IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil

Abstract Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is i...

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Veröffentlicht in:Journal of the neurological sciences 2012-07, Vol.318 (1), p.31-35
Hauptverfasser: Moss, Heather E, Mejico, Luis J, de la Roza, Gustavo, Coyne, Thomas M, Galetta, Steven L, Liu, Grant T
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container_end_page 35
container_issue 1
container_start_page 31
container_title Journal of the neurological sciences
container_volume 318
creator Moss, Heather E
Mejico, Luis J
de la Roza, Gustavo
Coyne, Thomas M
Galetta, Steven L
Liu, Grant T
description Abstract Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4–related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.
doi_str_mv 10.1016/j.jns.2012.04.010
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Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4–related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. 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subjects Adult
Biological and medical sciences
Biopsy
Central nervous system
Central Nervous System - immunology
Central Nervous System - pathology
Female
Granuloma, Plasma Cell - drug therapy
Granuloma, Plasma Cell - pathology
Human viral diseases
Humans
IgG4-related disease
Immunoglobulin G
Immunoglobulin G - biosynthesis
Immunohistochemistry
Immunosuppressive Agents - administration & dosage
Infectious diseases
Inflammation
Inflammatory pseudotumor
Intracranial mass
Medical sciences
Meningitis - drug therapy
Meningitis - pathology
Middle Aged
Mycophenolate mofetil
Mycophenolic Acid - administration & dosage
Mycophenolic Acid - analogs & derivatives
Neurology
Optic neuropathy
Pseudotumors
Skull
Viral diseases
Viral diseases of the lymphoid tissue and the blood. Aids
title IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil
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