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

doi:10.1016/S0022-510X(02)00133-8

Journal of the Neurological Sciences

Volume 200, Issues 1–2, 15 August 2002, Pages 57-62

https://doi.org/10.1016/S0022-510X(02)00133-8 Get rights and content

Abstract

Background: Chorea associated with non-ketotic hyperglycemia and high signal intensity lesions on T1-weighted brain magnetic resonance images (C-H-BG) is recognized as a unique syndrome that affects elderly women exclusively. However, its overall clinical features are unclear. Material and Methods: The literature describing patients with C-H-BG from 1985 to 2001 was reviewed using MEDLINE. Their clinical features and those of four patients with C-H-BG at this hospital were analyzed. Results: This study included 49 patients from the literature and four patients at this hospital. Their mean age at the onset was 71.1 years (range=22–92 years). Women were affected more frequently than men (men/women=17:30). The mean serum glucose level measured after the onset of chorea was 481.5 mg/dl (ranging from 169 to 1264), HbA1c level was 14.4% (ranging from 9.9 to 19.2), and the serum osmolarity was 305.9 mmol/kg (ranging from 291 to 335). Forty-seven patients developed hemichorea. Six patients developed bilateral chorea, and magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. MRI showed that putamen was involved in all cases (isolated putamen=31 patients, additional basal ganglia lesions=22 patients). None had lesions confined to the caudate nucleus or the globus pallidus. In all, except one, the anterior limb of the internal capsule was spared. Follow-up MRI studies were performed in 22 patients. In most, hemichorea improved along with the disappearance of the lesions. In 39 patients, chorea had ameliorated completely. The remaining 14 cases showed some improvement during the follow-up period. The chorea recurred in seven patients. Conclusion: C-H-BG is a benign disorder affecting the elderly. It affects men much more frequently than has been reported. The high signal intensity basal ganglia lesion on the T1-weighted brain MRI study was reversible, and correlated with the clinical improvement in chorea.

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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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

Abstract

Introduction

Section snippets

Material and methods

Results

Discussion

Acknowledgments

J. Neurol. Sci.

J. Neurol. Sci.

Parkinsonism Relat. Disord.

Metabolism

Lancet

Nonketotic hyperglycemia appearing as choreoathetosis or ballism

Arch. Int. Med.

Hemiballism–hemichorea and non-ketotic hyperglycemia

J. Neurol. Neurosurg. Psychiatry

Alternating choreoathetosis associated with uncontrolled diabetes mellitus and basal ganglia calcification

Diabetes Care

Putaminal petechial hemorrhage as the cause of chorea: a neuroimaging study

J. Neurol. Neurosurg. Psychiatry

Hemichorea–hemiballism: an explanation for MR signal changes

Am. J. Neuroradiol.

Exploration of motor cortex excitability in a diabetic patient with hemiballism–hemichorea

Mov. Disord.

Hyperglycemia and hemorrhagic transformation of cerebral infarcts

Stroke

Hemiballismus–hemichorea in older diabetic women: a clinical syndrome with MRI correlation

Neurology

Two diabetics with hemichorea–hemiballism and striatal lesions

No to Shinkei

Chorea–ballismus with nonketotic hyperglycemia in primary diabetes mellitus

Am. J. Neuroradiol.

Hemichorea–hemiballism associated with non-ketotic hyperglycemia and presenting with unilateral hyperintensity of the putamen on MRI T1 weighted images

No to Shinkei

Hemichorea in hyperglycemia associated with increased blood flow in the contralateral striatum and thalamus

Int. Med.

Putaminal hemorrhage accompanied by hemichorea–hemiballism

Stroke