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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
Summary
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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