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[A case of dissecting aneurysm associated with mixed connective tissue disease]
1Department of Neurosurgery, Tsuruoka City Shonai Hospital, Yamagata, Japan.
Insights
A rare case report details a 41-year-old woman with mixed connective tissue disease (MCTD) who developed a dissecting aneurysm (DA) in her cerebral artery. This association highlights potential arterial fragility in MCTD patients, leading to serious complications like subarachnoid hemorrhage.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Mixed connective tissue disease (MCTD) is a rare autoimmune disorder with overlapping features of other connective tissue diseases.
- Cerebral artery aneurysms, particularly dissecting aneurysms, can lead to severe neurological complications such as subarachnoid hemorrhage.
- The association between MCTD and vascular abnormalities, including aneurysms, is not well-established but is increasingly recognized.
Observation:
- A 41-year-old female patient presented with symptoms indicative of a cerebral vascular event.
- Diagnostic criteria for MCTD were met, confirming the presence of the autoimmune condition.
- Cerebral angiography revealed characteristic signs of a dissecting aneurysm (DA) in the right posterior cerebral artery, including the double-lumen sign and contrast pooling.
Findings:
- The patient was diagnosed with both mixed connective tissue disease (MCTD) and a dissecting aneurysm (DA) of the right posterior cerebral artery.
- The findings suggest that the inherent fragility of the arterial wall, stemming from the primary MCTD, likely contributed to the development of the dissection.
- The dissection and subsequent subarachnoid hemorrhage were attributed to the compromised arterial integrity associated with MCTD.
Implications:
- This case underscores the importance of considering vascular complications in patients diagnosed with MCTD.
- Early diagnosis and management of cerebral artery dissection in MCTD patients are crucial to prevent potentially fatal outcomes.
- Further research is warranted to elucidate the precise mechanisms linking MCTD to arterial wall fragility and dissecting aneurysms.
Abstract:
A 41 year-old-female with mixed connective tissue disease (MCTD) associated with dissecting aneurysm (DA) of the right posterior cerebral artery is reported. This case satisfied the diagnostic criteria for MCTD, and cerebral angiography revealed the double-lumen sign and pooling of the contrast medium. Thus it was possible to make a definite diagnosis of both MCTD and DA. Fragility of the arterial wall due to the primary disease, MCTD, appeared to have played a role in the occurrence of dissection and the subarachnoid hemorrhage.