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Rare combo: moyamoya and lupus in men
Dilara Bulut Gökten1, Murat Gökten2, Çiğdem Deniz3
1Department of Rheumatology, Tekirdag Namik Kemal University, Tekirdag, Turkey. dilarabulutgokten@gmail.com.
Abstract:
Moyamoya syndrome (MMS) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries. Cerebral angiography images are used for detection through measurement. Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause multisystemic involvement. The coexistence of SLE and MMS has been rarely reported in the literature. A 46-year-old male patient with malar rash, Raynaud phenomenon presented to the hospital with a complaint of weakness in the left lower extremity, which began 3 days before the date of the visit. In the diffusion magnetic resonance imaging, multiple diffusion restrictions were observed in the right frontal region. The patient underwent MR angiography, revealing stenosis in the terminal and supraclinoid segments of the right internal carotid artery, which made us consider moyamoya disease. This patient, with a malar rash and Raynaud's, a positive antibody profile, was diagnosed as a male with SLE accompanied by MMS.
Insights
Systemic lupus erythematosus (SLE) can coexist with moyamoya syndrome (MMS), a rare cerebrovascular condition. This case highlights a male patient diagnosed with both SLE and MMS, presenting with neurological symptoms.
Area of Science:
- Neurology
- Rheumatology
- Radiology
Background:
- Moyamoya syndrome (MMS) is a progressive cerebrovascular disorder affecting intracranial arteries.
- Systemic lupus erythematosus (SLE) is an autoimmune disease with potential multisystemic impact.
- The co-occurrence of SLE and MMS is infrequently documented.
Observation:
- A 46-year-old male presented with left lower extremity weakness.
- Imaging revealed diffusion restrictions in the right frontal lobe and stenosis in the right internal carotid artery.
- The patient exhibited malar rash and Raynaud phenomenon, indicative of SLE.
Findings:
- Diagnostic workup confirmed stenosis consistent with moyamoya disease.
- The patient's clinical presentation and serological profile led to a diagnosis of SLE.
- The case established a diagnosis of SLE with coexisting moyamoya syndrome.
Implications:
- This rare association underscores the importance of considering MMS in SLE patients with neurological deficits.
- Early diagnosis and management of coexisting SLE and MMS can potentially improve patient outcomes.
- Further research is warranted to understand the pathogenic mechanisms linking SLE and MMS.
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