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.

The spectrum of immune-mediated autonomic neuropathies: insights from the clinicopathological features: Table 1

“…Antecedent infections have been reported irrespective of the presence or absence of the antiganglionic AChR antibody in AAG patients 13 15. Upper respiratory tract infections and gastrointestinal tract infections, which are likely attributable to viral infections, are the most common antecedent infections 21.…”

“…Autonomic symptoms may develop immediately after infectious diseases 32. Epididymitis,15 aseptic meningitis30 and encephalitis15 have also been reported as antecedent events. AAG may also be associated with vaccination,17 surgical procedures17 or interferon therapy 36…”

“…This monophasic clinical course and frequent presence of a history of antecedent infections led to the speculation that immune mechanisms similar to those involved in Guillain-Barré syndrome (GBS) contribute to primary autonomic neuropathies 14 15. The discovery of the antiganglionic acetylcholine receptor (AChR) antibody confirmed the involvement of immune mechanisms, especially in patients with pure autonomic neuropathy 16.…”

Differential Recovery in Cardiac and Vasomotor Sympathetic Functional Markers in a Patient with Acute Autonomic Sensory and Motor Neuropathy

“…In this patient, dysautonomia was observed in both the peripheral sympathetic and parasympathetic nervous systems. From the viewpoint of autonomic neuropathy with a monophasic clinical course similar to GBS, studies have described at least three major groups based on concomitant sensory motor involvement (13,14): (1) pure autonomic neuropathy (15)(16)(17), (2) acute autonomic and sensory neuropathy (13,18,19) and (3) AASMN (3-6). The nosological entity of AASMN should be considered carefully because autonomic dysfunction has been frequently reported in patients with GBS (14, 20-22).…”

Miller-fisher syndrome on the borders of guillain-barre syndrome: about one case and review of literature

“…Our case included, in addition to the classic MFS triad, facial, bulbar and dysautonomic impairment. According to some authors, neuropathies, acute sensory small-fiber, acute sensory and autonomic and acute pandysautonomy probably represent postinfectious autoimmune diseases similar to those of GBS [11][12][13].…”