Symptomatic epidural lipomatosis in ectopic Cushing's syndrome

Department of Internal Medicine and Endocrinology, Maxima Medical Centre, The Netherlands, PO Box 90052, 5600 PD Eindhoven, The Netherlands.

We report a case of spinal epidural lipomatosis (SEL) caused by ectopic Cushing's syndrome and give a review of the literature. The most common cause of SEL is prolonged therapy with glucocorticoids, only a very few cases are related to endogenous Cushing's syndrome. The pathophysiological mechanism is not clear but there is a possible role for the autonomic nervous system in the stimulation of growth of epidural fat. Severe neurological symptoms which indicate myelopathy and radiculopathy can occur, but there is often a delay in diagnosis because the non-specific initial symptoms are not recognized. The epidural fat is mostly located in the thoracic and lumbar region. Magnetic resonance imaging can establish the diagnosis rapidly. In patients with severe neurological symptoms, surgical decompression of the myelum and removal of the epidural fat is the treatment of choice. Most patients have partial or complete recovery of neurological deficits after surgical treatment or after discontinuing glucocorticoid therapy; mild cases can also be treated conservatively. Routine imaging for the detection of epidural-located lipomatosis in patients at risk is probably useful.