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Brain, Vol. 106, No. 4, 911-928, 1983
© 1983 Guarantors of Brain

research-article

PERIPHERAL NEUROPATHY IN TANGIER DISEASE

M. POLLOCK, H. NUKADA, R. W. FRITH, J. P. SIMCOCK and S. ALLPRESS

Division of Neurology, University of Otago Medical School and the Neurology Department, Auckland Hospital New Zealand

Peripheral nerve morphometry was assessed in four patients with Tangier disease. Three patients with a relapsing and remitting multiple mononeuropathy had prominent peripheral nerve demyelination and remyelination with affected internodes clustered along particular nerve fibres. Putative lipid vacuoles were almost exclusively confined in this multifocal neuropathy syndrome to Remak cells. By contrast a fourth patient with a slowly progressive syringomyelia-like neuropathy had advanced peripheral nerve degeneration and a more global distribution of lipid vacuoles within peripheral nerve. A review of Tangier disease in the literature indicated the possibility of additional peripheral nerve syndromes. The clinical heterogeneity raises the possibility of different metabolic erros in Tangier disease or a common metabolic error subject to genetic influences. The results of this study indicate that normal serum cholesterol levels do not exclude a diagnosis of Tangier disease. It is therefore advisable to determine both high density lipoproteins and serum cholesterol levels in patients with undiagnosed multifocal neuropathy or syringomyelia-like syndromes.

Received January 4, 1983. Accepted March 15, 1983.


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