Abstract | OBJECTIVE: METHODS: Retrospective analysis was performed on 28 cases of PACNS confirmed by biopsy, and the age, gender, pathological results, course of the disease, imaging manifestations, treatment, and prognosis of the patients were analyzed and summarized. RESULTS: The cohort (age 16-60 years) comprised of 16 males. The average time from the visit to diagnosis was 6 months. The first symptom was chronic headache in 18 patients. The pathological results were accompanied by demyelination in 10 cases and glial hyperplasia in 6 cases. A total of 27 patients received treatments including glucocorticoid+cyclophosphamide; of these, 3 cases of craniotomy were improved. Among the 28 patients, 15 patients improved after the treatment, 12 patients had no significant improvement, and 1 patient was deceased. Patients with a long course of the disease before diagnosis, a Karnofsky performance status (KPS) score <60 at the time of diagnosis, a behavioral, cognitive abnormality before treatment, and a short-term relapse (0.3-1 month) have a poor outcome. CONCLUSIONS:
PACNS patients are prone to misdiagnosis and mistreatment, with unknown etiology and poor prognosis due to delayed treatment. Therefore, early biopsy, pathological diagnosis, and timely treatment with glucocorticoid shock are recommended, and patients with obvious mass effect should be treated by surgical resection.
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Authors | Hong-Bo Chang, Ming Gao, Jian-Ning Zhang, Wei-Dong Cao, Sheng-Li Guo, Peng Wang, Gang Cheng, Hu-Lin Zhao |
Journal | Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
(J Stroke Cerebrovasc Dis)
Vol. 29
Issue 12
Pg. 105400
(Dec 2020)
ISSN: 1532-8511 [Electronic] United States |
PMID | 33096491
(Publication Type: Journal Article)
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Copyright | Copyright © 2020 Elsevier Inc. All rights reserved. |
Chemical References |
- Glucocorticoids
- Immunosuppressive Agents
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Topics |
- Adolescent
- Adult
- Biopsy
- Central Nervous System
(pathology)
- Craniotomy
- Early Diagnosis
- Female
- Glucocorticoids
(therapeutic use)
- Humans
- Immunosuppressive Agents
(therapeutic use)
- Male
- Middle Aged
- Predictive Value of Tests
- Prognosis
- Retrospective Studies
- Risk Factors
- Time Factors
- Time-to-Treatment
- Vasculitis, Central Nervous System
(etiology, pathology, therapy)
- Young Adult
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