Purpose or Case Report:
Juvenile-onset systemic lupus erythematosus is a form of systemic lupus erythematosus, an autoimmune disease that occurs in approximately 10–20% of cases before the age of 16. When the central nervous system is involved, the condition is termed neuropsychiatric lupus. These symptoms typically develop within the first year after diagnosis and are linked to a higher risk of mortality.
A 15-year-old male patient with a medical history of systemic lupus erythematosus, antiphospholipid syndrome, and lupus nephritis presented to the emergency department with decreased visual acuity and paralysis of the left sixth cranial nerve. Additionally, elevated blood pressure levels were recorded during his admission.
Given the symptoms, a brain MRI without contrast was performed. Punctate and linear focal areas in the right hemipontine and bulbopontine junction, which were hyperintense on T2/FLAIR and hypointense on T1, with no diffusion restriction or post-gadolinium enhancement, suggestive of malacia. Additionally, irregularities in the caliber of the left vertebral, basilar, and right posterior cerebral arteries were observed, characterized by alternating dilated and stenotic areas resembling a pearl necklace, along with wall irregularities indicative of vasculitis. Treatment was initiated, however the response was inadequate. During hospitalization, the patient experienced a sudden neurological deterioration. A non-contrast head CT and CT angiography were performed, revealing Fisher IV subarachnoid hemorrhage and persistent findings in the posterior cerebral arterial circulation. Later, he developed right hemiparesis, a second brain MRI, which identified a cerebral vascular event in the left middle cerebral artery's partial territory in a subacute stage. Additionally, there was a reduction in caliber and intensity of both middle cerebral arteries, suggestive of vasospasm, while the posterior cerebral circulation showed no changes compared to previous studies. Finally, cerebral angiography was conducted, confirming the previously described findings.
Vasculitis is a characteristic feature of juvenile-onset systemic lupus erythematosus, the incidence of CNS vasculitis in systemic lupus erythematosus is around 7–10%. MRI is the preferred modality for characterization; however, diagnosis is challenging for radiologists due to the diverse clinical manifestations, which present a broad spectrum of severity and nonspecific imaging findings.
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