Meninges, IgG4-related pachymeningitis [LMP65234]

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Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Diseases of the Immune System
Diagnosis: 
IgG4-related pachymeningitis
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Male
Age: 
72 years
Diagnosis Code: 
ICD-10: M35.9
Slide Contributor Information
Gao, A., Munoz, D.

Keywords

IgG4, IgG4-related disease, hypertrophic pachymeningitis, brain, meninges

Clinical History

72-year-old man with new-onset seizure. Neuroimaging shows dural-based mass of the left lateral sphenoid wing with mass effect on the frontal and temporal lobes.

Clinical Discussion

IgG4-related pachymeningitis is a presumed autoimmune disease of the meninges that can occur in isolation or in the setting of systemic IgG4-related disease. Diagnostic histopathologic criteria for IgG4-related pachymeningitis are dense lymphoplasmacytic infiltration of the meninges with more than 10 IgG4-positive cells per high power field. IgG4-related disease is typically responsive to steroids but long term patient outcomes are still lacking.