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

Published on May 1, 2016in European Spine Journal2.513
· DOI :10.1007/s00586-015-4251-0
Rui Gu5
Estimated H-index: 5
(JLU: Jilin University),
Peng-yuan Hao1
Estimated H-index: 1
(JLU: Jilin University)
+ 2 AuthorsQingsan Zhu5
Estimated H-index: 5
(JLU: Jilin University)
Abstract
Purpose To report a case of cervicothoracic spinal cord compression caused by IgG4-related sclerosing pachymeningitis (IgG4-RSP).
  • References (18)
  • Citations (8)
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References18
Newest
#1坂井 利行H-Index: 1
#2近藤 昌秀H-Index: 1
Last. 冨本 秀和H-Index: 2
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症例は32歳の女性である.頭痛,視力低下で発症し,尿崩症,球後視神経炎,四肢腱反射亢進と軽度筋力低下を呈し,血清IgG4上昇,髄液細胞数軽度増多と蛋白増加をみとめ,中枢性IgG4関連疾患と診断した.頭部と脊髄造影MRIにて下垂体炎,および頭蓋,頸椎,腰椎と仙椎レベルの肥厚性硬膜炎を,胸部CTで左肺小結節をみとめた.硬膜生検ではリンパ球系細胞浸潤をみとめたがIgG4免疫染色陽性形質細胞はみられなかった.ステロイドパルス療法にて視力は回復し,血清IgG4は正常になり肥厚性硬膜炎と左肺小結節は軽快した.IgG4関連下垂体炎,および頭蓋,頸椎,腰椎と仙椎レベルの肥厚性硬膜炎を同時期に呈した最初の報告例である.
5 CitationsSource
AbstractObjective. Spinal epidural haematoma (SEH) causing spinal cord compression is potentially a cause of long-term neurological disability. We reviewed the relevant literature with the intention of establishing the factors that influence long-term outcome and the timeframe within which operative intervention must be undertaken for optimal results. Methods. A total of 1177 papers were identified using PubMed among which 31 papers were selected and analysed. The grades of neurological deficit,...
15 CitationsSource
#1Hisanori Umehara (Kanazawa Medical University)H-Index: 24
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 63
Last. Dai Inoue (Kanazawa University)H-Index: 15
view all 22 authors...
IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz’s disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm...
427 CitationsSource
#1Julio C. Furlan (U of T: University of Toronto)H-Index: 28
#2Vanessa K. Noonan (UBC: University of British Columbia)H-Index: 27
Last. Michael G. Fehlings (U of T: University of Toronto)H-Index: 96
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Abstract While the recommendations for spine surgery in specific cases of acute traumatic spinal cord injury (SCI) are well recognized, there is considerable uncertainty regarding the role of the timing of surgical decompression of the spinal cord in the management of patients with SCI. Given this, we sought to critically review the literature regarding the pre-clinical and clinical evidence on the potential impact of timing of surgical decompression of the spinal cord on outcomes after traumati...
168 CitationsSource
#1Kazuichi Okazaki (Kansai Medical University)H-Index: 63
#2Kazushige Uchida (Kansai Medical University)H-Index: 35
Last. Makoto Takaoka (Kansai Medical University)H-Index: 23
view all 5 authors...
Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more...
204 CitationsSource
Purpose of review IgG4-related systemic disease (ISD) is a recently recognized syndrome affecting multiple organs. Autoimmune pancreatitis (AIP) is the pancreatic manifestation of ISD and mimics pancreatic cancer. Current data show frequent association with serum IgG4 elevation and other serologic abnormalities. Here we explore the diagnostic and possible prognostic utility and pathogenetic implications of serologic abnormalities in ISD. Recent findings Serum IgG4 elevations (>140 mg/dl) are see...
215 CitationsSource
Purpose of reviewTo summarize the existing knowledge of various clinical presentations of IgG4-related systemic disease (IgG4-RSD) and to review the evolving list of organs affected by IgG4-RSD.Recent findingsThe term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirel
203 CitationsSource
#1Sang-Ho ChoiH-Index: 34
#2Sang Hoon LeeH-Index: 2
Last. Sang Ryong JeonH-Index: 1
view all 4 authors...
Immunoglobulin G (IgG)4-related sclerosing disease is an emerging clinicopathologic entity. It is defined as a systemic disease characterized histopathologically by extensive IgG4-positive plasma cell infiltration of various organs together with T-lymphocyte infiltration.1 Pancreas is the most frequently affected organ. This disease was included within the category of autoimmune pancreatitis before the concept of IgG4-related sclerosing disease was introduced.2 There have been several reports of...
40 CitationsSource
#1Hiroki Takahashi (Sapporo Medical University)H-Index: 45
#2Motohisa YamamotoTetsuya (Sapporo Medical University)H-Index: 33
Last. ImaiKohzoh (Sapporo Medical University)H-Index: 82
view all 6 authors...
