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Isolated Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Hypertrophic Pachymeningitis
Jeffrey Maneval1, Bronwyn Bryant2, Adam Ulano3
1From the Departments of Neurology.
Abstract:
A 65-year-old man with no pertinent medical history presented with 1 month of progressive holocephalic positional headaches (worse supine), photophobia, progressive gait instability resulting in multiple falls (ambulatory to walker in only 2 months), and weight loss. Testing found positive ANCA 1:160 perinuclear patter, myeloperoxidase >8.0. Cerebrospinal fluid found lymphocytic pleocytosis. We present his neuroimaging of isolated hypertrophic pachymeningitis with clinicoradiographic resolution after immunomodulatory pharmacotherapy along with histology from his meningeal biopsy. Isolated vasculitic myeloperoxidase-antineutrophil cytoplasmic antibody hypertrophic pachymeningitis is quite rare.
Insights
This study details a rare case of vasculitic hypertrophic pachymeningitis associated with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). Prompt immunomodulatory treatment led to significant clinical and imaging improvements.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Hypertrophic pachymeningitis is a rare condition characterized by thickening of the dura mater.
- Vasculitis, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, can affect the central nervous system.
- Myeloperoxidase-ANCA (MPO-ANCA) is a specific serological marker associated with certain types of vasculitis.
Observation:
- A 65-year-old man presented with progressive headaches, gait instability, and weight loss.
- Diagnostic workup revealed positive perinuclear ANCA (p-ANCA) with myeloperoxidase specificity, and lymphocytic pleocytosis in cerebrospinal fluid.
Findings:
- Neuroimaging demonstrated isolated hypertrophic pachymeningitis.
- The patient experienced clinicoradiographic resolution following immunomodulatory pharmacotherapy.
Implications:
- This case highlights the importance of considering ANCA-associated vasculitis in the differential diagnosis of hypertrophic pachymeningitis.
- Early diagnosis and treatment with immunomodulatory agents are crucial for favorable outcomes in this rare condition.
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