Endocrine Abstracts

Background: Cushing’s syndrome is rare (1.8-3.2 cases/million population)1, with Cushing’s disease (CD) accounting >70%. We seek to highlight a rare complication, spinal epidural lipomatosis (SEL), rare cause of spinal compression (prevalence-2.5%2), commonly related to exogenous steroid use with 3 reported cases secondary to CD3,4,5.

Case: 36-years old man, background asthma and alcohol excess presented with palpitations. He appeared cushingoid with moon-face, facial plethora, central adiposity and purple abdominal striae and referred to Endocrinology.

Investigations: Unsuppressed cortisol 466nmol/l post overnight dexamethasone suppression test. Raised 24h urinary cortisol concentration (928nmol/day). Low dose dexamethasone suppression test confirmed ACTH dependent CD (Unsuppressed ACTH: 190ng/l) with normal pituitary hormonal profile, MRI pituitary, and CT adrenal. Inferior Petrosal Sinus Sampling (IPSS) revealed central source with left-sided gradient. During investigation & treatment initiation (block and replace therapy: metyrapone 250 mg TDS and dexamethasone 0.25 mg BD.), patient presented with bilateral lower-limb weakness over 4-5 weeks without bowel/bladder symptoms, leading to being wheel chair bound. Examination demonstrated thoracic myelopathy with grade 3/5 power, hyper-reflexia, bilateral clonus and reduced sensations below T10-11 level but preserved perianal sensation. MRI spine demonstrated diffuse excessive epidural fatty tissue posteriorly over thoracic cord, spondylodiscitis (T11-T12) with generalised narrowing of thecal dimension, old superior end plate compression fracture at T11 and significant cord compression in thoracic spine. Diagnosis was paraparesis secondary to SEL. Spinal MDT suggested conservative management with optimization of CD, weight-bearing exercises and DLSO brace. Trans-sphenoidal complete hypophysectomy led to post-operative pan hypopituitarism and commencement on Desmopressin 50 mg BD, Hydrocortisone 20 mg/10 mg and thyroxine 50 mg OD.

Conclusion: SEL a manifestation of metabolic syndrome secondary to CD has significant morbidity and necessitates early diagnosis and treatment. This highlights necessary awareness regarding myelopathy with thoracic compression fractures related to SEL, which may require conservative or surgical management.