Authors: Risa Isonaka Courtney Holmes Glen A Cook Patti Sullivan Yehonatan Sharabi David S Goldstein
Publish Date: 2017/02/10
Volume: 27, Issue: 2, Pages: 97-101
Abstract
Pure autonomic failure is a rare form of chronic autonomic failure manifesting with neurogenic orthostatic hypotension and evidence of sympathetic noradrenergic denervation unaccompanied by signs of central neurodegeneration It has been proposed that pure autonomic failure is a Lewy body disease characterized by intraneuronal deposition of the protein alphasynuclein in Lewy bodies and neurites A middleaged man with previously diagnosed pure autonomic failure experienced a sudden fatal cardiac arrest He was autopsied and tissues were harvested for neurochemical and immunofluorescence studies Postmortem microscopic neuropathology showed no Lewy bodies Lewy neurites or alphasynuclein deposition by immunohistochemistry anywhere in the brain The patient had markedly decreased immunofluorescent tyrosine hydroxylase in sympathetic ganglion tissue without detectable alphasynuclein even in rare residual nests of tyrosine hydroxylasecontaining ganglionic fibers In pure autonomic failure sympathetic noradrenergic denervation can occur without concurrent Lewy bodies or alphasynuclein deposition in the brain or sympathetic ganglion tissue
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