Dataset Information

Lumbar spinal epidural lipomatosis: A case report and review of the literature.

ABSTRACT:

Introduction

Lumbar spinal epidural lipomatosis (SEL) is a rare condition defined by an excessive deposition of adipose tissue in the lumbar spinal canal. The objective of this case report is to document a clinical case of SEL presenting within a multidisciplinary spine clinic and to compare our clinical findings and management with the available literature.

Case presentation

A 51-year-old female presented at a spine clinic with low back pain, bilateral leg pain and difficulty walking. Magnetic resonance imaging of the lumbar spine showed evidence of severe central canal stenosis due to extensive epidural lipomatosis. She was initially advised to lose weight and undergo a 3-month course of physiotherapy. However, because of lack of improvement, she was scheduled for and underwent L4-S1 posterior spinal decompression and L4-L5 posterior spinal instrumented fusion. At 12-month follow-up, the patient reported no pain and retained the ability to walk regular distances without experiencing discomfort.

Discussion

This case report describes the conservative and surgical management of a case of lumbar spinal stenosis due to SEL. The therapeutic approach of patients with this condition is not standardized. As such, a discussion of the literature with respect to the diagnosis, clinical presentation, epidemiology, imaging appearance, risk factors, etiology, and management of SEL is also presented.

SUBMITTER: Mallard F

PROVIDER: S-EPMC7736757 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Publications

Lumbar spinal epidural lipomatosis: A case report and review of the literature.

Mallard Fabrice F Buni Manar M Nolet Paul S PS Emary Peter P Taylor John A JA Moammer Gemah G

International journal of surgery case reports 20201130

<h4>Introduction</h4>Lumbar spinal epidural lipomatosis (SEL) is a rare condition defined by an excessive deposition of adipose tissue in the lumbar spinal canal. The objective of this case report is to document a clinical case of SEL presenting within a multidisciplinary spine clinic and to compare our clinical findings and management with the available literature.<h4>Case presentation</h4>A 51-year-old female presented at a spine clinic with low back pain, bilateral leg pain and difficulty wal ...[more]

PMID: 33310475

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Project description:BackgroundClassic cerebrotendinous xanthomatosis (CTX; OMIM #213700) manifests with chronic diarrhea, juvenile cataracts, tendon xanthomas and neurological symptoms. It is due to biallelic inactivation of CYP27A1 wich leads to cholestanol accumulation in the central nervous system, eyes and tendons. Less commonly, the disease can present in young adults as spastic paraparesis in the absence of xanthomas.Case presentationWe report a 38-year old woman who presented with chronic diarrhea and progressive spastic paraparesis in her twenties. Brain magnetic resonance imaging (MRI) showed cerebral atrophy with diffuse periventricular white matter hyperintensities. Spinal MRI was normal. CYP27A1 gene sequencing confirmed the diagnosis of CTX. Chenodeoxycholic acid (CDCA) treatment was introduced with remission of diarrhea. Unfortunately, the treatment had to be discontinued several times and the patient developed psychosis and an severe ataxospastic gait. Spinal MRI revealed new linear hyperintensities of the corticospinal and gracile tracts. Thirty-three spinal CTX patients were identified by searching in Pubmed, EMBASE™ and Web of Science databases. All patients presented pyramidal signs and 48% had dorsal column signs. Juvenile cataracts were described in 78% of patients, chronic diarrhea in 65%, and tendon xanthomas in 31%. Disease improvement or stabilization with chenodeoxycholic acid was observed in 69% of patients. A higher prevalence of the Arg395Cys allele was observed in patients with spinal CTX as compared to CTX in general (ᵡ2; p < 0.00001).ConclusionsThe diagnosis of spinal CTX can be easily missed or delayed in absence of xanthomas. There is a higher prevalence of the Arg395Cys allele in spinal CTX as compared to classic childhood-onset CTX. CDCA treatment seems to stabilize or improve clinical symptoms in most patients. However, as seen in our patient and in two previously reported cases, sudden interruption of CDCA may lead to irreversible neurological complications.

Dataset Information

Lumbar spinal epidural lipomatosis: A case report and review of the literature.

Introduction

Case presentation

Discussion

Publications

Lumbar spinal epidural lipomatosis: A case report and review of the literature.

Similar Datasets

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