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Hemiballismus in Patients with Poorly Controlled Type 2 Diabetes Mellitus

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Hemiballismus in Patients with Poorly Controlled Type 2 Diabetes Mellitus CASE IN POINT Hemiballismus in Patients With Poorly Controlled Type 2 Diabetes Mellitus Pressley Chakales, MD; Ariel Park; Austin Amos, MS; Esther Baldinger, MD; Igor Sirotkin, MD; and Alfred Frontera, MD Swift identification of hemiballismus as a complication of poorly controlled type 2 diabetes mellitus may help facilitate optimal care through glycemic control and prevent debilitating loss of patients' function and autonomy, prolonged hospital stays, and overuse of resources. Austin Amos and Ariel emiballismus is an acquired hyperki- numerous case reports have described pa- Park are Medical Students, netic movement disorder character- tients with DM with acute and subacute Pressley Chakales was a ized by unilateral, involuntary, often onset of hemiballistic and hemichorei- Medical Student at the time H the article was written; large-amplitude limb movements. Ballis- form movements while in a hyperglyce- Esther Baldinger and tic movements are now considered to be mic state or after its resolution. Reported Alfred Frontera are on the choreiform spectrum.1 Movements cases have been limited to small numbers Associate Professors of Neurology; and Igor Sirotkin usually involve both the arm and leg, and of patients with only a few larger-scale re- 7,9 is an Assistant Professor of in half of cases, facial movements such as views of more than 20 patients. Most re- 2,3 Neurology; all at the tongue clucking and grimacing are seen. ported cases involve geriatric patients and University of Central Florida College of Medicine in Presentations of hemiballismus vary in more commonly, females of Eastern Asian Orlando. Igor Sirotkin is an severity from intermittent to nearly con- descent with an average age of onset of Assistant Professor of tinuous movements, which, in some cases, 71 years.4,10 Patients typically present with Radiology at the may lead to exhaustion, injury, or disability. glucose levels from 500 to 1,000 mg/dL University of Southern Florida in Tampa. Esther Some patients are unable to ambulate or and hemoglobin A1c (HbA1c) levels almost Baldinger and Alfred feed themselves with the affected limb. double the normal values. Interestingly, Frontera are Neurologists neuroimaging findings in these patients and Igor Sirotkin is a BACKGROUND have consistently shown hyperintense sig- Neuroradiologist, all at Bay Pines VA Healthcare System The 2 most common causes of nal in the contralateral basal ganglia on in Florida. hemichorea-hemiballismus are stroke and T1-weighted magnetic resonance images Correspondence: hyperglycemia, with an incidence of 4% (MRIs). Noncontrast computed tomogra- Pressley Chakales 1,3,4 ([email protected]) and unknown incidence, respectively. phy (CT) shows well-defined unilateral in- Other causes include HIV, traumatic brain creased density in the contralateral basal injury, encephalitis, vasculitis, mass effect, ganglia without mass effect.1,9,11 multiple sclerosis, and adverse drug reac- This report aims to illustrate and enhance tions.4-7 Acute or subacute hemiballismus is the understanding of hemiballismus associ- classically attributed to a lesion in subtha- ated with hyperglycemia. One patient pre- lamic nucleus (STN), but this is true only sented to the US Department of Veterans in a minority of cases. Hemiballismus can Affairs (VA) Bay Pines VA Healthcare System be caused by any abnormality in various (BPVAHCS) in Florida, which motivated us subnuclei of the basal ganglia, including the to search for other similar cases. We reviewed classic location in the STN, striatum, and the charts of 2 other patients who presented globus pallidus.4 Evidence shows the le- to BPVAHCS over the past 10 years. The sions typically involve a functional network first case presented with severe hypergly- connected to the posterolateral putamen.8 cemia and abnormal movements that were Although not commonly recognized, not clearly diagnosed as hemiballismus. MRI hyperglycemia in patients with type 2 findings were characteristic and assisted in diabetes mellitus (T2DM) is the sec- making the diagnosis. The second case was ond most common cause of hemichorea- misdiagnosed as hemiballismus secondary to hemiballismus.3 Over the past 90 years, ischemic stroke. The third case was initially 282 • FEDERAL PRACTITIONER • JUNE 2020 mdedge.com/fedprac FIGURE 1 Noncontrast Axial T1-Weighted Magnetic Resonance Imaging of Case 1 Head A B C Contiguous axial slices demonstrate the varying levels of basal ganglia involvement. Arrows indicate increased signal in the right basal ganglia putamen (A), globus pallidus (B), and caudate (C). diagnosed as conversion disorder until move- Case 2 ments worsened and the correct