Paraplegia in a Patient With IgG4-Related Sclerosing Disease: A Case Report
1
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
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.
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.
참고문헌 (10)
- Kamisawa T, "IgG4-related sclerosing disease", World J Gastroenterol, 14, pp.3948~3955 (2008)
- Wallace ZS, "Stemmer-Rachamimov A, et al. IgG4-related disease and hypertrophic pachymeningitis", Medicine (Baltimore), 92, pp.206~216 (2013)
- Lindstrom KM, "IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria", Acta Neuropathol, 120, pp.765~776 (2010)
- Hamano H, "High serum IgG4 concentrations in patients with sclerosing pancreatitis", N Engl J Med, 344, pp.732~738 (2001)
- Saab ST, "IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?", Mod Pathol, 24, pp.606~612 (2011)
- Ishihara M, "Immunohistochemical study of multiple inflammatory pseudotumors with both brain and spinal cord involvement: case report", Neurol Med Chir (Tokyo), 50, pp.246~250 (2010)
- Chan SK, "IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease", Am J Surg Pathol, 33, pp.1249~1252 (2009)
- Choi SH, "IgG4-related sclerosing pachymeningitis causing spinal cord compression", Neurology, 75, pp.1388~1390 (2010)
- Tajima Y, "Cranial neuropathy because of IgG4-related pachymeningitis; intracranial and spinal mass lesions", BMJ Case Rep (2012)[DOI]
- Della-Torre E, "Cerebrospinal fluid analysis in immunoglobulin G4-related hypertrophic pachymeningitis", J Rheumatol, 40, pp.1927~1929 (2013)