal cord, optic nerves, etc), neurologic features may be limited to spinal cord features (transverse myelitis), cerebellar features, etc.
Laboratory Findings
Electrolyte and Biomarker Studies
- CSF protein is usually modestly elevated (50-150 mg/dl)
- CSF lymphocytic pleocytosis is present, usually <200 cells/ul.
- Serum white count may be normal or elevated
MRI and CT
- Radiographic features:
- MRI
- Extensive gadolinium enhancement of white matter of the brain and spinal cord
- Often extensive and relatively symmetric, often also involving the posterior fossa
- Most lesions enhance with gadolinium, suggestive that all lesions are active, and that the disease is therefore monophasic.
- CT is less sensitive and is sometimes falsely negative
- MRI
Risk Stratification and Prognosis
- Prognosis varies with the severity of the neurologic disease. Some patients will not survive the acute illness, and some will be left with neurologic sequelae. Some remarkably recover completely.
- The disease is characteristically monophasic, but descriptions of multiphasic disease have been reported.
- Affected children will often suffer from persistent seizures and behavioral and learning disorders. Adults are somewhat less likely than children to have neurologic sequelae.
Treatment
- There are case reports of improvement with treatment with plasma exhange and intravenous immunoglobulin in patients who have not responded to steroids.
- The first treatment is usually steroids and intensive care is often required.
Pharmacotherapy
Acute Pharmacotherapies
- Most patients are treated with pulse intravenous methylprednisolone with subsequent taper.
Acknowledgements
The content on this page was first contributed by: ELLISON L. SMITH, M.D.
Related Chapters
External links
- Acute Disseminated Encephalomyelitis (ADEM)Information page-> including pathogenesis/aetiology/treatment/prognosis at adem.org
- Acute Disseminated Encephalomyelitis (ADEM) at myelitis.org
- Murthy J (2002). "Acute disseminated encephalomyelitis". Neurology India. 50 (3): 238–43. PMID 12391446.
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Multiple sclerosis Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Acute disseminated encephalomyelitis On the Web |
|
American Roentgen Ray Society Images of Acute disseminated encephalomyelitis |
|
Risk calculators and risk factors for Acute disseminated encephalomyelitis |
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