Paraplegia in a Patient With IgG4-Related Sclerosing Disease: A Case Report.
10.5535/arm.2014.38.6.856
- Author:
Sung Heon KIM
1
;
Yeon KANG
;
Sung Han OH
;
Soya PAIK
;
Joo Sup KIM
Author Information
1. Department of Rehabilitation Medicine, Bundang Jesaeng Hospital, Seongnam, Korea. glide@dmc.or.kr
- Publication Type:Case Report
- Keywords:
Immunoglobulin G;
Spinal cord compression;
Paraplegia
- MeSH:
Central Nervous System;
Female;
Humans;
Immunoglobulin G;
Immunoglobulins;
Lower Extremity;
Magnetic Resonance Imaging;
Meningitis;
Neurologic Manifestations;
Pancreas;
Paraplegia*;
Rehabilitation;
Spinal Cord;
Spinal Cord Compression
- From:Annals of Rehabilitation Medicine
2014;38(6):856-860
- CountryRepublic of Korea
- Language:English
-
Abstract:
Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease, characterized by mass forming inflammatory lesions which respond well to steroid therapy. Pancreas is the most common site of involvement, and other organ involvements are also common. However, there are only a few reports about central nervous system involvement. We report a case of IgG4-related sclerosing disease which involves spinal cord causing paraplegia. A middle-aged female presented with sudden lower limb weakness. Magnetic resonance imaging showed a soft tissue mass which was diffusely compressing spinal cord along the C7 to T5 levels. Intravenous steroid pulse therapy and emergent operation was performed. The immunopathologic findings revealed IgG4-related sclerosing pachymeningitis postoperatively. There was no evidence of other organ involvement. Her neurologic deficit remained unchanged after two months of comprehensive rehabilitation therapy.