VA Neurology Report – Pachymeningitis!

Pachymeningitis is a  very rare disease associated with diffuse thickening of the dura and arachnoid mater. The earliest described cases were associated with tuberculosis and syphilis, but we now recognize a spectrum of potential causes.

 IgG4 disease is a relatively new entity that should be considered in the context of any cryptic/idiopathic infiltrative process!

Even with a rare disease (unknown to many in the room), we were able to build a DDx for pachymeningitis using the “Big Three” categories of medicine: Infection, Rheumatologic/Inflammatory, and Malignancy. We were also able to take a page out of our playbook for infiltrative diseases.

The DDx for pachymeningitis includes:
1) Infection: Tuberculosis, Syphilis

2) Rheum/Inflammatory: IgG4 disease, neurosarcoidosis, granulomatosis w/ polyangiitis (GPA), rheumatoid arthritis, and idiopathic pachymeningitis (the last is a dx of exclusion)

3) Malignancy: Lymphoma, en plaque meningioma

For a brief anatomy review:
Meninges: the three membranes that cover the brain and spinal cord
1) pia mater (closest to the CNS structures)
2) arachnoid mater
3) dura mater (furthest from CNS structures)

Leptomeninges = pia/arachnoid mater

Pachymeninges = arachnoid/dura mater


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