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


METHODS
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.


CONCLUSIONS
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.
Nabika
Shinya
S
Department of Neurosurgery, Hiroshima Prefectural Hospital, Minami-Ku, Hiroshima 734-8530, Japan. snabika@go4.enjoy.ne.jp
Kiya
Katsuzo
K
Satoh
Hideki
H
Mizoue
Tatsuya
T
Araki
Hayato
H
Oshita
Jumpei
J
Nishisaka
Takashi
T
Kurisu
Kaoru
K
Sugiyama
Kazuhiko
K
D000368:Aged; D001706:Biopsy; D020295:Brain Stem Neoplasms / Q000175:diagnosis*; D002536:Cerebral Arteries / Q000473:pathology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004401:Dysarthria / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D004660:Encephalitis / Q000473:pathology / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D020546:Fourth Ventricle / Q000473:pathology / Q000503:physiopathology; D005911:Gliosis / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008213:Lymphocyte Activation; D008279:Magnetic Resonance Imaging; D008526:Medulla Oblongata / Q000098:blood supply* / Q000473:pathology* / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology; D013116:Spinal Cord / Q000098:blood supply / Q000473:pathology / Q000503:physiopathology; D013256:Steroids / Q000627:therapeutic use; D017211:Treatment Failure; D020293:Vasculitis, Central Nervous System / Q000473:pathology* / Q000503:physiopathology
D013256:Steroids