Multiple Symmetric Lipomatosis with Peripheral Neuropathy: A case report.
- Author:
Joo Young LEE
1
;
Young Ok PARK
;
Hee Seung YANG
;
Eun Kyoung KANG
;
Min Jeong LIM
Author Information
1. Department of Rehabilitation Medicine, Seoul Verterans Hospital, Korea. annelee77@daum.net
- Publication Type:Original Article
- Keywords:
Multiple symmetric lipomatosis;
Peripheral polyneuropathy
- MeSH:
Abdomen;
Alcohol Drinking;
Biopsy;
DNA, Mitochondrial;
Humans;
Lipoma;
Lipomatosis, Multiple Symmetrical*;
MERRF Syndrome;
Myelin Sheath;
Neck;
Peripheral Nervous System Diseases*;
Point Mutation;
Polyneuropathies;
Prevalence;
Radiculopathy;
Shoulder;
Subcutaneous Tissue;
Sural Nerve
- From:Journal of the Korean Academy of Rehabilitation Medicine
2004;28(6):622-626
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Multiple symmetric lipomatosis is a rare disorder characterized by massive fatty deposits arranged symmetrically around the neck, shoulder, abdomen and back. It is typically associated with high alcohol consumption and a high prevalence of peripheral neuropathy. The pathogenesis of the syndrome is still unknown, but mitochondrial abnormality or metabolic abnormalities are occasionally found in the affected patients. In our patient, clinical and electrophysiologic signs of a generalized peripheral sensorimotor neuropathy and a multiple bilateral lumbosacral radiculopathy were observed. Sural nerve biopsy demonstrated many small unmyelinated fibers with complete loss of axoplasm and a extensive loss of myelinated fibers. Lipoma biopsy demonstrated non-capsulated mature adipose cells in the subcutaneous tissue. Serum lipid studies were normal. MERRF point mutation of mitochondrial DNA were negative in blood. We reported a case of multiple symmetric lipomatosis and peripheral polyneuropathy with the review of literature.
