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European Spine Journal

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01-05-2016 | Case Report

Cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis: a case report and literature review

Authors: Rui Gu, Peng-yuan Hao, Jia-bei Liu, Zhe-hui Wang, Qing-san Zhu

Published in: European Spine Journal | Special Issue 1/2016

Abstract

Purpose

To report a case of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP).

Methods

A 43-year-old male patient presented with ‘neck pain for 15 days, exacerbated accompanying motor and sensory dysfunction of lower limbs with bowel and bladder dysfunction for 4 days’ was admitted to our department. Combined with the history of ‘acupuncture treatment’, MRI results and rapid-developing progression, we considered the great possibilities of spinal cord compression by intradural hematoma and timely performed the emergency operation of cervical double-door laminoplasty and thoracic decompression with internal fixation.

Results

After combined therapy of dexamethasone, mannitol and neurotrophic drugs, sensory recovery of lower limbs started at the fifth day after operation and the sensory function became normal at the fourteenth day after operation with still complete loss of muscle strength. Pathological examination strongly suggested the diagnosis of IgG4-related sclerosing pachymeningitis (IgG4-RSP).

Conclusions

IgG4-related sclerosing pachymeningitis (IgG4-RSP) is a newly recognized disease. This case of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP) has never been reported in China with merely three case reports worldwide. Prompt surgical decompression is recommended and pathological examination is essential for diagnosis and comprehensive treatment.

Mukerji N, Todd N (2013) Spinal epidural haematoma; factors influencing outcome [J]. Br J Neurosurg 27(6):712–717CrossRefPubMed

Yoshida K, Toki F, Takeuchi T et al (1995) Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis [J]. Dig Dis Sci 40:1561–1568CrossRefPubMed

Hamano H, Kawa S, Horiuchi A et al (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis [J]. N Engl J Med 344:732–738CrossRefPubMed

Kamisawa T, Funata N, Hayashi Y et al (2003) A new clinicopathological entity of IgG4-related autoimmune disease [J]. J Gastroenterol 38:982–984CrossRefPubMed

Takahashi H, Yamamoto M, Suzuki C et al (2010) The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity [J]. Autoimmun Rev 9:591–594CrossRefPubMed

Frulloni L, Lunardi C, Simone R et al (2009) Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med 361:2135–2142CrossRefPubMed

Raina A, Yadav D, Krasinskas AM et al (2009) Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol 104:2295–2306CrossRefPubMed

Umehara H, Okazaki K, Masaki Y et al (2012) A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details [J]. Mod Rheumatol 22(1):1–14CrossRefPubMedPubMedCentral

Khosroshahi A, Stone JH (2011) A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 23:57–66CrossRefPubMed

10.

Zhang L, Smyrk TC (2010) Autoimmune pancreatitis and IgG4-related systemic diseases [J]. Int J Clin Exp Pathol 3(5):491–504PubMedPubMedCentral

11.

Masaki Y, Dong L, Kurose N et al (2009) Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders [J]. Ann Rheum Dis 68(8):1310–1315CrossRefPubMed

12.

Okazaki K, Uchida K, Koyabu M et al (2011) Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4 related disease. J Gastroenterol 4:277–288CrossRef

13.

Sah RP, Chari ST (2011) Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol 23:108–113CrossRefPubMed

14.

Chan SK, Cheuk W, Chan KT et al (2009) IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol 33(8):1249–1252CrossRefPubMed

15.

Choi SH, Lee SH, Khang SK et al (2010) IgG4-related sclerosing pachymeningitis causing spinal cord compression. Neurology 75(15):1388–1390CrossRefPubMed

16.

Sakai T, Kondo M, Yoshii S et al (2014) IgG4-related disease that presented cranial, cervical, lumbar and sacral hypertrophic pachymeningitis associated with infundibulo-hypophysitis. Rinsho Shinkeigaku 54(8):664–667CrossRefPubMed

17.

Furlan JC, Noonan V, Cadotte DW et al (2011) Timing of decompressive surgery of spinal cord after traumatic spinal cord injury: an evidence-based examination of pre-clinical and clinical studies [J]. J Neurotrauma 28(8):1371–1399CrossRefPubMedPubMedCentral

Title: Cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis: a case report and literature review
Authors: Rui Gu
Peng-yuan Hao
Jia-bei Liu
Zhe-hui Wang
Qing-san Zhu
Publication date: 01-05-2016
Publisher: Springer Berlin Heidelberg
Published in: European Spine Journal / Issue Special Issue 1/2016
Print ISSN: 0940-6719
Electronic ISSN: 1432-0932
DOI: https://doi.org/10.1007/s00586-015-4251-0

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis: a case report and literature review

Abstract

Purpose

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

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