The spectrum of clinicopathological features in pure autonomic neuropathy

Haruki Koike, Rina Hashimoto, Minoru Tomita, Yuichi Kawagashira, Masahiro Iijima, Shigeru Koyano, Takayuki Momoo, Hiroyuki Yuasa, Shigehisa Mitake, Mana Higashihara, Kenichi Kaida, Daisuke Yamamoto, Shin Hisahara, Shun Shimohama, Yoshiharu Nakae, Ken Johkura, Steven Vernino, Gen Sobue

Research output: Contribution to journalArticlepeer-review

28 Scopus citations


We assessed the clinicopathological features of nine patients with pure autonomic neuropathy, that is, neuropathy without sensory or motor deficits. The duration from symptom onset to diagnosis ranged from 1 month to 13 years. Of eight patients in whom serum antiganglionic acetylcholine receptor antibody was determined, four were positive. All patients who tested positive for this antibody manifested widespread autonomic dysfunction, with the exception of one patient who only experienced orthostatic hypotension. However, patients who were negative for the antiganglionic acetylcholine receptor antibody presented with partial autonomic failure. One of these patients had diffuse parasympathetic failure and generalized hypohidrosis but no orthostatic hypotension, which is clinically compatible with postganglionic cholinergic dysautonomia. Electron microscopic examination revealed a variable degree of reduction in unmyelinated fibers. Compared with normal controls, the patients had a significantly increased density of collagen pockets (p<0.05). Additionally, the percentage of Schwann cell subunits with axons (out of the total number of Schwann cell subunits associated with unmyelinated fibers) was significantly decreased (p<0.01). The density of unmyelinated fibers tended to decrease with increasing time between the onset of autonomic symptoms and biopsy (p<0.05). In conclusion, the clinical and pathological features of pure autonomic neuropathy vary in terms of progression, autonomic involvement, presence of the antiganglionic acetylcholine receptor antibody, and loss of unmyelinated fibers.

Original languageEnglish (US)
Pages (from-to)2067-2075
Number of pages9
JournalJournal of neurology
Issue number10
StatePublished - Oct 2012


  • Antiganglionic acetylcholine receptor antibody
  • Autoimmune autonomic ganglionopathy
  • Autonomic neuropathy
  • Pure autonomic failure
  • Unmyelinated fiber loss

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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