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Published on: May 10, 2018
Persistent chorea triggered by hyperglycemic crisis in diabetics
J E Ahlskog1, H Nishino, V G Evidente
1Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Insights
Severe hyperglycemia can cause persistent chorea (involuntary movements) in women, even after blood sugar levels normalize. This movement disorder may require long-term neuroleptic treatment.
Area of Science:
- Neurology
- Endocrinology
- Movement Disorders
Background:
- Hyperglycemia, particularly severe episodes (500-1,000 mg/dL), is a known but poorly understood cause of neurological dysfunction.
- Chorea, a hyperkinetic movement disorder, has been anecdotally linked to hyperglycemic crises, often in older individuals with established diabetes.
Observation:
- Five female patients presented with new-onset chorea coinciding with or following severe hyperglycemic episodes.
- Four patients had no prior history of diabetes mellitus, suggesting hyperglycemia as the primary trigger.
- The chorea was subacute, varied in presentation (generalized, unilateral), and persisted long-term (6 months to 5 years) despite glucose normalization.
Findings:
- Unlike previously reported cases, the chorea in these patients was largely irreversible.
- Magnetic resonance imaging (MRI) revealed basal ganglia abnormalities (T1 hyperintensity) in four patients, but not in the most clinically affected individual.
- No other predisposing factors besides hyperglycemia were identified, highlighting its critical role.
Implications:
- This study suggests a subset of patients may develop persistent, treatment-refractory chorea following severe hyperglycemia.
- The findings challenge the notion of chorea exclusively as a reversible complication of hyperglycemia.
- Further research into the pathophysiology of hyperglycemia-induced persistent chorea is warranted to guide therapeutic strategies.
Abstract:
Five female patients developed chorea concurrent with, or shortly after a hyperglycemic episode (admission glucose values 500-1,000 mg/dL). In four of these five patients, there was no prior history of diabetes mellitus. The chorea continued despite correction of blood glucose and persisted to the time of last follow-up, 6 months to 5 years later. The chorea developed subacutely over 2 days to 1 month and was generalized in one, unilateral in three, and involved right > left lower extremity in the other; the severity initially reached ballistic proportions in two. Associated clinical features were nil in four of these patients, but cognitive impairment and personality change occurred in one. The histories and laboratory studies identified no predisposing factors other than the hyperglycemia. The chorea was sufficiently troublesome to require administration of neuroleptic medication in all five cases. Four of the five cases had high signal intensity within basal ganglia on T1-weighted magnetic resonance (MR) imaging, as has previously been described; however, this was not seen in one case (who had the most severe clinical condition). Most previously described cases have involved a reversible clinical syndrome, in contrast to our patients. The pathogenic mechanisms remain uncertain.
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