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  • Title: Autonomic nerve calcification and peripheral neuropathy in olivopontocerebellar atrophy.
    Author: Staal A, Stefanko SZ, Busch HF, Jennekens FG, De Bruinj WC.
    Journal: J Neurol Sci; 1981 Sep; 51(3):383-94. PubMed ID: 7276985.
    Abstract:
    This report concerns an unusual form of olivopontocerebellar atrophy (OPCA) of adult onset, inherited as an autosomal dominant. We examined 5 patients in one generation and performed neuropathological investigations in 3 of these. The clinical and pathological features were different from those of OPCA types I to V. Apart from olivopontocerebellar degeneration, there was dementia in 4, massive atrophy of the spinal cord in 3, and focal degeneration of the optic nerves in at least 1 case. The most remarkable findings were, however, the involvement of the peripheral nervous system and the abundant intrafascicular calcification in sympathetic nerve fibers and in their ganglia.
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