A Tubercus Sclerosis Case Accompanied by Cystic Angiomyolipoma and Chronic Kidney Disease Diagnosed during Treatment for Acute Cerebral Infarction

Heejun Kim; Inho Moh; Da-Hye Jung; Young-Ki Lee; Ji-Young Woo; Yul Lee; Jung-Woo Noh

doi:10.7180/kmj.2013.28.2.177

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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

Case Report

Kosin Medical Journal 2013;28(2):177-182.

Published online: 19 January 2013

DOI: https://doi.org/10.7180/kmj.2013.28.2.177

A Tubercus Sclerosis Case Accompanied by Cystic Angiomyolipoma and Chronic Kidney Disease Diagnosed during Treatment for Acute Cerebral Infarction

Heejun Kim¹, Inho Moh¹, Da-Hye Jung¹, Young-Ki Lee¹, Ji-Young Woo², Yul Lee², Jung-Woo Noh¹

¹Department of Internal Medicine, Hallym Kidney Research Institute, Seoul, Korea

²Radiology, Hallym University College of Medicine, Seoul, Korea

Corresponding author: Jung Woo Noh, Department of Internal Medicine, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, 948-1 Daerim 1-dong, Yeoungdeungpo-gu, Seoul 150-950, Korea TEL: +82-2-829-5510 FAX: +82-2-848-9821 E-mail: jwn8671@unitel.co.kr

Received 7 September 201211 October 2012 Accepted 22 October 2012

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Angiomyolipoma, Cerebral infarction, Chronic renal failure, Tuberous sclerosis

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Fig. 1.

Genogram. ◎ (patient)

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).

Fig. 3.

Cystic angiomyolipoma showing fat component (arrows) in the left kidney in abdominal CT.

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.

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