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Cerebellar mutism in acute disseminating encephalomyelitis
Sarah McAndrew1, Robert Listernick2, Nancy Kuntz1
1Department of Pediatrics, Northwestern University, Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, Illinois.
Pediatric Neurology
|March 25, 2014
Summary
Acute disseminated encephalomyelitis (ADEM) can cause rare cerebellar mutism in children, leading to long-term cognitive and language deficits. This case highlights ADEM
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Neuroradiology
Background:
- Cerebellar mutism is a known complication following posterior fossa tumor resection in children.
- Cerebellar mutism associated with acute disseminated encephalomyelitis (ADEM) is exceptionally rare.
- ADEM typically presents as a post-infectious or post-vaccination demyelinating process.
Observation:
- A 7-year-old boy presented with acute onset fever, vomiting, diarrhea, altered mental status, and expressive aphasia.
- Neuroimaging revealed new diffusion restriction in the bilateral dentate nuclei and right cerebellum.
- The patient experienced initial improvement in verbalization with treatment.
Findings:
- The case demonstrates a rare presentation of cerebellar mutism secondary to ADEM.
- Cerebellar involvement in ADEM can result in significant expressive aphasia and cognitive impairment.
- Long-term language and cognitive difficulties were observed despite initial treatment response.
Implications:
- ADEM, though often benign, can precipitate severe and persistent neurological deficits affecting the cerebellum.
- This case underscores the importance of considering ADEM in the differential diagnosis of pediatric cerebellar mutism.
- Early recognition and management of ADEM are crucial to mitigate potential long-term neurodevelopmental consequences.
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