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[A rare neurologic syndrome due to a common medical condition]
Insights
Chorea Hyperglycemia Basal Ganglia syndrome, a rare condition, presents with involuntary movements in elderly patients with uncontrolled diabetes. Prompt insulin treatment can improve both neurological symptoms and brain imaging findings.
Area of Science:
- Neurology
- Endocrinology
- Geriatrics
Background:
- Chorea is an uncommon neurological disorder in the elderly, often associated with various underlying conditions.
- This case highlights a patient with multiple comorbidities including hypertension, diabetes mellitus, and renal failure, presenting with recurrent chorea.
Observation:
- A thorough diagnostic work-up failed to identify common causes of chorea in this elderly patient.
- The patient exhibited significantly elevated HbA1c levels (>17%) and brain CT scans revealed characteristic dense lesions in the basal ganglia.
Findings:
- The patient was diagnosed with Chorea Hyperglycemia Basal Ganglia syndrome, characterized by chorea, hyperglycemia, and basal ganglia lesions.
- Hemichorea, a type of chorea, was directly linked to non-ketotic hyperglycemia and demonstrated improvement with insulin therapy.
Implications:
- This case underscores the importance of considering Chorea Hyperglycemia Basal Ganglia syndrome in elderly patients with unexplained chorea and uncontrolled diabetes.
- Effective management of hyperglycemia through insulin treatment is crucial for clinical and radiological improvement in this syndrome.
Abstract:
We describe a patient with hypertension, diabetes mellitus, recurrent strokes, congestive heart failure, chronic atrial fibrillation and renal failure,who was hospitalized twice for chorea. A thorough work-up excluded all common causes for chorea in the elderly. The patient's HbA1c was > 17% and the brain computerized tomography revealed dense lesions in the basal ganglia compatible with Chorea Hyperglycemia Basal Ganglia syndrome. This syndrome includes a combination of chorea, hyperglycemia and dense lesions in the basal ganglia. The hemichorea in this syndrome is provoked by non-ketotic hyperglycemia and improves with insulin treatment. The radiographic picture also improves after there is control of glucose levels and clinical improvement.
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