Tangier disease

Tangier disease (TD) is a rare lipoprotein metabolism disorder characterized biochemically by an almost complete absence of plasma high-density lipoproteins (HDL), and clinically by liver, spleen, lymph node and tonsil enlargement along with peripheral neuropathy in children and adolescents, and, occasionally, cardiovascular disease in adults.

Polyneuropathy

A generalized disorder of peripheral nerves.


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PMID (PMCID)
2997405
 MALE Adult
Severe polyneuropathy in Tangier disease mimicking syringomyelia or leprosy. Clinical, biochemical, electrophysiological, and morphological evaluation, including electron microscopy of nerve, muscle, and skin biopsies.
Gibbels E, Schaefer HE, Runne U, Schroder JM, Haupt WF, Assmann G.
J Neurol. 1985;232(5):283-94.
Polyneuropathy in Tangier disease can be divided into three clinical types.
2992211
 MALE
Tangier disease. A case with sensorimotor distal polyneuropathy and lipid accumulation in striated muscle and vasa nervorum.
Marbini A, Gemignani F, Ferrarini G, Maccari S, Lucci B, Bragaglia MM, Plancher C, Vergani C.
Acta Neuropathol. 1985;67(1-2):121-7.
This Tangier patient differs from known cases for the presence of a distal symmetrical sensorimotor polyneuropathy (not previously reported in Tangier disease) and because of the morphological findings of de-/remyelination coexisting with aspects of axonal atrophy and previous degeneration, and of lipid accumulation within striated muscle and vasa nervorum.