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Hypertrophic cranial pachymeningitis associated with mixed connective tissue disease; a comparison with idiopathic
1Department of Neurology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Abstract:
This is the first report describing hypertrophic cranial pachymeningitis which developed in association with mixed connective tissue disease (MCTD). A 56-year-old man with a two-year history of MCTD gradually developed symptoms of headache and blurred vision. Bilateral papilledema and increased opening pressure and mild pleocytosis of the cerebrospinal fluid were noted. Magnetic resonance imaging of the brain revealed a diffuse thickening and a gadolinium-enhancement of the cranial dura mater. While five previous patients, diagnosed as idiopathic or infectious hypertrophic cranial pachymeningitis in our department, had either otitis media or sinusitis and showed multiple cranial nerve involvement, the present patient had neither otitis media nor sinusitis and did not show any cranial nerve involvement other than papilledema.
Insights
This study reports hypertrophic cranial pachymeningitis in a patient with mixed connective tissue disease (MCTD). This rare association highlights a new potential complication of MCTD, impacting the dura mater.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Mixed connective tissue disease (MCTD) is a rare autoimmune disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis.
- Hypertrophic cranial pachymeningitis (HCP) is a chronic inflammatory condition characterized by diffuse thickening of the dura mater.
Observation:
- A 56-year-old male with a two-year history of MCTD presented with headache and blurred vision.
- Clinical examination revealed bilateral papilledema.
- Cerebrospinal fluid analysis showed increased opening pressure and mild pleocytosis.
Findings:
- Brain MRI demonstrated diffuse dural thickening with gadolinium enhancement, consistent with HCP.
- Unlike previously reported HCP cases (idiopathic or infectious), this patient had no history of otitis media or sinusitis.
- Cranial nerve involvement was limited to papilledema, differing from the multiple cranial nerve deficits seen in other HCP patients.
Implications:
- This case suggests a potential link between MCTD and the development of HCP.
- It expands the spectrum of neurological complications associated with MCTD.
- Further research is warranted to elucidate the underlying mechanisms and clinical significance of this association.