Staal A, Stefanko S Z, Busch H F, Jennekens F G, De Bruinj W C
J Neurol Sci. 1981 Sep;51(3):383-94. doi: 10.1016/0022-510x(81)90116-7.
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.
