Primary angiitis of the central nervous system mimicking dissemination from brainstem neoplasm: a case report

• Published in: Surgical neurology 2008-08, Vol.70 (2), p.182-185
• Main Authors: Nabika, Shinya, MD, Kiya, Katsuzo, MD, Satoh, Hideki, MD, Mizoue, Tatsuya, MD, Araki, Hayato, MD, Oshita, Jumpei, MD, Nishisaka, Takashi, MD, Kurisu, Kaoru, MD, Sugiyama, Kazuhiko, MD
• Format: Article
• Language: English
• Subjects: Aged; Autopsy; Biopsy; Brain Stem Neoplasms - diagnosis; Cerebral Arteries - pathology; Cerebral Arteries - physiopathology; Diagnosis, Differential; Disease Progression; Dysarthria - etiology; Dysarthria - pathology; Dysarthria - physiopathology; Encephalitis - pathology; Encephalitis - physiopathology; Fatal Outcome; Female; Fourth Ventricle - pathology; Fourth Ventricle - physiopathology; Gliosis - etiology; Gliosis - pathology; Gliosis - physiopathology; Humans; Lymphocyte Activation; Magnetic Resonance Imaging; Medulla Oblongata - blood supply; Medulla Oblongata - pathology; Medulla Oblongata - physiopathology; Neurology; Primary angiitis of central nervous system; Respiratory Insufficiency - etiology; Respiratory Insufficiency - physiopathology; Spinal Cord - blood supply; Spinal Cord - pathology; Spinal Cord - physiopathology; Steroids - therapeutic use; Surgery; Treatment Failure; Vasculitis, Central Nervous System - pathology; Vasculitis, Central Nervous System - physiopathology
• ISSN: 0090-3019; 1879-3339
• DOI: 10.1016/j.surneu.2007.05.008

Abstract Background Diagnosis of PACNS is very difficult because of the variety of clinical manifestation, especially neurologic and MRI findings. We report the case of a 68-year-old woman with PACNS. Case Description Her clinical symptoms mimicked malignant brainstem neoplasm disseminated to third and fourth ventricles and cervical spinal cord. Brain biopsy could not be correctly diagnostic. In spite of a trial of high-dose steroids, repeated MRI showed an increasing number of lesions. Two weeks after surgery, she died of respiratory dysfunction. Postmortem examination suggested PACNS, after consideration of histories, laboratory and radiologic findings, and the lack of systemic disease. Conclusion Although brain biopsy represents the gold standard measure, combination with several other examinations might be necessary to arrive at an early and definitive diagnosis. PACNS should be considered the differential diagnosis of intracranial tumors.