Syringomyelia as a complication of Paget's disease. Case report

Area of Science:

• Neurology
• Neurosurgery
• Radiology

Background:

• Paget's disease of the bone is a chronic disorder that can affect the skull.
• Cranial settling and basilar invagination are skeletal abnormalities at the skull base and upper cervical spine.
• Syrinx formation is the development of fluid-filled cavities within the spinal cord.

Observation:

• A patient with Paget's disease of the cranium presented with cranial settling and basilar invagination.
• This condition resulted in compression at the craniovertebral junction.
• A syrinx was identified in the patient's spinal cord.

Findings:

• The mechanism of craniovertebral junction compression in this case is similar to that observed in Chiari malformations.
• Medullospinal compression due to basilar invagination is a potential cause of syrinx formation.
• This case highlights an unusual presentation of syringomyelia secondary to Paget's disease.

Implications:

• Understanding this mechanism can aid in diagnosing and managing syringomyelia in patients with Paget's disease.
• It suggests that skeletal abnormalities at the craniovertebral junction, regardless of the underlying cause, can lead to syringomyelia.
• Further research may explore the prevalence and specific imaging findings of this association.