Immunotherapy Provides Electrophysiological Recovery and Excellent Clinical Response in Sjogren's Syndrome-Linked Quite Severe Autonomic Neuropathy.

Abstract

INTRODUCTION:

The autonomic system is frequently affected in Sjogren's syndrome (SS), but presentation with severe autonomic neuropathy is infrequent. Herein, we present a patient with primary SS-linked autonomic neuropathy, which is significantly clinic and electrophysiological responsive to immunotherapy.

CASE REPORT:

A 29-year-old female patient was admitted to our neurology department with recurrent syncope, postural light-headedness, and weight loss. Neurological examination revealed tonic pupils. The baseline composite autonomic symptom score-31 was 51 (0 to 75), and baseline functional ability score was 10 (0 to 100%). In the follow-up, syncope episodes that frequently develop during the day required the patient to lie in the supine position in bed all day and were triggered even by coming to a slightly sitting position. Neurophysiologic testing showed evidence of cardiovagal and sudomotor impairment. The patient was diagnosed with SS after detailed investigations. A 5-day course of intravenous immunoglobulin (IVIg) was given, and she continued IVIg once a month. After 6 months, she could walk long distances without support, and gastrointestinal complaints and syncopes had significantly decreased. After ~1.5 years, she had a composite autonomic symptom score-31 score of 11 and a functional ability score of 80%. Control heart rate variability analysis showed a significant improvement in the values of SD of the RR interval and root mean square of successive RR interval differences.

CONCLUSIONS:

In SS-linked severe autonomic neuropathy, immunotherapy can provide electrophysiological recovery in addition to excellent clinical response.

• Authors: Okan Sokmen, Cagri Mesut Temucin, Cansu Ayhan Seker, Ersin Tan
• Journal: The neurologist (Neurologist) Vol. 28 Issue 3 Pg. 204-206 (May 01 2023) ISSN: 2331-2637 [Electronic] United States
• PMID: 36223306 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.

Chemical References

• Immunoglobulins, Intravenous

Topics

• Female
• Humans
• Adult
• Sjogren's Syndrome (complications, therapy, diagnosis)
• Immunoglobulins, Intravenous (therapeutic use)
• Nervous System Diseases
• Immunotherapy