Dataset Information

Sudomotor dysfunction in autoimmune autonomic ganglionopathy.

ABSTRACT:

Background

Autoimmune autonomic ganglionopathy is characterized by impairment of multiple autonomic domains of which sudomotor function is among the most common. Many patients with this disorder have difficulties with thermoregulation and anhidrosis. Our objective was to characterize the distribution and severity of sudomotor dysfunction in this disorder.

Methods

Sudomotor function was analyzed in a cohort of 21 patients with ganglionic alpha3 nicotinic acetylcholine receptor (nAChR) antibody positive autoimmune autonomic ganglionopathy. Standard measurements of sudomotor function were used including the Thermoregulatory Sweat Test and Quantitative Sudomotor Axon Reflex Test.

Results

The clinical presentation in all patients was characterized by widespread sudomotor dysfunction. Sudomotor impairment was predominantly postganglionic in 17 of the 21 patients studied. Higher ganglionic alpha3 nAChR antibody levels resulted in progressive postganglionic predominant dysfunction (postganglionic, r = 0.637, p = 0.002; mixed ganglionic, r = 0.709, p < 0.001). The pattern of anhidrosis on Thermoregulatory Sweat Testing was consistent with a ganglionopathy in the majority of patients (14 of 21) and a distal pattern in a minority of patients (8 of 21). These patterns of anhidrosis coupled with increasing postganglionic dysfunction in a proximal to distal pattern (foot > distal leg > proximal leg > forearm) indicate lesions at both the ganglia and distal axon of the postganglionic sudomotor sympathetic neuron.

Conclusions

Our data characterize the unique sudomotor dysfunction in autoimmune autonomic ganglionopathy as widespread, predominantly postganglionic, and a result of lesions at both the ganglia and distal axon. This study provides important support to the hypothesis that this disorder represents a ganglionic neuropathy.

SUBMITTER: Kimpinski K

PROVIDER: S-EPMC2779006 | biostudies-literature | 2009 Nov

REPOSITORIES: biostudies-literature

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Publications

Sudomotor dysfunction in autoimmune autonomic ganglionopathy.

Kimpinski K K Iodice V V Sandroni P P Fealey R D RD Vernino S S Low P A PA

Neurology 20091101 18

<h4>Background</h4>Autoimmune autonomic ganglionopathy is characterized by impairment of multiple autonomic domains of which sudomotor function is among the most common. Many patients with this disorder have difficulties with thermoregulation and anhidrosis. Our objective was to characterize the distribution and severity of sudomotor dysfunction in this disorder.<h4>Methods</h4>Sudomotor function was analyzed in a cohort of 21 patients with ganglionic alpha3 nicotinic acetylcholine receptor (nAC ...[more]

PMID: 19884578

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Project description:BackgroundAutoimmune autonomic ganglionopathy (AAG) is characterized by serum autoantibodies against the ganglionic acetylcholine receptor (gAChR). Immunomodulatory treatments may alleviate AAG symptoms, but the most appropriate treatment strategy is unclear.ObjectiveThis study aimed to confirm the effectiveness of treatments, particularly immunotherapy, in patients with seropositive AAG in Japan, as well as to determine the most effective treatment and the best assessment method for clinical response to treatment.MethodsWe collected data from a previous cohort study of patients with seropositive AAG. The clinical autonomic and extra-autonomic symptoms were objectively counted and subjectively assessed using the modified Composite Autonomic Symptom Score. Post-treatment changes in the gAChR antibody level were evaluated.ResultsThirty-one patients received immunotherapy. Among them, 19 patients received intravenous methylprednisolone; 27, intravenous immunoglobulin; 3, plasma exchange; 18, oral steroids; 2, tacrolimus; 1, cyclosporine; and 1, mycophenolate mofetil. Patients who received immunotherapy showed improvements in the total number of symptoms (from 6.2 ± 2.0 to 5.1 ± 2.0) and modified Composite Autonomic Symptom Score (from 37.4 ± 15.3 to 26.6 ± 12.8). Orthostatic intolerance, sicca, and gastrointestinal symptoms were ameliorated by immunotherapy. Immunotherapy decreased the antibody levels (gAChRα3 antibodies, from 2.2 ± 0.4 to 1.9 ± 0.4, p = 0.08; gAChRβ4 antibodies, from 1.6 ± 0.1 to 1.0 ± 0.2, p = 0.002), but antibody levels increased in 10 patients despite immunotherapy. The rate of improvement in the total number of symptoms was higher in patients with combined therapy than in patients with non-combined therapy (70.7% vs 28.6%).ConclusionsThe scores in many items on the rating scale decreased after immunotherapy in patients with seropositive AAG, particularly in the combined immunotherapy group. However, more accurate assessment scales for clinical symptoms and multicenter randomized, placebo-controlled prospective studies are warranted to establish future treatment strategies.

Project description:BackgroundsCardiac autonomic neuropathy is a common but always overlooked. More convenient diagnostic methods are needed.HypothesisCardiac autonomic neuropathy risk score evaluated by SUDOSCAN has a fine diagnostic efficacy detecting cardiac autonomic neuropathy.MethodsThis is a cross-sectional study among patients with diabetes mellitus. Subjects undertook SUDOSCAN tests and cardiac autonomic reflex tests, including heart rate variability due to Valsalva maneuver, heart rate response due to deep breathing and heart rate response due to standing up. Presenting 2 abnormal results was defined as cardiac autonomic neuropathy.ResultsSubjects with cardiac autonomic neuropathy has significantly higher cardiac autonomic neuropathy risk score (32.88±1.60 vs 27.64±1.24,P = 0.010). Cardiac autonomic neuropathy risk score was correlated significantly with the heart rate response due to deep breathing(P = 0.004). Multiple regression analysis including significant variables showed an independent association of cardiac autonomic neuropathy risk score and heart rate response due to deep breathing (P = 0.031) and age (P = 0.000). In receiver operating characteristic curve analysis evaluating the relationship between cardiac autonomic neuropathy risk score and cardiac autonomic neuropathy, The cut-off value was 20.5, with the sensitivity of 90.48%, the specificity of 29.5%, and the positive predictive value of 46.9%. In two-step diagnostic methods, Setting 20.5 as the cut-off value of cardiac autonomic neuropathy risk score and abnormal heart rate response due to standing up as the second diagnostic step's positive result, and setting 16.5 as the cut-off value of cardiac autonomic neuropathy risk score and abnormal heart rate response due to deep breathing as the second diagnostic step's positive result, both achieved good diagnostic efficacy.ConclusionCardiac autonomic neuropathy risk score evaluated by SUDOSCAN is a good screening test for cardiac autonomic neuropathy. The two-step diagnostic methods could be considered as surrogate diagnostic methods.

Dataset Information

Sudomotor dysfunction in autoimmune autonomic ganglionopathy.

Background

Methods

Results

Conclusions

Publications

Sudomotor dysfunction in autoimmune autonomic ganglionopathy.

Similar Datasets

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