Acquired progressive hypotonia in infancy: consider compressive cervical myelopathy

Area of Science:

• Pediatric Neurology
• Congenital Abnormalities
• Spinal Surgery

Background:

• Vertebral anomalies, particularly of the atlas (C1 vertebra), can present with varied clinical manifestations.
• Congenital bony defects of the cervical spine are uncommon causes of pediatric neurological deficits.
• Progressive hypotonia in infants warrants a thorough etiological investigation, including spinal imaging.

Observation:

• A 2-month-old girl presented with feeding difficulties and rapidly progressing hypotonia with diminished upper extremity reflexes.
• Magnetic resonance imaging revealed compressive myelopathy at the C1 vertebral level.
• The infant's symptoms were attributed to a congenital bony defect of the atlas.

Findings:

• Surgical decompression via C1 laminectomy was performed.
• Post-operatively, the patient experienced a complete resolution of neurological symptoms, including hypotonia and reflex deficits.
• This case underscores the link between congenital atlas defects and pediatric myelopathy.

Implications:

• Symptomatic vertebral anomalies causing neurological compression require surgical management.
• Early diagnosis and surgical intervention can lead to favorable neurological outcomes in pediatric spinal cord compression.
• Congenital bony defects of the atlas should be considered in the differential diagnosis of infantile hypotonia with neurological signs.