Spinal Epidural Lipomatosis with Osteoporotic Vertebral Fractures Presenting as Acute Thoracic Myelopathy in a 33-Year-Old Man with Cushing Disease.

BACKGROUND

Steroid-related spinal epidural lipomatosis (SEL) is a well-known condition most commonly encountered in patients taking chronic steroid medications. Endogenous Cushing syndrome is a rare cause of SEL. The authors report an unusual presentation of acute thoracic myelopathy due to SEL with osteoporotic vertebral compression fractures in a patient with Cushing disease.

CASE DESCRIPTION: A 33-year-old man presented with thoracic myelopathy due to SEL and multiple vertebral compression fractures. He had Cushingoid clinical features, and investigations revealed a pituitary microadenoma as the cause of hypercortisolism. The diagnosis was missed on multiple occasions before the onset of paraparesis. The patient was treated with urgent thoracic laminoplasty followed by transsphenoidal surgery on the same day. Although normalization of the serum cortisol levels was delayed by 6 months, he had a complete neurologic recovery and remains symptom free at 2 years follow-up.

CONCLUSIONS

This is a rare presentation of Cushing disease and has been reported only twice before in the 1990s. This case highlights the need to keep a high degree of suspicion for development of thoracic myelopathy in a patient with Cushing syndrome who develops thoracic compression fractures associated with SEL. Early recognition of this clinical association and addressing the primary endocrine problem urgently could have prevented spinal cord involvement and the subsequent spine surgery. This scenario is also documented in previous published case reports of this association where the diagnosis of Cushing syndrome has been revealed retrospectively after the development of neurologic symptoms.