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Acute Disseminated Encephalomyelitis: A Gray Distinction
Amal Abu Libdeh1, Howard P Goodkin1, Denia Ramirez-Montealegre1
1Division of Pediatric Neurology, Department of Neurology, University of Virginia, Charlottesville, Virginia.
Background:
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory acquired demyelinating syndrome predominantly affecting the white matter of the central nervous system.
Methods:
We describe a three-year-old boy whose clinical presentation was suspicious for ADEM but whose initial imaging abnormalities were confined to the deep gray matter (without evidence of white matter involvement). His clinical course was fluctuating and repeat imaging one week after presentation demonstrated interval development of characteristic white matter lesions.
Results:
Treatment with adjunctive intravenous immunoglobulin and high-dose corticosteroids resulted in significant clinical improvement.
Conclusions:
Isolated deep gray matter involvement can precede the appearance of white matter abnormalities of ADEM, suggesting that repeat imaging is indicated in individuals whose findings are clinically suspicious for ADEM but who lack characteristic imaging findings.
Insights
Acute disseminated encephalomyelitis (ADEM) can initially present with gray matter involvement before white matter lesions appear. Early recognition and treatment with immunotherapy led to significant improvement in a pediatric case.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory demyelinating syndrome.
- ADEM predominantly affects the white matter of the central nervous system.
Observation:
- A three-year-old boy presented with symptoms suggestive of ADEM, but initial brain imaging revealed abnormalities exclusively in the deep gray matter, with no white matter involvement.
- The patient experienced a fluctuating clinical course.
- Repeat imaging performed one week later showed the development of characteristic white matter lesions, consistent with ADEM.
Findings:
- The case demonstrates that isolated deep gray matter involvement can be an initial manifestation of ADEM.
- This presentation preceded the development of typical white matter lesions in the patient.
- Treatment with high-dose corticosteroids and intravenous immunoglobulin resulted in substantial clinical improvement.
Implications:
- Clinicians should consider ADEM in cases with initial isolated deep gray matter abnormalities, even without concurrent white matter lesions.
- Repeat neuroimaging is crucial for patients with clinical suspicion of ADEM but lacking characteristic initial imaging findings.
- This case highlights the importance of serial imaging in diagnosing ADEM and guiding timely therapeutic interventions.
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