diagnosis of A white male aged 71 years with a history hyperglycemia-induced hemichorea hemibal- of T2DM, hypertension, and hyperlipid- lismus was confirmed by the pathognomonic emia was admitted due to increased jerky neuroimaging findings. movements in the left upper extremity. On admission, his vital signs were within nor- CASE PRESENTATIONS mal limits and his physical examination dem- Case 1 onstrated choreoathetoid movements with A 65-year-old male with a history of un- ballistic components of his left upper extrem- controlled T2DM presented with repetitive ity. His laboratory results showed a glucose twitching and kicking movements that in- level of 528 mg/dL with a HbA1c of 16.3%. volved his left upper and lower extremities An initial CT obtained in the emergency de- for 3 weeks. The patient reported that he did partment (ED) demonstrated a well-defined not take his medications or follow the rec- hyperdensity in the striatal (caudate and len- ommended diabetes diet. His HbA1c on ad- tiform nucleus) region (Figure 2). There was mission was 12.2% with a serum glucose of no associated edema/mass effect that would 254 mg/dL. The MRI showed a hyperintense be typical for an intracranial hemorrhage. T1 signal within the right basal ganglia in- An MRI obtained 1 week later showed cluding the right caudate with sparing of the hyperintense TI signal corresponding to the internal capsule (Figure 1). There was no as- basal ganglia (Figure 3). In addition, there sociated mass effect or restricted diffusion. It was a questionable lacunar infarct in the was compatible with a diagnosis of hypergly- right internal capsule. Due to lack of aware- cemia-induced hemichorea-hemiballismus. ness regarding hyperglycemic associated The patient was advised to resume taking basal ganglia changes, the patient’s move- glipizide 10 mg daily, metformin 1,000 mg ment disorder was presumed to be ischemic by mouth twice daily, and to begin 10 units in etiology. The patient was prescribed oral of 70/30 insulin aspart 15 minutes before amantadine 100 mg 3 times daily for the meals twice daily, and to follow a low car- hemiballismus in conjunction with treat- bohydrate diet, with reduce dietary intake ment of his T2DM. The only follow-up oc- of sugar. At his 1-month follow-up visit, the curred 5 weeks later, which showed no patient reported an improvement in his in- improvement of uncontrollable movements. voluntary movements. At the 5-month Imaging at that time (not available) indi- follow-up, the patient’s HbA1c level was cated the persistence of the abnormal signal 10.4% and his hyperkinetic movements had in the right basal ganglia. This patient died completely resolved. later that year without further follow-up. mdedge.com/fedprac JUNE 2020 • FEDERAL PRACTITIONER • 283 Hemiballismus in Type 2 Diabetes FIGURE 2 Noncontrast Axial Computed FIGURE 3 Noncontrast Axial T1-Weighted Tomography Image of Case 2 Head Magnetic Resonance Image of Case 2 Head Arrow shows a well-defined region of increased Arrow shows a well-defined region of increased signal in the attenuation in the right basal ganglia (putamen, right basal ganglia. globus pallidus, and caudate head). Case 3 ceding the onset of his symptoms. A 78-year-old white male with a history of On admission a MRI showed a uni- syncope, transient ischemic attacks (TIAs), lateral right-sided T1 hyperintensity and poorly controlled T2DM presented with in the basal ganglia, no acute ischemia a 1-month history of progressively worsen- (Figure 4). In retrospect, subtle increased ing involuntary, left-sided movements that T1 signal can be seen on the earlier MRI began in his left shoulder and advanced to in- (Figure 5). In view of the patient’s left-sided volve his arm, hand, and leg, and the left side symptoms, DM, and MRI findings, a diag- of his face with grimacing and clucking of his nosis of hyperglycemia-induced hemicho- tongue. Three weeks earlier, the patient had rea-hemiballismus was made as the etiology been discharged from the ED with a diag- of the patient’s symptoms. nosis of conversion disorder particularly be- The patient was prescribed numerous cause he experienced decreased movements medications to control his hyperkinesia in- when given a dose of Vitamin D. It was over- cluding (and in combination): benztropine, looked that administration of haloperidol gabapentin, baclofen, diphenhydramine, had occurred a few hours before, and because benzodiazepines, risperidone, olanzapine, the sounds made by his tongue were not felt and valproic acid, which did not control his to be consistent with a known movement movements. Ultimately, his hyperglycemic disorder. A MRI of the brain was read as hemiballismus improved with tight glycemic normal. control and oral tetrabenazine 12.5 mg twice The patient returned 3 weeks later (the daily. This patient underwent a protracted original presentation)
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