Nemoto H, Fujioka T, Okada S, Wakata N, Kinoshita M
Fourth Department of Internal Medicine, Toho University.
Rinsho Shinkeigaku. 1992 May;32(5):543-6.
A 61-year-old male with carcinomatous autonomic and sensory neuropathy was presented. The disease started with numbness in his lower extremities and loss of sensation gradually developed over the upper extremities within two months. Finally he became unable to walk because of orthostatic hypotension and dystaxia due to loss of deep sensation. Physical examination on admission revealed mydriasis with negative light reflex, orthostatic hypotension, sensory loss of below C-4, areflexia, positive Babinski's sign and sexual impotence. Laboratory examinations revealed serum antineural antibody, elevation of protein and oligoclonal IgG band in the CSF. Sural nerve biopsy specimen showed severe loss of myelinated fibers, especially in the large-sized fibers. The pharmacological functional tests of the autonomic nerves disclosed disturbance of parasympathetic nervous system as well as the postsynaptic fibers of the sympathetic nerves. An instillation test of 2.5% mecholyl solution demonstrated contraction of both pupils. Chest X-ray showed abnormal shadow at the right hilus and transbronchial biopsy revealed small cell carcinoma (oat cell type). Plasma exchange improved orthostatic hypotension and decreased the titer of the antineural antibody. In this case, the autonomic ganglion including the ciliary ganglia and the dorsal root ganglia cells were predominantly involved and such cases have been described as a variant of paraneoplastic syndrome in the literature.