Primary angiitis of the central nervous system (PACNS) is a poorly understood form of vascular inflammatory disease that is restricted to the brain and spinal cord. A 38-year-old woman presented with severe headache and transient aphasia. Her cerebrospinal fluid exhibited aseptic meningitis, and high-resolution vessel-wall MRI (HRVW-MRI) revealed narrowing of multiple intracranial vessels with concentric wall thickening and diffuse enhancement, suggestive of PACNS. High-dose steroid and azathioprine therapy resulted in a significant improvement in vessel wall thickening, and enhancement was observed on the follow-up HRVW-MRI.
Adult ; Aphasia ; Azathioprine ; Brain ; Central Nervous System* ; Cerebrospinal Fluid ; Female ; Follow-Up Studies ; Headache ; Humans ; Magnetic Resonance Imaging* ; Meningitis, Aseptic ; Spinal Cord ; Vasculitis* ; Vasculitis, Central Nervous System

Signet-ring cell carcinoma of the ampulla of Vater is so rare that only 16 cases have been reported. Here we report a case of a 47-year-old man with signet-ring cell carcinoma in the ampulla of Vater. The patient was hospitalized for right upper quadrant abdominal pain and jaundice which had developed 10 days prior. Computed tomography (CT) and ultrasonography (US) revealed dilatation of the common bile duct and the intrahepatic bile duct. On duodenoscopy, a bulging mass with an irregular margin in the major papilla was noted. Histologic findings showed a signet-ring cell carcinoma. Surgical resection was not done because lymph node metastasis was noted during the operation. Hence, conservative treatment including chemotherapy and bile drainage was done.
Abdominal Pain ; Ampulla of Vater ; Bile ; Bile Ducts, Intrahepatic ; Common Bile Duct ; Dilatation ; Drainage ; Duodenoscopy ; Humans ; Jaundice ; Lymph Nodes ; Middle Aged ; Neoplasm Metastasis