SCIENTIFIC
A Case of Uncontrolled Diabetes Complicated by Nonketotic Hyperglycemic Hemichorea
AUTHORS : Ryan Hayes 1
G . Hudson Beard , MD 2 Andrea Labus , MD 2
1
West Virginia University School of Medicine
2
Department of Internal Medicine , West Virginia University School of Medicine
CASE PRESENTATION
A 53-year-old male from West Virginia ( WV ) presented to the emergency department ( ED ) with a two-month history of mild tremors in the left upper extremity that suddenly progressed to dramatic , uncontrollable , ballistic movements of the left lower and upper extremities ( LLUE ). Medical history was significant for Type II diabetes mellitus ( T2DM ), hypertension , and hemorrhagic stroke in the right hemisphere involving the putamen 17 years prior . This patient was previously on treatment for T2DM , but not for the two years prior to presentation . On initial presentation , the patient was alert , oriented , and suffered no neurologic symptoms beyond his LLUE hemichorea and partial loss of sensation in the same extremities . Evaluation in the ED revealed a blood pressure of 215 / 150 mmHg , serum glucose of 520 mg / dL , and hemoglobin A1c of 12.7 %. Urinalysis was negative for the presence of ketones . A non-contrasted computed tomography ( CT ) scan of the head indicated a hyperdensity of the right striatum ( Figure 1 ). Based on his clinical presentation , laboratory , and CT findings , the patient was diagnosed with nonketotic hyperglycemic hemichorea ( NHH ).
After diagnosis , the patient was admitted and was prescribed a basal-bolus insulin regimen with sliding scale insulin . By the third day of admission , the patient achieved a serum glucose between 175-218 mg / dL and achieved near-remission of his hemichorea with some residual neuropathic pain in the left hand . On the fourth
ABSTRACT
day , the patient requested to be discharged home . He was instructed to follow up at an out-patient internal medicine clinic to establish care , receive further endocrine evaluation , and additional diabetes education . His discharge medications included glargine 25 units nightly , lispro 8 units three times daily after meals with sliding scale lispro . Of note , the patient did not attend the follow-up appointments with the endocrine clinic and was unable to be contacted despite multiple attempts .
DISCUSSION
T2DM is a chronic condition of systemic insulin resistance , eventually causing decreased insulin production by the pancreas . T2DM can be complicated by multiple conditions like neuropathy , nephropathy , hypertension , and more .
In the United States ( US ), 10 % of adults ages 18 years and older are diagnosed with T2DM . West Virginia has the second-highest prevalence with 15 % of adults being diagnosed . 1 , 2 As rates of T2DM increase in WV and the US , so will the rates of secondary complications like NHH . Furthermore , with increased incidence of NHH , there is an increased likelihood of atypical presentations of this condition , like the mild tremors that preceded hemichorea as seen in this case .
Chorea is an involuntary , large amplitude movement of the proximal limbs , and occasionally face . These movements are rapid , irregular , and occur in a nonstereotyped pattern . 3 This case illustrates the potential effects of uncontrolled hyperglycemia on central motor control . NHH is a rare and
Nonketotic hyperglycemic hemichorea ( NHH ) is a rare complication of uncontrolled Type II diabetes mellitus ( T2DM ). This report shares the case of a 53-year-old man from West Virginia presenting with an acute onset of hemichorea and a several-year history of uncontrolled T2DM . The patient ’ s physical examination , laboratory , and radiologic findings indicated severe hyperglycemia and striatal hyper-density , leading to the diagnosis of NHH . NHH should be included in the differential diagnosis of patients with acute movement disorders , especially in West Virginia with its high rate of Type II diabetes .
distressing complication of T2DM that results in unilateral hyperkinetic movement , often described as choreiform or ballistic . Despite the rare occurrence of NHH , it is the second-most common cause of acute , acquired chorea behind stroke , accounting for approximately 4 % of cases . 4-7 NHH has an average age onset of 71 years 8 and should be added to the differential diagnosis of any patient with sudden hyperkinetic movement with a known history of T2DM .
While a definitive causative mechanism for NHH has not been identified , it has been proposed that the hyperglycemic state may cause alterations in the blood-brain barrier and / or neurotransmitter metabolism . Due to the hyperglycemia seen in NHH , the brain depends upon anaerobic metabolism to obtain energy . This process suppresses the tricarboxylic acid cycle , making gamma-aminobutyric acid ( GABA ) the primary energy source . In nonketotic hyperglycemia , the lack of acetoacetic acid causes a rapid depletion of GABA . The loss of GABA can disturb thalamic-cortical feedback , causing the extrapyramidal symptoms seen in the disorder . 9 Furthermore , the presentation in this case demonstrates a slow onset of tremors that progressed to hemichorea . It must be considered that in some cases of chronic hyperglycemia , subacute hyperkinetic symptoms can be seen along with acute hemichorea .
Differentiating NHH from other types of acquired chorea can be an overwhelming task due to the numerous causes of acquired chorea , some of which are listed in Table 1 . 10 Genetic causes of chorea differ in their timing of onset but are major
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