Lipid abnormalities in hereditary neuropathy. Part 3. Plasma and erythrocyte lipids of HMSN-III (Dejerine-Sottas disease)

Jeffrey K. Yao, Peter J Dyck

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Abstract

Plasma glucosylceramide levels of 5 patients with Hereditary Motor and Sensory Neuropathy Type III (Dejerine-Sottas disease) were approximately 50% higher than in controls. Erythrocyte glucosylceramides, however, were within the range of normal values. Elevated plasma glucosylceramide levels provide further evidence that an abnormality of lipid metabolism may underlie this recessively inherited hypertrophic neuropathy. No abnormality was observed among other glycosphingolipid fractions. An altered sphingomyelin fatty acid composition and reduced phosphatidylcholine level was also found, but this may not be disease-specific.

Original languageEnglish (US)
Pages (from-to)59-65
Number of pages7
JournalJournal of the Neurological Sciences
Volume38
Issue number1
DOIs
StatePublished - 1978

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Hereditary Sensory and Motor Neuropathy
Glucosylceramides
Erythrocytes
Lipids
Glycosphingolipids
Sphingomyelins
Phosphatidylcholines
Lipid Metabolism
Reference Values
Fatty Acids
Lipid III

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

Cite this

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AB - Plasma glucosylceramide levels of 5 patients with Hereditary Motor and Sensory Neuropathy Type III (Dejerine-Sottas disease) were approximately 50% higher than in controls. Erythrocyte glucosylceramides, however, were within the range of normal values. Elevated plasma glucosylceramide levels provide further evidence that an abnormality of lipid metabolism may underlie this recessively inherited hypertrophic neuropathy. No abnormality was observed among other glycosphingolipid fractions. An altered sphingomyelin fatty acid composition and reduced phosphatidylcholine level was also found, but this may not be disease-specific.

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