Ataxia as the main manifestation of tumor-like primary angiitis of the central nervous system: a case report and literature review

• Published in: BMC medical imaging 2019-10, Vol.19 (1), p.79-79, Article 79
• Main Authors: Huang, Yu-Jing, Zhang, Li, Mao, Ying, Nan, Guang-Xian
• Format: Article
• Language: English
• Subjects: Aged; Ataxia; Ataxia - diagnostic imaging; Ataxia - etiology; Biopsy; Blood; Brain MRI examination; Care and treatment; Case Report; Case reports; Case studies; Central nervous system; Central nervous system diseases; Cerebellum; Cerebellum - diagnostic imaging; Cerebellum - pathology; Cyclophosphamide; Data collection; Dexamethasone; Dexamethasone - administration & dosage; Dexamethasone - therapeutic use; Diagnosis; Disease; Glucocorticoid therapy; Glucocorticoids; Glucocorticoids - administration & dosage; Glucocorticoids - therapeutic use; Hemispheric laterality; Histochemistry; Histopathology; Humans; Immunoglobulins; Infusions, Intravenous; Intravenous administration; Laboratories; Literature reviews; Male; Medical imaging; Mimicry; Nervous system; NMR; Nuclear magnetic resonance; Prognosis; Radiology; Rare diseases; Signs and symptoms; Spectrum analysis; Therapy; Treatment Outcome; Tuberculosis; Tumor-like primary angiitis of the central nervous system; Tumors; Vasculitis; Vasculitis, Central Nervous System - complications; Vasculitis, Central Nervous System - diagnostic imaging; Vasculitis, Central Nervous System - drug therapy
• ISSN: 1471-2342; 1471-2342
• DOI: 10.1186/s12880-019-0382-9

Primary angiitis of the central nervous system (PACNS) is a rare disease, and tumor-like primary angiitis of the central nervous system is even rarer. Histopathology is the gold standard for tumor-mimicking PACNS. However, pathological diagnosis is relatively limited due to fewer biopsy opportunities. A 68-year-old male presented with ataxia, and was diagnosed with tumor-like primary angiitis of the central nervous system. The patient underwent Intravenous drip glucocorticoid therapy (10 mg of dexamethasone, daily). After 10 days, the symptoms of the patient were completely relieved. Radiology revealed that the low density lesion in the right cerebellar hemisphere obviously narrowed. Cyclophosphamide therapy was not initiated. It is crucial for clinicians to be aware of changes in radiology that indicate PACNS, since the diagnosis of tumor-like PACNS remains quite challenging. Glucocorticoid therapy is an effective therapy in this condition, and the prognosis can be favorable.