title: The spectrum of clinicopathological features in pure autonomic neuropathy.
abstract: 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.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, 466-8550, Japan. koike-haruki@med.nagoya-u.ac.jp
authors.forename: Haruki
authors.initials: H
authors.lastname: Koike
authors.forename: Rina
authors.initials: R
authors.lastname: Hashimoto
authors.forename: Minoru
authors.initials: M
authors.lastname: Tomita
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Kawagashira
authors.forename: Masahiro
authors.initials: M
authors.lastname: Iijima
authors.forename: Shigeru
authors.initials: S
authors.lastname: Koyano
authors.forename: Takayuki
authors.initials: T
authors.lastname: Momoo
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Yuasa
authors.forename: Shigehisa
authors.initials: S
authors.lastname: Mitake
authors.forename: Mana
authors.initials: M
authors.lastname: Higashihara
authors.forename: Kenichi
authors.initials: K
authors.lastname: Kaida
authors.forename: Daisuke
authors.initials: D
authors.lastname: Yamamoto
authors.forename: Shin
authors.initials: S
authors.lastname: Hisahara
authors.forename: Shun
authors.initials: S
authors.lastname: Shimohama
authors.forename: Yoshiharu
authors.initials: Y
authors.lastname: Nakae
authors.forename: Ken
authors.initials: K
authors.lastname: Johkura
authors.forename: Steven
authors.initials: S
authors.lastname: Vernino
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D001342:Autonomic Nervous System Diseases / Q000150:complications / Q000276:immunology* / Q000473:pathology*; D001369:Axons / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D046529:Microscopy, Electron, Transmission; D008875:Middle Aged; D009410:Nerve Degeneration / Q000209:etiology / Q000473:pathology; D011950:Receptors, Cholinergic / Q000276:immunology; D013497:Sural Nerve / Q000648:ultrastructure
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D011950:Receptors, Cholinergic