Differentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome

Primary angiitis of the central nervous system (PACNS) is a rare, inflammatory condition affecting small- and medium-sized vessels in the brain and spinal cord. Its diagnosis presents challenges due to nonspecific symptoms that mimic various neurological and psychiatric disorders. This case underscores the diagnostic complexity of PACNS and its treatment difficulties. Although a brain biopsy remains the gold standard for diagnosis, its invasiveness, potential to exacerbate neurological deficits, risk of bleeding and potential damage to surrounding brain tissue make it impractical for all patients. In this case, standard glucocorticoid and immunosuppressive therapies failed to halt disease progression, demonstrating PACNS's typically poor prognosis. This case highlights the urgent need for improved diagnostic strategies and novel treatments to achieve better clinical outcomes.

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Publication Details

Journal
BMJ Case Reports
Published
2025-10-01
DOI
https://doi.org/10.1136/bcr-2025-266850
Primary Topic
Vasculitis and related conditions
Type
article
Field-Weighted Citation Impact
0.00
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article

Differentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome

Daina Pastare, Anastasija Solodjankina
BMJ Case Reports
Vasculitis and related conditions
article

Differentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome

Daina Pastare, Anastasija Solodjankina
article en

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare, inflammatory condition affecting small- and medium-sized vessels in the brain and spinal cord. Its diagnosis presents challenges due to nonspecific symptoms that mimic various neurological and psychiatric disorders. This case underscores the diagnostic complexity of PACNS and its treatment difficulties. Although a brain biopsy remains the gold standard for diagnosis, its invasiveness, potential to exacerbate neurological deficits, risk of bleeding and potential damage to surrounding brain tissue make it impractical for all patients. In this case, standard glucocorticoid and immunosuppressive therapies failed to halt disease progression, demonstrating PACNS's typically poor prognosis. This case highlights the urgent need for improved diagnostic strategies and novel treatments to achieve better clinical outcomes.

BMJ Case ReportsVol. 18(10)
Riga East University Hospital (LV)
Openalex Percentile: Top 59%
Vasculitis and related conditions
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