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Primary angiitis of the central nervous system as a mimic of multiple sclerosis: A case report
Eduardo Macedo de Souza Tieppo1, Tomás Fraga Ferreira da Silva1, Roger Santana de Araujo1
1Neuroimmunology Division, Department of neurology, Hospital das Clínicas da Faculdade de Medicina da USP, Brazil.
Background:
Primary angiitis of the central nervous system is a rare inflammatory vasculopathy and it is a difficult diagnosis to make because of its kaleidoscopic presentation and its multiple mimics, including multiple sclerosis.
Case Presentation:
A 21-year-old men presented a four-year history of progressive gait deterioration. Magnetic resonance imaging of the brain and spine showed hyperintense round-shaped lesions on T2 images, many with contrast enhancement, in supra/infratentorial and spinal segments. He received treatment for multiple sclerosis but presented clinical worsening, and follow-up neuroimaging showed persistent contrast enhancement lesions and a cerebellar hematoma. Brain biopsy was performed and demonstrated inflammatory infiltrations in blood vessels. The patient received 6 monthly schedules of 5 g methylprednisolone and 1 g cyclophosphamide with clinical stabilization.
Discussion:
Our patient presented a primary angiitis central nervous system according to the Birnbaum and Hellmann proposed criteria. This case reinforces the importance of advancing the differential diagnosis of patients that present red flags in brain neuroimaging.
Conclusion:
The presence of the micro/macrobleeds and persistent contrast enhancing lesions should raise the suspicion of vasculitis in the differential diagnosis of multiple sclerosis.
Insights
Primary central nervous system vasculitis is a rare condition mimicking multiple sclerosis. Early suspicion of vasculitis is crucial for patients with concerning neuroimaging findings like bleeds and enhancing lesions.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare vasculopathy with diverse presentations, often mimicking other neurological conditions like multiple sclerosis (MS).
- Diagnosing PACNS is challenging due to its varied clinical manifestations and overlapping features with conditions such as MS.
Observation:
- A 21-year-old male presented with a four-year history of progressive gait deterioration.
- Neuroimaging revealed hyperintense lesions in the brain and spine, some with contrast enhancement. Despite treatment for MS, the patient worsened.
- Brain biopsy confirmed inflammatory infiltrates in blood vessels, indicating vasculitis.
Findings:
- The patient was diagnosed with PACNS based on established criteria.
- Treatment with methylprednisolone and cyclophosphamide led to clinical stabilization.
Implications:
- This case highlights the importance of considering vasculitis in the differential diagnosis of patients with neurological red flags on imaging.
- The presence of micro/macrobleeds and persistent contrast-enhancing lesions on MRI should prompt suspicion for vasculitis, especially when MS is considered.
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