A 64-year-old man previously diagnosed with Waldenstrom's macroglobulinemia presented to our hospital with confusion. Magnetic resonance imaging (MRI) revealed diffuse meningeal enhancement. The patient was diagnosed with Bing-Neel syndrome (BNS) based on an elevated IgM index and the presence of monoclonal IgM protein, as detected by immunofixation electrophoresis of the cerebrospinal fluid. The patient underwent intrathecal and systemic chemotherapy but ultimately died of pneumonia. An autopsy revealed extensive meningeal and perivascular infiltration by malignant cells throughout the brain and spine. Thus, BNS may cause more extensive malignant infiltration into the central nervous system than is revealed by MRI.
A 48-year-old man had convulsions, and magnetic resonance angiography (MRA) showed diffuse constriction of the cerebral arteries. He was suspected of having primary angiitis of the central nervous system (PACNS) and treated with steroid for three days. The MRA abnormality disappeared after a week. After 69 days, he developed dizziness, and MRA revealed recurrence of cerebral artery stenosis. Nevertheless, the symptoms and abnormal MRA findings recovered promptly without treatment. He was diagnosed with reversible cerebral vasoconstriction syndrome (RCVS) without headache. This case suggests that RCVS should be a differential diagnosis in patients without headache whose MRA findings show multiple cerebral artery stenosis.
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