말초신경병증을 동반한 다발성 대칭형 지방종증-증례보고-
Multiple Symmetric Lipomatosis with Peripheral Neuropathy-A case report-
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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.
참고문헌 (10)
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