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An orphan disease: IgG4-related spinal pachymeningitis: report of 2 cases.

Published on Dec 1, 2016in Journal of Neurosurgery
· DOI :10.3171/2016.4.SPINE1674
Bishan Dass Radotra19
Estimated H-index: 19
,
Ashish Aggarwal20
Estimated H-index: 20
+ 2 AuthorsDebajyoti Chatterjee4
Estimated H-index: 4
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Abstract
IgG4-related disease is relatively new disease entity and a rare one, and our knowledge of this entity continues to evolve. It was first described in the pancreas and since then has been described in virtually every organ. Spinal involvement resulting in pachymeningitis is rare, and there are only 8 reported cases of the same to date, with the cervicothoracic spine being the most commonly affected region. The authors describe 2 cases in which the patients presented with spinal compression resulting in myeloradiculopathy (Case 1) and radiculopathy (Case 2). Imaging of spine in both cases revealed an ill-defined contrast-enhancing lesion at the lumbar level. Preoperatively, a diagnosis of spinal tumor was made, but intraoperatively no spinal tumor was found. The diagnosis was established histopathologically. The disease has no particular defining features clinically or radiologically and can mimic common spinal tumors. It is important to accurately diagnose this rare entity because of its multisystem involv...
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References9
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#1Arezou Khosroshahi (Emory University)H-Index: 25
#2Zachary S. Wallace (Harvard University)H-Index: 17
Last.Hiroshi Goto (TMU: Tokyo Medical University)H-Index: 24
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#1Kazuichi Okazaki (Kansai Medical University)H-Index: 62
#2Kazushige Uchida (Kansai Medical University)H-Index: 34
Last.Makoto Takaoka (Kansai Medical University)H-Index: 22
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#1Katherine M. Lindstrom (UVA: University of Virginia)H-Index: 1
#2John B. Cousar (UVA: University of Virginia)H-Index: 31
Last.M. Beatriz S. Lopes (UVA: University of Virginia)H-Index: 28
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#1John H. Stone (Harvard University)H-Index: 67
#2Arezou Khosroshahi (Harvard University)H-Index: 25
Last.James R. Stone (Harvard University)H-Index: 48
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Cited By5
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#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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#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
Last.John H. Stone (Harvard University)H-Index: 67
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#1Kelly J. Bridges (OHSU: Oregon Health & Science University)H-Index: 2
#2Caitlin H. DeDeaux (OHSU: Oregon Health & Science University)
Last.Khoi D. Than (OHSU: Oregon Health & Science University)H-Index: 5
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#1Bernardo BaptistaH-Index: 1
#2Alina Casian (Guy's and St Thomas' NHS Foundation Trust)H-Index: 6
Last.Claire M Rice (Southmead Hospital)H-Index: 2
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