Seizure syndrome as a first manifestation of solitary tumor-like mass lesion of PACNS

Abstract

Rationale:Primary angiitis of the central nervous system (PACNS) is an inflammatory disease involving cerebrovascular and parenchymal, and solitary tumor-like mass lesion of PACNS (TLML-PACNS) is frequently misdiagnosed as neoplastic or other inflammatory diseases. However, seizure syndrome as a first manifestation of TLML-PACNS has rarely reported before.Patient concerns:Here, we report 2 cases of seizure syndrome, which was the first sign that presented prior to the diagnosis of TLML-PACNS by brain biopsy.Diagnoses:A mass lesion in the white and gray matters was detected by magnetic resonance imaging. The pathology for leptomeningeal lesion biopsy observed a transmural inflammation of the artery, with T lymphocyte infiltration. Patients were diagnosed with PACNS and epileptic seizure by biopsy and electroencephalogram.Interventions:Patients were treated with glucocorticoid pulse therapy for 3 days, and subsequently oral prednisone was continued, in combination with immunosuppressant.Outcomes:Luckily, both two patients were improved after treatment, and only mild cognitive impairment remained without adverse event.Lessons:Patient with mass lesion in CNS, which is similar to tumor, presented with seizure, headache, or cerebrovascular events without any other risk factors for stroke or tumor, should be considered the feasible with the disease of TLML-PACNS.

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

“…PACNS imaging methods can be divided into three categories based on the structures examined (16), of which MRI is the most common choice in practice (17). MRI findings of PACNS are sensitive but non-specific, and several studies have reported an MRI sensitivity ~90–100% (7, 14).…”

“…The solitary tumor-like mass lesions of PACNS are rare, which usually show high signal intensity on T2 and heterogeneously enhancing signals on Gd-enhanced images (17, 22–26). Previously reported mass-forming PACNS cases in adults from 2000 to the present are summarized in the Table 1.…”

Association Between Cerebrovascular Accident and Vasculitis: Myth or Reality?

“…Many cases were misdiagnosed as a brain tumor or an intracranial infection. Brain biopsy is the gold standard diagnostic modality, and cerebral angiography has low diagnostic power [34]. Magnetic resonance imaging is the preferred imaging in some hospitals, which shows single stenotic lesions in multiple blood vessels [35].…”

“…Also, there are no studies that prefer one immunosuppressive agent over others. One study showed good outcomes of using steroids and cyclophosphamide [34].…”

Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease

“…An underrecognized and rarer subset of PCNSV, approximately 5%-29%, can present with "masslike" or "tumefactive" lesions mimicking a neoplasm. 3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] To date, imaging features of tumefactive PCNSV (t-PCNSV) have not been well described and definitive diagnosis can only be made confidently on histopathology. [5][6][7][8][9][10] In this article, we performed a retrospective review of 10 histopathologically proved cases of t-PCNSV and analyzed pertinent imaging features with histopathologic correlation.…”