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A case of suspected MPO-ANCA-related hypertrophic pachymeningitis with atypical presentation
Tomoyuki Koura1, Keiichiro Kita2, Hirofumi Konishi3
1Department of Toyama Primary Care University of Toyama School of Medicine Toyama Japan.
Abstract:
A 66-year-old man presented to our emergency department with acute consciousness disorder, headache, and fever. Initial laboratory data indicated a high level of inflammatory reaction. Enhanced magnetic resonance imaging revealed a thickening of the right cranial dura mater on T1-weighted images. We presumed the patient was diagnosed as having myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-related hypertrophic pachymeningitis (HP). Acute impaired consciousness without focal signs is an unusual presentation of HP. This treatable disease should be considered as a differential diagnosis when a patient presents with drowsiness and headache.
Insights
Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-related hypertrophic pachymeningitis (HP) can present with acute consciousness disorder. This case highlights HP as a treatable differential diagnosis for patients with drowsiness and headache.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Hypertrophic pachymeningitis (HP) is a rare condition characterized by inflammation and thickening of the dura mater.
- Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis is a potential cause of HP.
- Unusual presentations of HP can delay diagnosis and treatment.
Observation:
- A 66-year-old male presented with acute consciousness disorder, headache, and fever.
- Laboratory tests revealed a significant inflammatory response.
- Enhanced MRI demonstrated thickening of the right cranial dura mater.
Findings:
- The patient was diagnosed with MPO-ANCA-related hypertrophic pachymeningitis (HP).
- Acute impaired consciousness without focal neurological signs was an atypical initial manifestation.
- The findings underscore the importance of considering HP in the differential diagnosis.
Implications:
- Early recognition of MPO-ANCA-related HP is crucial for timely and effective treatment.
- Clinicians should consider HP in patients presenting with unexplained drowsiness and headache, even without focal signs.
- This case expands the understanding of HP's clinical spectrum and diagnostic considerations.
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