Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 16, 2024; 12(32): 6551-6558
Published online Nov 16, 2024. doi: 10.12998/wjcc.v12.i32.6551
Immunoglobulin G4-related spinal pachymeningitis: A case report
Tae-Seok Chae, Da-Sol Kim, Gi-Wook Kim, Yu Hui Won, Myoung-Hwan Ko, Sung-Hee Park, Jeong-Hwan Seo
Tae-Seok Chae, Da-Sol Kim, Gi-Wook Kim, Yu Hui Won, Myoung-Hwan Ko, Sung-Hee Park, Jeong-Hwan Seo, Department of Physical Medicine & Rehabilitation, Jeonbuk National University Medical School, Jeonju 54907, South Korea
Da-Sol Kim, Gi-Wook Kim, Yu Hui Won, Myoung-Hwan Ko, Sung-Hee Park, Jeong-Hwan Seo, Department of Physical Medicine & Rehabilitation, Research Institute of Clinical Medicine of Jeonbuk National University - Biomedical Research Institute of Jeonbuk National University Hospital, Jeonju 54907, South Korea
Author contributions: Chae TS prepared the figures and collected the data, and contributed to the manuscript drafting; Kim GW, Won YH, Park SH, Ko MH, and Seo JH contributed to the data analysis; Kim DS was responsible for the manuscript revision and intellectual content; all authors issued final approval for the version to be submitted.
Informed consent statement: The patient provided informed consent prior to study inclusion.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Da-Sol Kim, MD, PhD, Professor, Department of Physical Medicine & Rehabilitation, Jeonbuk National University Medical School, 20, Geonji-ro, Deokjin-gu, Jeonju-si, Jeollabuk-do, Jeonju 54907, South Korea. murunoon@naver.com
Received: February 5, 2024
Revised: June 24, 2024
Accepted: August 28, 2024
Published online: November 16, 2024
Processing time: 231 Days and 4.2 Hours
Abstract
BACKGROUND

Immunoglobulin G4-related disease (IgG4-RD) is a complex immune-mediated condition that causes fibrotic inflammation in several organs. A significant clinical feature of IgG4-RD is hypertrophic pachymeningitis, which manifests as inflammation of the dura mater in intracranial or spinal regions. Although IgG4-RD can affect multiple areas, the spine is a relatively rare site compared to the more frequent involvement of intracranial structures.

CASE SUMMARY

A 70-year-old male presented to our hospital with a two-day history of fever, altered mental status, and generalized weakness. The initial brain magnetic resonance imaging (MRI) revealed multiple small infarcts across various cerebral regions. On the second day after admission, a physical examination revealed motor weakness in both lower extremities and diminished sensation in the right lower extremity. Electromyographic evaluation revealed findings consistent with acute motor sensory neuropathy. Despite initial management with intravenous immunoglobulin for presumed Guillain-Barré syndrome, the patient exhibited progressive worsening of motor deficits. On the 45th day of hospitalization, an enhanced MRI of the entire spine, focusing specifically on the thoracic 9 to lumbar 1 vertebral level, raised the suspicion of IgG4-related spinal pachymeningitis. Subsequently, the patient was administered oral prednisolone and participated in a comprehensive rehabilitation program that included gait training and lower extremity strengthening exercises.

CONCLUSION

IgG4-related spinal pachymeningitis, diagnosed on MRI, was treated with corticosteroids and a structured rehabilitation regimen, leading to significant improvement.

Keywords: Immunoglobulin G4 related disease; Immunoglobulin G4 related spinal pachymeningitis; Manual motor test; Electromyography; Magnetic resonance imaging; Case report

Core Tip: This case of immunoglobulin G4-related spinal pachymeningitis with neurological symptoms provides valuable insights into diagnosis and treatment strategies in rehabilitative medicine. Detailed examination of this rare condition highlights the importance of considering immunoglobulin G4-related disease in patients with unexplained spinal and neurological symptoms. It emphasizes the need for comprehensive diagnostic approaches, including advanced imaging techniques and electromyography, to ensure accurate diagnosis. Additionally, the case underscores the potential for effective treatment through steroid therapy and rehabilitation, significantly improving patient outcomes. This information helps clinicians recognize and manage similar cases, enhancing patient care in rehabilitative settings.