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Type 2 Diabetes Presenting With Persistent Chorea

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his late 60s with a diagnosis of type 2 diabetes for more than 10 years that was complicated by neuropathy and retinopathy presented with persistent chorea in the left upper extremity for about 1 month despite aggressive correction with insulin. He was alert and oriented with healthy vital signs. No evidence of cognitive impairments was present on bedside testing results. The patient denied any recent infections and risk for human immunodefiency virus (HIV) infection. Neurological examination results were significant for choreiform and hemiballistic movements involving the left upper extremity. His home medications were carefully reviewed to rule out any medications that could cause chorea. A review of his previous records indicated an admission to an outside facility 3 weeks beforehand with severe hyperglycemia and a hemoglobin A1c level of 14.1% (to convert to the proportion of total hemoglobin, multiply by 0.01). Despite an aggressive correction of blood glucose levels and metabolic correction, his choreiform movements had remained unresolved. On admission, a complete electrolyte and toxicology panel, including calcium, magnesium, and phosphorus, yielded normal results. The patient’s serum glucose level was 159 mg/dL (to convert to millimoles per liter, multiply by 0.0555) and his hemoglobin A1c was 10.9% without ketoacidosis. Test results for antinuclear antibodies, lupus anticoagulants, and antiphospholipid antibodies were negative. Serum thyrotropin levels, liver function test results, and parathyroid hormone levels were healthy. Copper studies, a peripheral smear, and a routine electroencephalogram yielded normal results. A general screening for cancers yielded negative results. The patient underwent brain magnetic resonance imaging (MRI) (Figure), which showed a T1 hyperintensity in the right caudate head and a lentiform nucleus with no restricted diffusion. Oral administration of olanzapine, 2.5 mg daily, in divided doses with careful monitoring of blood glucose levels resulted in the complete resolution of chorea.

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C. Diabetic striatopathy

The patient’s presentation of a unilateral subacute onset of choreiform movement that was associated with pathognomonic MRI findings in the setting of long-standing diabetes led us to the diagnosis of diabetic striatopathy. Diabetic striatopathy is a constellation of neurologic abnormalities that is characterized by sudden-onset hemiballism or hemichorea and striatal abnormalities on neuroimaging in a poorly controlled patient with diabetes.1 Delayed onset of hemichorea after an episode of hyperglycemia with or without ketoacidosis has also been reported.2 Dyskinesias, including hemichorea-hemiballism, occur because of the dysfunction of the corpus striatum and are usually reversible with the restoration of homeostasis. Diabetic striatopathy is an underdiagnosed complication of diabetes that is more frequently encountered in type 2 diabetes than type 1 diabetes.3 Chorea has also been reported after hypoglycemic episodes in people with diabetes.4 The main mode of treatment includes the correction of the metabolic derangement by fluids and insulin infusion. Patients with refractory disease require additional drugs, including γ-aminobutyric acid–ergic, neuroleptic, or dopamine-depleting agents as in this case.5 In 90% of the patients, clinical symptoms completely resolve with or without radiologic resolution, varying from 2 to 28 days. As noted in the workup, metabolic disorders, such as hypernatremia and hyponatremia, hypercalcemia and hypocalcemia, hypomagnesemia, hyperthyroidism, and hypoparathyroidism and hyperparathyroidism can cause chorea and they need to be ruled out.

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Article Information

Corresponding Author: Mayur Chalia, MD, Department of Neurology, Penn State Milton S. Hershey Medical Center, 500 University Dr, Hershey, PA 17033 (mayurchalia@gmail.com).

Published Online: December 21, 2018. doi:10.1001/jamaneurol.2018.4089

Conflict of Interest Disclosures: Dr Subramanian reports receiving grant support from the National Institutes of Health, Commonwealth of Pennsylvania Tobacco Settlement Funds, American Parkinson Disease Foundation, Anne and Phillip Gladfelter III Foundation, Ron and Pratima Gatehouse Trust Fund, Allergan, Intec, and US Worldmeds and serving on the scientific advisory boards of Teva, Acadia, Adamas, and US Worldmeds.

References
References
1.
Abe  Y, Yamamoto  T, Soeda  T,  et al.  Diabetic striatal disease: clinical presentation, neuroimaging, and pathology.  Intern Med. 2009;48(13):1135-1141. doi:10.2169/internalmedicine.48.1996PubMedGoogle ScholarCrossref
2.
Lin  CJ, Huang  P.  Delayed onset diabetic striatopathy: hemichorea-hemiballism one month after a hyperglycemic episode.  Am J Emerg Med. 2017;35(7):1036.e3-1036.e4. doi:10.1016/j.ajem.2017.02.018PubMedGoogle ScholarCrossref
3.
Su  CS, Chang  YY, Liu  KT, Lan  MY, Liu  JS.  Risk factors for prolonged hemichorea-hemiballism caused by hyperglycemia.  Parkinsonism Relat Disord. 2012;18(1):96-98. doi:10.1016/j.parkreldis.2011.06.003PubMedGoogle ScholarCrossref
4.
Lee  D, Lee  D, Ahn  TB, Hong  IK, Kim  DY.  Recurrent hemichorea after a hypoglycemic episode.  Parkinsonism Relat Disord. 2014;20(6):676-677. doi:10.1016/j.parkreldis.2014.03.006PubMedGoogle ScholarCrossref
5.
Bashir  H, Jankovic  J.  Treatment options for chorea.  Expert Rev Neurother. 2018;18(1):51-63. doi:10.1080/14737175.2018.1403899PubMedGoogle ScholarCrossref
6.
Dean  SL, Singer  HS.  Treatment of Sydenham’s chorea: a review of the current evidence.  Tremor Other Hyperkinet Mov (N Y). 2017;7:456.PubMedGoogle Scholar
7.
Williams  KA, Swedo  SE.  Post-infectious autoimmune disorders: Sydenham’s chorea, PANDAS and beyond.  Brain Res. 2015;1617:144-154. doi:10.1016/j.brainres.2014.09.071PubMedGoogle ScholarCrossref
8.
Bowen  J, Mitchell  T, Pearce  R, Quinn  N.  Chorea in new variant Creutzfeldt-Jacob disease.  Mov Disord. 2000;15(6):1284-1285. doi:10.1002/1531-8257(200011)15:6<1284::AID-MDS1043>3.0.CO;2-YPubMedGoogle ScholarCrossref
9.
Suri  R, Rodriguez-Porcel  F, Donohue  K,  et al.  Post-stroke movement disorders: the clinical, neuroanatomic, and demographic portrait of 284 published cases.  J Stroke Cerebrovasc Dis. 2018;27(9):2388-2397. doi:10.1016/j.jstrokecerebrovasdis.2018.04.028PubMedGoogle ScholarCrossref
10.
Nakawah  MO, Lai  EC.  Post-stroke dyskinesias.  Neuropsychiatr Dis Treat. 2016;12:2885-2893. doi:10.2147/NDT.S118347PubMedGoogle ScholarCrossref
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