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Acquired progressive hypotonia in infancy: consider compressive cervical myelopathy

I N E Verbeek1, A Vollebregt, F J Halbertsma

  • 1Pediatrics, Máxima Medical Centre, Veldhoven, The Netherlands.

Acta Paediatrica (Oslo, Norway : 1992)
|March 1, 2011
PubMed

Insights

A rare congenital bony defect of the atlas caused progressive hypotonia in an infant. Surgical decompression resolved the neurological symptoms, highlighting the need for intervention in symptomatic vertebral anomalies.

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.
Abstract

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