Correspondence

Spinal Epidural Lipomatosis in a Patient with the Ectopic Corticotropin Syndrome

N Engl J Med 1999; 341:1399-1400October 28, 1999

Article

To the Editor:

Spinal epidural lipomatosis, or excess epidural fat, can compress nerve roots or the spinal cord. It occurs in patients with morbid obesity, hypothyroidism, and exogenous Cushing's syndrome1 but rarely in those with endogenous Cushing's syndrome.2-4 We describe a patient with spinal epidural lipomatosis who had the ectopic corticotropin syndrome.

The patient was a 36-year-old man who presented with severe back pain and weakness. He had a cushingoid appearance, weakness of his hip flexor muscles, and bandlike hyperesthesia at level T6 but no other neurologic abnormalities. His serum cortisol concentration, urinary cortisol excretion, and plasma corticotropin concentration were high. Additional tests suggested the presence of a bronchial carcinoid. A lumbar puncture, performed because of episodic confusion, showed normal findings except for a cerebrospinal fluid protein concentration of 620 mg per deciliter, suggesting the presence of a spinal blockage. Magnetic resonance imaging (MRI) showed anterior wedging of multiple midthoracic vertebrae and spinal epidural lipomatosis at T5 through T10, with a 9-mm protrusion of epidural fat and anterior mass effect on the spinal cord (Figure 1AFigure 1T₁-Weighted MRI Scans of the Thoracic and Lumbar Spine in a Patient with Cushing's Syndrome before and after Treatment.).

We considered performing decompressive laminectomy but decided to treat the patient with inhibitors of steroidogenesis (metyrapone and ketoconazole) for two months. After treatment, MRI studies showed that the thickness of the spinal epidural fat was 7 mm. After the removal of the bronchial carcinoid, the plasma corticotropin and serum cortisol concentrations were undetectable. Three months later (five months after the diagnosis of spinal epidural lipomatosis), the patient was asymptomatic, the findings on the neurologic examination were normal, and MRI studies of the thoracic and lumbar spine (Figure 1B) showed that the thickness of the epidural fat was normal (3 mm).

Cushing's syndrome typically causes an accumulation of fat that involves the face, neck, and trunk, and it also causes hypertrophy of adipose tissue normally present in the spinal canal. In one study, MRI evaluation of the thoracolumbar spine showed that the mean thickness of the epidural fat in the sagittal plane was 4.6 mm (range, 3 to 6) in normal subjects, whereas the thickness was more than 6 mm (range, 7 to 15) in patients with spinal epidural lipomatosis.5 The mechanism by which hypercortisolism induces epidural lipomatosis is unclear. We recommend careful neurologic examination of patients with Cushing's syndrome and the performance of spinal MRI studies for the detection of spinal epidural lipomatosis, if there is a neurologic deficit.

Christian A. Koch, M.D.
John L. Doppman, M.D.
Joseph C. Watson, M.D.
Nicholas J. Patronas, M.D.
Lynnette K. Nieman, M.D.
National Institutes of Health, Bethesda, MD 20892

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Citing Articles (15)

Citing Articles

1. 1

   K. Bhatia, E. Frydenberg, T. Steel, M. Ow-Yang, K. Ho, E. Grainger. (2010) Spinal epidural lipomatosis due to a bronchial ACTH-secreting carcinoid tumour. Journal of Clinical Neuroscience 17:11, 1461-1462
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2. 2

   Giulio Zuccoli, Nicolò Pipitone, Nicola Carli, Luigi Vecchia, Stefano C. Bartoletti. (2010) Acute spinal cord compression due to epidural lipomatosis complicated by an abscess: magnetic resonance and pathology findings. European Spine Journal 19:S2, 216-219
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   José De Andrés, Miguel Angel Reina, Alberto Prats. (2009) Epidural space and regional anesthesia. European Journal of Pain Supplements 3:2, 55-63
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4. 4

   Darweesh Al-Khawaja, Kevin Seex, Guy D. Eslick. (2008) Spinal epidural lipomatosis – A brief review. Journal of Clinical Neuroscience 15:12, 1323-1326
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   A Nasoodi, J McAleese, A Grey, S Stranex. (2008) Twisted tail: Spinal epidural lipomatosis responding to chemotherapy in a patient with non-small-cell lung cancer. Journal of Medical Imaging and Radiation Oncology 52:5, 525-526
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   E. H. Pinkhardt, A.-D. Sperfeld, V. Bretschneider, A. Unrath, A. C. Ludolph, J. Kassubek. (2008) Is spinal epidural lipomatosis an MRI-based diagnosis with clinical implications? A retrospective analysis. Acta Neurologica Scandinavica 117:6, 409-414
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   Shahram Izadyar, Justin Y. Kwan, Yadollah Harati. (2006) Myeloneuropathic Presentation of Spinal Epidural Lipomatosis. Journal of Clinical Neuromuscular Disease 7:3, 133-140
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   P. A. Mackowiak, M. Garcia-Lazaro, A. Cano, E. Vidal, A. Rivero, J. M. Kindelan, P. A. Mackowiak. (2006) Pain in the Lower Limbs of a Patient with AIDS. Clinical Infectious Diseases 42:3, 428-430
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   Giles H. Vince, Claudia Brucker, Peter Langmann, Christian Herbold, Laszlo Solymosi, Klaus Roosen. (2005) Epidural Spinal Lipomatosis With Acute Onset of Paraplegia in an HIV-Positive Patient Treated With Corticosteroids and Protease Inhibitor. Spine 30:17, E524-E527
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    Guy R. Fogel, Paul Y. Cunningham, Stephen I. Esses. (2005) Spinal epidural lipomatosis: case reports, literature review and meta-analysis. The Spine Journal 5:2, 202-211
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    Constantin Schizas, Caridad Ballesteros, Pratik Roy. (2003) Cauda Equina Compression After Trauma. Spine 28:8, E148-E151
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    Dominique Dumont-Fischer, Anne-Christine Rat, Nathalie Saidenberg-Kermanac’h, Sylvana Laurent, Régis Cohen, Marie-Christophe Boissier. (2002) Spinal epidural lipomatosis revealing endogenous Cushing’s syndrome. Joint Bone Spine 69:2, 222-225
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    Mildreth Camacho, Bénédicte Mugnier, Catherine Foutrier-Morello, Hubert Roux. (2001) Glucocorticoid-induced spinal epidural lipomatosis. Joint Bone Spine 68:4, 354-359
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    J. R. Ebright, M. A. Stellini, A. C. Tselis. (2001) Spinal Epidural Lipomatosis in a Human Immunodeficiency Virus-Positive Patient Receiving Steroids and Protease Inhibitor Therapy. Clinical Infectious Diseases 32:5, e90-e91
    CrossRef

15. 15

    Christian A Koch, John L Doppman, Nicholas J Patronas, Lynnette K Nieman, George P Chrousos. (2000) Do Glucocorticoids Cause Spinal Epidural Lipomatosis? When Endocrinology and Spinal Surgery Meet. Trends in Endocrinology & Metabolism 11:3, 86-90
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