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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
PubMed
Abstract

Insights

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.
Keywords:
acute disseminating encephalomyelitisaphasiacerebellar mutismdentate nucleus

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