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Progressive Disordered Movements in an Infant Leads to Rare Diagnosis

Sarah Pasquale1, Aaron Dam1, Christopher Kelly1

  • 1New York Methodist Hospital, Department of Emergency Medicine, Brooklyn, New York.

Clinical Practice and Cases in Emergency Medicine
|June 1, 2018
PubMed

Insights

Desmoplastic infantile ganglioglioma (DIG), a rare brain tumor, was diagnosed in an infant presenting with seizures and movement disorders. Complete surgical resection led to an excellent outcome with no neurological deficits.

Area of Science:

  • Pediatric Neuro-oncology
  • Neuroscience
  • Developmental Neuroscience

Background:

  • Desmoplastic infantile ganglioglioma (DIG) is an exceptionally rare supratentorial tumor, accounting for less than 0.1% of central nervous system tumors.
  • Approximately 60 cases of DIG have been documented in medical literature, highlighting its rarity.

Observation:

  • A 3-month-old infant presented with progressive neurological symptoms including stiffening, eye blinking, drooling, and altered alertness.
  • Seizure activity was observed, prompting urgent imaging studies.

Findings:

  • Magnetic resonance imaging (MRI) revealed a large (8.6cm × 7.9cm × 5.1cm) solid and cystic mass in the temporal region.
  • Post-surgical pathology confirmed the diagnosis of Desmoplastic Infantile Ganglioglioma (DIG).

Implications:

  • Complete surgical resection of DIG can lead to excellent post-operative outcomes.
  • Infants with DIG can achieve expected developmental milestones after successful treatment.

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