From the Guidelines
Acute Disseminated Encephalomyelitis (ADEM) is a rare inflammatory condition that affects the brain and spinal cord, typically occurring after a viral infection or vaccination, and is treated with high-dose corticosteroids, such as methylprednisolone, as the first line of treatment.
Definition and Treatment
ADEM is characterized by widespread inflammation in the central nervous system, and its treatment usually involves high-dose corticosteroids, typically methylprednisolone 1 g intravenously daily for at least 3–5 days 1. Some key points about ADEM include:
- It is a monophasic condition, meaning it occurs only once, but some patients may experience residual neurological deficits
- Close follow-up is essential to monitor for potential conversion to multiple sclerosis, particularly in cases with recurrent episodes
- Early diagnosis and prompt treatment significantly improve outcomes in ADEM patients
- In severe cases unresponsive to steroids, plasma exchange or intravenous immunoglobulin (IVIG) may be considered as alternative treatments 1
Management and Care
Supportive care is also crucial in managing ADEM, including:
- Management of increased intracranial pressure
- Seizure control
- Rehabilitation therapy to improve functional outcomes It is essential to note that while the exact treatment protocol may vary depending on the individual case, high-dose corticosteroids remain the primary treatment for ADEM, with alternative treatments considered in cases where patients do not respond to initial therapy 1.
From the Research
Definition and Characteristics of Acute Disseminated Encephalomyelitis (ADEM)
- ADEM is an inflammatory demyelinating disease characterized by an acute onset of polyfocal central nervous system (CNS) deficits, including encephalopathy, demonstrating multifocal lesions on MRI 2.
- It is typically a monophasic disorder, but recurrent and multiphasic courses have been described 2.
- ADEM can be preceded by infection or recent vaccination, and it is often seen in children and young adults, although cases in older adults have been reported 3.
Clinical Presentation and Diagnosis
- The clinical presentation of ADEM includes encephalopathy, focal neurological deficits, and rapid neurological decline 2, 3.
- Diagnosis is based on a combination of clinical and radiologic features, including multifocal lesions on MRI, and exclusion of diseases that resemble ADEM 2.
- There is no single test confirming the diagnosis of ADEM, and a broad workup including infectious, immunologic, and metabolic tests is indicated to establish an accurate diagnosis 2.
Treatment Options
- The treatment approach for ADEM consists of intravenous methylprednisolone, followed by an oral corticosteroid taper 2.
- Intravenous immunoglobulin (IVIG) is a therapeutic option in case of insufficient response or contraindications to corticosteroids 2.
- Plasmapheresis should be considered early in the disease course for severe or life-threatening cases of ADEM 2, 4, 5.
- Rituximab has been used as a treatment option in some cases of ADEM, particularly in those associated with infections such as Salmonella 6.