Ann Rehabil Med.  2014 Dec;38(6):856-860. 10.5535/arm.2014.38.6.856.

Paraplegia in a Patient With IgG4-Related Sclerosing Disease: A Case Report

• Kim SH
• Kang Y
• Oh SH
• Paik S
• Kim JS

• KMID: 2267099
• DOI: http://doi.org/10.5535/arm.2014.38.6.856

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.

Keyword

Immunoglobulin G; Spinal cord compression; Paraplegia

MeSH Terms

Central Nervous System
Female
Humans
Immunoglobulin G
Immunoglobulins
Lower Extremity
Magnetic Resonance Imaging
Meningitis
Neurologic Manifestations
Pancreas
Paraplegia*
Rehabilitation
Spinal Cord
Spinal Cord Compression
Immunoglobulin G
Immunoglobulins

Figure

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  Fig. 1 Cervicothoracic magnetic resonance image shows tubular smooth-margined intradural extramedullary soft tissue mass from C7 to T5 levels with signal change on spinal cord. Sagittal T2-weighted (A) and gadoliniumenhanced T1-weighted (B) images. Axial T2-weighted (C) and gadolinium-enhanced T1-weighted (D) images.

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  Fig. 2 Findings of intraoperative specimen. (A) Anterior located intradural extramedullary mass compressing the thoracic spinal cord. (B) Separation of the mass after sacrifice of the thoracic rootlets. (C) Whole mass after excision.

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  Fig. 3 Findings of microscopic examination. Dense lymphoid cell infiltration associated with fibrosis characterized by sclerotic collagen fibers. (A) The lymphoid cell infiltration consists of a mixture of mature plasma cells and small lymphocytes (H&E stain, ×100). (B) Immunostaining reveals IgG4 positive cells (×400).

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