Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases
Introduction
Chorea is rarely observed in patients with non-ketotic hyperglycemia [1], [2], [3]. A high signal intensity lesion at the basal ganglia may be seen by T1-weighted brain magnetic resonance imaging (MRI). Although chorea associated with non-ketotic hyperglycemia and high signal intensity lesions on T1-weighted brain MRI (C-H-BG) is recognized as a peculiar syndrome, its overall clinical features are not well defined. In this paper, results of an analysis of the clinical and radiological features of the 49 reported patients and four patients with C-H-BG at this hospital are presented.
Section snippets
Material and methods
The medical records from 1990 to 2001 at both Yongdong Severance Hospital (general hospital with 800 beds) and Asan Medical Center (general hospital with 2140 beds) were searched to identify patients with C-H-BG. The literature from 1985 to October 2001 of patients with C-H-BG was also searched using MEDLINE. Only cases with detailed clinical and radiological findings were included in this review. The clinical data obtained from the patients at this hospital and those in the literature were
Results
Twenty articles that reported 49 patients with C-H-BG were found [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23]. Two patients from the medical records of each hospital were also found. A total of 53 patients with C-H-BG was analyzed.
Discussion
A combination of chorea, non-ketotic hyperglycemia, and a high signal basal ganglia lesion on the T1-weighted brain MRI study has been considered to be a unique syndrome. However, the precise mechanism of C-H-BG is unknown. Most reported patients are Asians, suggesting a genetic influence or an inadequate diabetes control system in underdeveloped countries.
C-H-BG occurs predominantly in women. One study including only eight women with C-H-BG suggested an involvement of dopamine hypersensitivity
Acknowledgments
This study was supported by Brain Korea 21 Projects, Yonsei University College of Medicine, Seoul, Korea.
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