Spinal epidural lipomatosis: a rare association of Cushing's disease.


Journal

Endocrinology, diabetes & metabolism case reports
ISSN: 2052-0573
Titre abrégé: Endocrinol Diabetes Metab Case Rep
Pays: England
ID NLM: 101618943

Informations de publication

Date de publication:
19 Oct 2020
Historique:
received: 26 08 2020
accepted: 29 09 2020
pubmed: 13 1 2021
medline: 13 1 2021
entrez: 12 1 2021
Statut: aheadofprint

Résumé

Excess cortisol is associated with hypertrophy and redistribution of adipose tissue leading to central obesity which is classically seen in Cushing's syndrome. Abnormal accumulation of fatty tissue in the spinal canal is most commonly associated with chronic steroid therapy and rarely reported with endogenous Cushing's syndrome. Herein, we describe a case of spinal epidural lipomatosis (SEL) associated with Cushing's disease. A 17-year-old man was referred with lower limb weakness, weight gain, multiple stretch marks, back pain and loss of height. He had clinical and biochemical features of Cushing's syndrome. MRI and Inferior Petrosal Sinus Sampling (IPSS) confirmed a pituitary adenoma as the source. On day 1 post trans-sphenoidal adenectomy he developed spastic paraparesis with a sensory deficit to the level of T5. MRI spine showed increased fat deposition in the spinal canal from T2 to T9 consistent with a diagnosis of SEL. He was managed conservatively and made a good recovery following restoration of eucortisolism and a period of rehabilitation. SEL is a serious complication of glucocorticoid excess and should be considered in any patient presenting with new lower limb neurological symptoms associated with hypercortisolism. It is important to distinguish symptomatic SEL from cortisol-induced proximal myopathy by good history and clinical examination. MRI of the spine is the gold standard investigation for making a diagnosis of SEL. Restoration of eucortisolism can lead to resolution of fat accumulation and good neurological outcome.

Identifiants

pubmed: 33434165
doi: 10.1530/EDM-20-0111
pii: EDM200111
pmc: PMC7576635
doi:
pii:

Types de publication

Journal Article

Langues

eng

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Auteurs

Sajjad Ahmad (S)

GIM/Diabetes & Endocrinology, University Hospital of Wales, Cardiff, UK.

Thomas Best (T)

Glan Clwyd Hospital, Bodelwyddan, UK.

Andrew Lansdown (A)

GIM/Diabetes & Endocrinology, University Hospital of Wales, Cardiff, UK.

Caroline Hayhurst (C)

Neurosurgery, University Hospital of Wales, Cardiff, UK.

Fiona Smeeton (F)

GIM/Diabetes & Endocrinology, Neville Hall Hospital, Abergavenny, UK.

Steve Davies (S)

GIM/Diabetes & Endocrinology, University Hospital of Wales, Cardiff, UK.

Aled Rees (A)

GIM/Diabetes & Endocrinology, University Hospital of Wales, Cardiff, UK.

Classifications MeSH