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Chorea-ballism associated with ketotic hyperglycemia
Chunli Chen1, Haiping Zheng, Li Yang
1Department of Neurology, Second Xiangya Hospital of Central South University, 139 Renmin Road, Changsha, 410011, Hunan, People's Republic of China.
Ketotic hyperglycemia (KHG) can cause chorea-ballism, a movement disorder. KHG-associated cases differ from nonketotic hyperglycemia (NKHG) cases, often presenting in younger patients with atypical brain imaging. Early glucose testing is crucial for diagnosis.
Area of Science:
- Neurology
- Metabolic Disorders
Background:
- Chorea-ballism is a rare movement disorder with involuntary movements.
- Nonketotic hyperglycemia (NKHG) and ketotic hyperglycemia (KHG) are common metabolic causes.
- Clinical features of NKHG-associated chorea-ballism are known, but KHG-associated features are not.
Purpose of the Study:
- To identify and characterize clinical and radiological differences between KHG- and NKHG-associated chorea-ballism.
Main Methods:
- Systematic literature search of MEDLINE (1960-May 2014) for patients with KHG-associated chorea-ballism.
- Analysis of 13 studies involving 15 patients.
Main Results:
- Patients with KHG-induced chorea-ballism were significantly younger than those with NKHG.
- A higher proportion of KHG patients exhibited atypical or negative brain imaging findings compared to NKHG patients.
Conclusions:
- Blood glucose testing on admission is recommended for early chorea-ballism diagnosis.
- Rapid glucose control with insulin is the primary treatment for KHG-induced chorea-ballism.
- Disease mechanisms may involve GABA, cerebrovascular insufficiency, or striatal dopaminergic alterations.
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