Journal List > Kosin Med J > v.28(2) > 1057003

Kim, Moh, Jung, Lee, Woo, Lee, and Noh: A Tubercus Sclerosis Case Accompanied by Cystic Angiomyolipoma and Chronic Kidney Disease Diagnosed during Treatment for Acute Cerebral Infarction

Abstract

Symptoms of tuberous scelrosis (TS) are mainly related with brain and kidneys. Seizure, mental retardation, other behavioral problems are dominant. A spectrum of renal tumors from benign angiomyolipoma (AML) to polycystic kidney disease, and rarely malignant renal cell carcinoma have been observed. Cystic AML is a rare phenotype of AML. No case of TS with renal cystic AML has been reported in Korea yet. And chronic kidney disease (CKD) in TS has been seldom reported. We experienced a TS case accompanied by renal cystic AML and CKD diagnosed in a 48-year-old female patient who was hospitalized for left side weakness and seizure under the diagnosis of acute cerebral infarction.

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Fig. 1.
Genogram. ◎ (patient)
kmj-28-177f1.tif
Fig. 2.
(A) Brain CT shows multiple calcifications suggesting calcified tubers in right frontal periventricular area and left frontal subcortical white matter (arrows), (B) FLAIR image of MRI shows multifocal linear and wedge shaped high signal lesions suggesting cortical and subcortical tubers in both frontal cortex and subcortical white matter (arrows).
kmj-28-177f2.tif
Fig. 3.
Cystic angiomyolipoma showing fat component (arrows) in the left kidney in abdominal CT.
kmj-28-177f3.tif
Fig. 4.
(A) T2, (B) T1 fat setting, (C) T1 GRE, (D) T1. About 3.8 cm sized cystic mass with mixed components suggesting cystic angiomyolipoma and multiple cysts are seen (arrows) in MRI examination.
kmj-28-177f4.tif
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