Hyperglycemic chorea/ballism ascertained over 15 years at a referral medical center

doi:10.1016/j.parkreldis.2017.12.032

Parkinsonism & Related Disorders

Volume 48, March 2018, Pages 97-100

https://doi.org/10.1016/j.parkreldis.2017.12.032 Get rights and content

Highlights

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Hyperglycemia causes about 1% of acquired chorea, which is usually persistent.
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Chorea developing within 1 month of an episode of hyperglycemia is suggestive.
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Putamen T1 hyperintensity occurs in half and is often misdiagnosed as hemorrhage.
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Advanced age and newly diagnosed type 2 diabetes are commonly seen.
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Most cases are unilateral and responsive to dopamine blocking or depleting agents.

Abstract

Objective

To describe chorea/ballism triggered by a hyperglycemic event.

Methods

We used the electronic records system at Mayo Clinic–Rochester to identify patients diagnosed with chorea or ballism from January 1st, 2000 through December 31st, 2014. Each record was reviewed to confirm chorea/ballism. From these cases we selected those that developed chorea/ballism within a month after a hyperglycemic episode (blood glucose >300 mg/dL). Clinical, laboratory, and imaging findings were analyzed.

Results

Of the 596 chorea cases, we identified 7 patients (5 women) whose chorea was preceded by a hyperglycemic episode (range 3–30 days) during 15 years of surveillance, including new-onset diabetes in four cases. Median age was 80 years (range, 53–86). The chorea/ballism was unilateral in 6/7 cases and half of these unilateral cases had contralateral putamen T1-hyperintensity on brain MRI. After glucose correction, the chorea resolved within one week without recurrence in only one case. Among the 6 cases with persistent chorea, it was controlled with dopamine blocking/depleting medications.

Conclusions

Chorea triggered by hyperglycemia is a rare complication of diabetes, with only seven cases identified at our tertiary medical center during 15 years of surveillance. This comprised about 1% of all chorea cases at our center during this time. Hyperglycemic chorea primarily developed in later life, with new-onset diabetes in the majority (4/7). Although MRI putamen T1-hyperintensity is reportedly typical, it was only seen in 3/6 cases. This MRI appearance may be mistaken for a hemorrhagic stroke, given the usual unilateral presentation. The chorea was controlled with dopamine blocking/depleting medications.

Introduction

Acquired, adult-onset chorea/ballism that is unrelated to medications or stroke has a relatively limited differential diagnosis, such as chorea associated with circulating lupus anticoagulant, JAK2-polycythemia vera or a paraneoplastic syndrome (e.g., CRMP-5).

A distinctive syndrome of chorea/ballism developing during or shortly after an episode of non-ketotic hyperglycemia may be initially overlooked or misdiagnosed [[1], [2], [3], [4]]. This is often associated with T1 MRI hyperintensity of the striatum/putamen [1,2,4,5], sometimes misinterpreted as striatal hemorrhage [6,7]. The presentation of the chorea/ballism may be dramatic, with the chaotic, hyperkinetic movements impairing normal motor function. The purpose of this study is to ascertain the frequency of this uncommon condition at a large referral center (Mayo Clinic - Rochester), and characterize the demographics, treatment responses and outcomes.

Section snippets

Methods

We used the electronic records system at Mayo Clinic–Rochester to identify patients diagnosed with chorea or ballism during a 15 year epoch, from January 1st, 2000 through December 31st, 2014. This would have included reviews of all in-patient and out-patient evaluations from the entire Mayo Clinic database. Each medical record was individually reviewed to confirm chorea/ballism. We then selected those cases where chorea/ballism was time-locked to hyperglycemic episodes (i.e., within 30 days;

Results

During the 15 year study period, 740 Mayo Clinic patients matched our search criteria. After chart review, 596 cases were confirmed as having chorea/ballism. We then identified patients where chorea/ballism subacutely developed in association with a discreet episode of marked hyperglycemia (glucose >300 mg/dL), totaling seven cases. These seven cases reflected approximately 1% of all Mayo Clinic chorea cases during the 15 years of study. Of the seven patients, four were also given a new

Discussion

Non-ketotic hyperglycemia appears to be a very uncommon cause of chorea, with only about 1% of our Mayo chorea cases having this etiology (7/596). Although not tabulated in this study, it seems very likely that chorea complicating non-ketotic hyperglycemia is even less common. This begs the question whether such affected patients have predisposing features. Although no unique factor surfaced in our analysis, notable among our affected patients were several demographic findings. This included

Conclusion

Neurologists, movement disorder specialists, primary care physicians, internists, and endocrinologists may encounter hyperglycemic chorea/hemiballism. The subacute onset and unilaterality may suggest a stroke, or the associated MRI T1 striatal hyperintensity may suggest a cerebral bleed. The correct diagnosis is suggested by the temporal association with a hyperglycemic episode. Often the chorea is pronounced, and a dopamine blocking drug (neuroleptic) is required. However, continuation of

Authors' roles

Conor Ryan (CR), J. Eric Ahlskog (JEA), Rodolfo Savica (RS).

1) Research project: A. Conception (JEA, RS), B. Organization (CR, JEA, RS), C. Execution (CR, RS);
2) Statistical Analysis: A. Design (CR, JEA, RS), B. Execution (CR, RS), C. Review and Critique (CR, JEA, RS);
3) Manuscript: A. Writing of the first draft (CR), B. Review and Critique (JEA, RS).

Financial disclosures and conflicts of interest

None (for any authors).

Funding sources

None.

References (11)

S.H. Lee
Chorea-ballism associated with nonketotic hyperglycaemia or diabetic ketoacidosis: characteristics of 25 patients in Korea
Diabetes Res. Clin. Pract.
(2011)
J.E. Ahlskog
Persistent chorea triggered by hyperglycemic crisis in diabetics
Mov. Disord.
(2001)
C. Cosentino
Hemichorea/Hemiballism associated with hyperglycemia: report of 20 cases
Tremor Other Hyperkinet. Mov. (N.Y.)
(2016)
N. Kandiah
Hyperglycemic choreoathetosis: role of the putamen in pathogenesis
Mov. Disord.
(2009)
K.H. Chang
Temporal features of magnetic resonance imaging and spectroscopy in non-ketotic hyperglycemic chorea-ballism patients
Eur. J. Neurol.
(2010)

There are more references available in the full text version of this article.

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Short communicationHyperglycemic chorea/ballism ascertained over 15 years at a referral medical center

Highlights

Abstract

Objective

Methods

Results

Conclusions

Introduction

Section snippets

Methods

Results

Discussion

Conclusion

Authors' roles

Financial disclosures and conflicts of interest

Funding sources

Diabetes Res. Clin. Pract.

Persistent chorea triggered by hyperglycemic crisis in diabetics

Mov. Disord.

Hemichorea/Hemiballism associated with hyperglycemia: report of 20 cases

Tremor Other Hyperkinet. Mov. (N.Y.)

Hyperglycemic choreoathetosis: role of the putamen in pathogenesis

Mov. Disord.

Temporal features of magnetic resonance imaging and spectroscopy in non-ketotic hyperglycemic chorea-ballism patients

Eur. J. Neurol.

Short communication
Hyperglycemic chorea/ballism ascertained over 15 years at a referral medical center