MAGNETIC-RESONANCE-IMAGING IN CEREBROTENDINOUS XANTHOMATOSIS - A PROSPECTIVE CLINICAL AND NEURORADIOLOGICAL STUDY

被引：34

作者：

BERGINER, VM

BERGINER, J

KORCZYN, AD

TADMOR, R

机构：

[1] BEN GURION UNIV NEGEV,SOROKA MED CTR,DEPT RADIOL,BEER SHEVA,ISRAEL

[2] BEN GURION UNIV NEGEV,FAC HLTH SCI,BEER SHEVA,ISRAEL

[3] TEL AVIV UNIV,SACKLER SCH MED,ICHILOV MED CTR,DEPT NEUROL,IL-69978 TEL AVIV,ISRAEL

[4] TEL AVIV UNIV,SACKLER SCH MED,SHEBA MED CTR,NEURORADIOL SECT,IL-69978 TEL AVIV,ISRAEL

来源：

JOURNAL OF THE NEUROLOGICAL SCIENCES | 1994年 / 122卷 / 01期

关键词：

CEREBROTENDINOUS XANTHOMATOSIS; DIAGNOSIS; MRI; CT;

D O I：

10.1016/0022-510X(94)90059-0

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We describe MRI findings in 13 persons with typical clinical, EEG, CT and biochemical features of cerebrotendinous xanthomatosis (CTX). MRI showed brain (13/13) and cerebellar (12/13) atrophy and diffuse white matter hypodensity (4/13) presumably reflecting sterol infiltration with demyelination. Focal lesions were rare (2/13). Mass effect, edema or enhancement were not observed. Treatment with chenodeoxycholic acid (CDCA) 750 mg/day orally improved neurological and biochemical abnormalities. MRI appears to be of little value in following improvement after treatment has begun. Otherwise, the MRI studies are very sensitive and useful in diagnosing early incomplete forms of CTX.

引用

页码：102 / 108

页数：7

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