Fibromatosis of dura presenting as infantile spasms

Area of Science:

• Neuroscience
• Pediatric Neurology
• Oncology

Background:

• Infantile spasms often indicate severe underlying neurological conditions with poor prognoses.
• Emprosthotonic posturing and hemiparesis are significant neurological signs in infants.

Observation:

• A 6-month-old male infant presented with emprosthotonic infantile spasms and right hemiparesis.
• A CT scan identified a large mass near the right temporal horn.
• Surgical exploration revealed a hard, white tumor of the tentorium cerebelli.

Findings:

• Microscopic and ultrastructural analysis confirmed the tumor as fibromatosis with invading myofibroblasts.
• The tumor was incompletely resected due to its nature and location.
• The patient experienced an excellent clinical recovery and remained well one year post-surgery.

Implications:

• This case suggests fibromatosis as a potential, treatable cause of infantile spasms and hemiparesis.
• Aggressive investigation and management of infantile spasms can lead to favorable outcomes.
• Early diagnosis and intervention are crucial for improving prognosis in pediatric neurological disorders.