A Case With IgG4-Related Spinal Pachymeningitis Causing Spinal Cord Compression.

Published on Jul 14, 2020in Frontiers in Neurology2.889
· DOI :10.3389/FNEUR.2020.00500
Rensheng Zhang1
Estimated H-index: 1
(JLU: Jilin University),
Jiguo Gao2
Estimated H-index: 2
(JLU: Jilin University)
+ 6 AuthorsShaokuan Fang4
Estimated H-index: 4
(JLU: Jilin University)
Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by sclerosing lesions and an increased serum IgG4 level. This condition can involve any organ, but IgG4-related spinal pachymeningitis is relatively rare.In the current study, we report a case of spinal cord compression caused by IgG4-related spinal pachymeningitis.A 39-year-old man presented to us with a 15-day history of back pain and a 3-day history of dysuresia, exacerbated by weakness in the lower extremities for 2 days. Cervical magnetic resonance imaging (MRI) showed strip-shaped abnormal signals along the anterior and posterior borders of the spinal cord at the C5–T4 levels. The IgG level in cerebrospinal fluid was 718.0 mg/L. Thoracic MRI revealed strip-shaped abnormal signals with remarkable enhancement along the anterior and posterior borders of the dural sac at the T1–T6 levels. Histopathological examination confirmed IgG4-related spinal pachymeningitis. The symptoms worsened rapidly, and surgical resection of the space-occupying lesion in the vertebral canal was performed for spinal decompression. Corticosteroid therapy was administrated, and the patient’s motor functions were mildly improved.IgG4-related disease can manifest as spinal pachymeningitis and cause spinal cord compression. Clinicians should be aware of this rare condition, and early diagnosis, timely surgical decompression, and appropriate corticosteroid therapy should be highlighted.
#1Linyi Peng (Peking Union Medical College Hospital)H-Index: 8
#2Panpan Zhang (Peking Union Medical College Hospital)H-Index: 7
Last. Xiaofeng Zeng (Peking Union Medical College Hospital)H-Index: 28
view all 11 authors...
OBJECTIVES: Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease that affects various organs of the body. The aim of this study is to elucidate the clinical characteristics of IgG4-RD with central nervous system (CNS) involvement. METHODS: Among 589 patients with IgG4-RD in a prospective single-centre cohort study in Peking Union Medical College Hospital, 15 patients had CNS involvement. The clinical data including demographic features, symptoms, involved organs, laboratory findings...
5 Citations
IgG4-related disease (IgG4-RD) is a disorder with various clinical manifestations. Central nervous system (CNS) involvement is well recognized, with hypertrophic pachymeningitis and hypophysitis being the most common manifestations. Spinal cord involvement is an extremely rare manifestation. We present the first case of an IgG4-RD patient with spinal cord parenchymal disease and concurrent hypophysitis. We review also the current literature about CNS parenchymal involvement in the context of IgG...
4 CitationsSource
Objective Meningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP). Methods Two IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and cas...
10 CitationsSource
Intrathecal administration of rituximab—an anti-CD20 monoclonal antibody—is emerging as a promising therapeutic strategy for B cell lymphomas of the central nervous system (CNS).1 2 The rationale for administering rituximab directly into the cerebrospinal fluid (CSF) stems from the need to achieve optimal therapeutic concentrations within the intrathecal compartment. Rituximab, in fact, has a high molecular weight and only 0.1%–0.5% of its plasmatic concentrations crosses the ‘blood-brain barrie...
20 CitationsSource
#1Y Yangue (Sapienza University of Rome)H-Index: 1
#2Giulio GambaracciH-Index: 3
Last. Michele Scialpi (University of Perugia)H-Index: 19
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AbstractFoetal magnetic resonance imaging (MRI) plays an increasingly important role in the diagnosis of foetal abnormalities. Over the years, we have successfully applied bi-parametric MRI (bp-MRI) to the following obstetric conditions: (1) neurologic vascular diseases; (2) assessment of lung parenchyma maturation; (3) renal pathologies, such as polycystic kidney, suspected renal infarction, unilateral or bilateral renal agenesis; (4) placental pathologies, as twin-twin transfusion syndrome or ...
7 CitationsSource
#1Zhang Lu (China-Japan Friendship Hospital)H-Index: 1
#2Liu Tongxi (China-Japan Friendship Hospital)H-Index: 1
Last. Lu Xin (China-Japan Friendship Hospital)H-Index: 1
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The aim of this study is to study the clinical, laboratory, imaging pathology, and prognosis features of IgG4-related spinal pachymeningitis. We worked with a 55-year-old man suffering from IgG4-related spinal pachymeningitis who had the most widespread lesion in his dura mater. We also review previous related studies and discuss the clinical characteristics of this rare disease. In total, eight IgG4-related spinal pachymeningitis patients have been reported in the literature since 2009. They we...
11 CitationsSource
#1Arezou Khosroshahi (Emory University)H-Index: 30
#2Zachary S. Wallace (Harvard University)H-Index: 25
Last. John H. Stone (Harvard University)H-Index: 76
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A. Khosroshahi, Z. S. Wallace, J. L. Crowe, T. Akamizu, A. Azumi, M. N. Carruthers, S. T. Chari, E. Della-Torre, L. Frulloni, H. Goto, P. A. Hart, T. Kamisawa, S. Kawa, M. Kawano, M. H. Kim, Y. Kodama, K. Kubota, M. M. Lerch, M. L€ ohr, Y. Masaki, S. Matsui, T. Mimori, S. Nakamura, T. Nakazawa, H. Ohara, K. Okazaki, J. H. Ryu, T. Saeki, N. Schleinitz, A. Shimatsu, T. Shimosegawa, H. Takahashi, M. Takahira, A. Tanaka, M. Topazian, H. Umehara, G. J. Webster, T. E. Witzig, M. Yamamoto, W. Zhang, T....
445 CitationsSource
#1Emanuel Della-Torre (UniSR: Vita-Salute San Raffaele University)H-Index: 25
#2Laura GalliH-Index: 1
Last. Maria Grazia Sabbadini (UniSR: Vita-Salute San Raffaele University)H-Index: 37
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Abstract Diagnosis of IgG4-Related Hypertrophic Pachymeningitis (IgG4-HP) relies on meningeal biopsies, because cerebrospinal fluid (CSF) diagnostic biomarkers are lacking. Here, we determined whether IgG4 intrathecal production could distinguish IgG4-HP from other disorders presenting with HP (OHP). In patients with IgG4-HP, the median CSF IgG4 concentration, IgG4 Index and IgG4Loc were significantly higher than in both controls and OHP. CSF IgG4 levels higher than 2.27 mg/dL identified 100% of...
39 CitationsSource
#1Hirotaka Ohara (Nagoya City University)H-Index: 42
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 68
Last. Hajime Takikawa (Teikyo University)H-Index: 32
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Background IgG4-sclerosing cholangitis (IgG4-SC) patients have an increased level of serum IgG4, dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall, and a good response to steroid therapy. However, it is not easy to distinguish IgG4-SC from primary sclerosing cholangitis, pancreatic cancer, and cholangiocarcinoma on the basis of cholangiographic findings alone because various cholangiographic features of IgG4-SC are similar to those of the above progre...
213 CitationsSource
#1Sing Yun Chang (Mayo Clinic)H-Index: 2
#2Karina A. Keogh (Mayo Clinic)H-Index: 22
Last. Eunhee S. YiH-Index: 47
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Granulomatosis with polyangiitis (Wegener's) (GPA) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral c...
59 CitationsSource
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