False-Positive Accumulation of Metaiodobenzylguanidine in a Case with Acute Intermittent Porphyria
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概要
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We report a 36-year-old woman presenting with hypertensive encephalopathy followed by bulbar palsy and quadriplegia. After an extensive screening for secondary causes of hypertension, the patient was suspected of having pheochromocytoma due to increased levels of catecholamines in the plasma and the urine, and positive 131I-metaiodobenzylguanidine (MIBG) accumulation in the gallbladder. However, MIBG accumulation was not reproducible without any tumors accompanying this accumulation in the gallbladder. A diagnosis of acute intermittent porphyria was finally confirmed based on the characteristic pictures, increased urinary excretion of porphobilinogen, and identification of a heterozygous missense mutation of R173W in the hydroxymethylbilane synthase gene. This case highlights a pitfall in utilizing MIBG to detect a source of excessive catecholamine and also suggests the importance of having a complete clinical history and extensive work-up of any possible differential diagnosis. We also review the potential mechanism by which falsepositive MIBG accumulation occurs.
著者
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YOSHIMURA Toshiro
The First Department of Internal Medicine, Nagasaki University School of Medicine
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Kawakami Atsushi
The First Department Of Internal Medicine Nagasaki University School Of Medicine
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Horie Yutaka
Department Of Pathology Tottori University Hospital
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Ando Takao
The First Department Of Internal Medicine Nagasaki University School Of Medicine
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Motomura Masakatsu
The First Department of Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
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Masuda Tomoko
The First Department of Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
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Ando Takao
The First Department of Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
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Ota Rie
The First Department of Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
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Maeda Naoto
The Second Department of Internal Medicine, Tottori University Faculty of Medicine, Japan
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Yoshimura Toshiro
The First Department of Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
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Horie Yutaka
Department of Gastroenterology, Shimaneken Saiseikai-Gotsu General Hospital, Japan
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Motomura Masakatsu
The First Department of Internal Medicine, Graduate School of Biomedical Science, Nagasaki University
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