Abstract IgG4-related disease is a distinct clinical entity, whose characteristic features are the following; Serum IgG4 is prominently elevated, IgG4-positive plasma cells infiltrate in involved tissues, various mass-forming lesions with fibrosis develop in a timely and spatial manner and the response to corticosteroids is prompt and good. IgG4-related diseases mainly target two organs. One is the pancreas (autoimmune pancreatitis; AIP), and the other comprises the lacrimal and salivary glands,...
88 CitationsSource
Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis that is characterized by lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis, and increased IgG4+ plasma cells. Serum IgG4 levels usually are elevated. Patients with AIP frequently have disease affecting other organs or sites; these tissues show similar histologic changes, including increased IgG4+ plasma cell infiltrate and response to corticosteroid therapy. A new clinicopathologic concept of IgG4-related...
61 Citations
Cited By8
Newest
#1Brian Park (UIHC: University of Iowa Hospitals and Clinics)H-Index: 4
#2Rachel D. Starks (UIHC: University of Iowa Hospitals and Clinics)H-Index: 1
Last. Brian J. Dlouhy (Roy J. and Lucille A. Carver College of Medicine)H-Index: 15
view all 5 authors...
Abstract Background The majority of the abnormalities and diseases that affect the craniovertebral junction (CVJ) have already been reported, and therefore it is exceedingly rare to identify new pathology that affects the CVJ. IgG4 related disease (IgG4-RD) is an immune mediated process treated with immunosuppressive medications. This is the first reported case of IgG4-RD affecting the CVJ. Case Description The authors report the case of a 71-year-old woman with IgG4-RD of the CVJ. She presented...
Source
#1Cléa Melenotte (AMU: Aix-Marseille University)H-Index: 8
#2Julie Seguier (AMU: Aix-Marseille University)H-Index: 2
Last. Nicolas Schleinitz (AMU: Aix-Marseille University)H-Index: 28
view all 23 authors...
Abstract Pachymeningitis is rare, either idiopathic or secondary to inflammatory disorders, after tumoral, surgical or infectious causes have been excluded. The fibroinflammatory IgG4-related disease is one of the etiologies of pachymeningitis with only few cases reported yet. From a single referral regional center, we evaluated the frequency of IgG4-related disease as the cause of inflammatory pachymeningitis in 10% of cases. From a National case registry of IgG4-related disease the pachymening...
Source
#1Stephen J. Slade (Virginia Mason Medical Center)
#2Erin M. Bauer (Virginia Mason Medical Center)
Last. Amish J. Dave (Virginia Mason Medical Center)
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Background Back pain is a leading reason for patients to seek medical attention. Although musculoskeletal causes are common, patients can also present with rarer etiologies. Case Description A 50-year-old man presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. During the next 3 months, his pain worsened, and he developed lower extremity dysesthesia and subjective weakness, despite normal neurological examination findings. Nonreveali...
Source
#1Kelly J. Bridges (OHSU: Oregon Health & Science University)H-Index: 3
#2Caitlin H. DeDeaux (OHSU: Oregon Health & Science University)H-Index: 1
Last. Khoi D. Than (OHSU: Oregon Health & Science University)H-Index: 24
view all 3 authors...
AbstractObject: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder affecting various anatomical sites, and only recently was identified to affect the dura of the spine. The authors pre...
3 CitationsSource
We report the rare case of an 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented to our neurological department with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral and postcentral cortex. A leptome...
Source
#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
Last. John H. Stone (Harvard University)H-Index: 70
view all 3 authors...
Summary IgG4-related disease can involve nearly any organ system, including the central and peripheral nervous systems. The pathology findings are consistent from organ to organ, but careful clinicopathological correlation is necessary to establish the diagnosis. Many non-neurological and neurological inflammatory conditions, previously regarded as idiopathic in nature, are now recognised to fall within the spectrum of IgG4-related disease. The condition is highly treatable, but probably remains...
11 CitationsSource
#1Mary D. Maher (CUMC: Columbia University Medical Center)H-Index: 1
#2George Zanazzi (CUMC: Columbia University Medical Center)H-Index: 19
Last. Tony T. Wong (CUMC: Columbia University Medical Center)H-Index: 4
view all 5 authors...
Abstract IgG4-related disease is an immune-mediated fibro-inflammatory condition that affects multiple organ systems. When the pachymeninges are involved, it is termed IgG4-related hypertrophic pachymeningitis (IgG4-RHP). We present a 79-year-old woman with back pain found to have IgG4-RHP localized exclusively in the spine who was also seropositive for myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA). This case is the first known report of IgG4-RHP in the spine with MPO-ANCA an...
4 CitationsSource
1 CitationsSource
#1Bernardo BaptistaH-Index: 1
#2Alina Casian (Guy's and St Thomas' NHS Foundation Trust)H-Index: 7
Last. Claire M Rice (Southmead Hospital)H-Index: 18
view all 5 authors...
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG...
13 CitationsSource