Left Lower Arm Hemichorea: an Unusual Presentation of New-Onset Type 2 Diabetes Maria Jose Velasco Acuna,1 Paul Labinson,2 and Joseph Mcdermott3

CASE STUDIES CLINICAL PHARMACOLOGY UPDATE

Left Lower Arm Hemichorea: An Unusual Presentation of New-Onset Type 2 Diabetes Maria Jose Velasco Acuna,1 Paul Labinson,2 and Joseph McDermott3

iabetes mellitus is a growing body temperature of 96.5°F, blood public health concern and pressure of 124/80 mmHg, heart Da chronic metabolic disease rate of 70 bpm, and respiration rate worldwide. The most common clini- of 18 breaths/min. His physical ex- cal manifestations at the onset of di- amination was positive for sporadic abetes are blurry vision, weight loss, twisting movements of the left fore- polyuria, polydipsia, and polyphagia. arm and hand, signs of lower-extrem- Neurological symptoms are rare but ity peripheral vascular disease, and have been reported to be associated decreased sensation of vibration in a with initial presentation of type 2 sock distribution. diabetes, especially hyperglycemic Initial laboratory test results hyperosmolar nonketotic syndrome included a serum random glucose of (HHNS). Hemichorea-hemiballism 873 mg/dL, A1C of 13.2%, serum (HCHB) is a spectrum of involun- osmolality of 335 mOsm/kg, anion tary, continuous, nonpatterned move- gap of 24 mEq/L, and venous pH of ments involving one side of the body 7.35. A head CT scan showed signs of that is usually caused by a variety of small-vessel ischemic disease without hereditary neurological diseases, met- evidence of acute intracranial pathol- abolic disorders, post-rheumatic fever ogy (Figure 1). Given these findings, sequelae, strokes, and other vascular the patient was admitted to the inten- diseases. Herein, we report the case sive care unit and started on insulin of a man with left-side arm hemicho- infusion and intravenous fluids. rea (HC) who presented with HHNS A brain MRI ordered as part of and had resolution of his symptoms the initial evaluation showed no acute when his blood glucose was back cerebral abnormalities. An electroen- under control. This case report high- lights the association between HC 1Department of Endocrinology, Diabetes and Metabolism, UConn Health, and HHNS and reinforces the need Farmington, CT to recognize HHNS promptly, espe- 2Department of Endocrinology, Hartford cially in elderly patients who present Hospital, Hartford, CT with a neurological complaint. 3Department of Internal Medicine, Luis Vernaza Hospital, Guayaquil, Ecuador Presentation Corresponding author: Maria Jose Velasco A 72-year-old Caucasian man pre- Acuna, [email protected] sented with a 2-week history of https://doi.org/10.2337/cd16-0024 progressive writhing movements of his left arm, with occasional sudden ©2017 by the American Diabetes Association. Readers may use this article as long as the work amplitude excursions, associated is properly cited, the use is educational and not with polyuria, polydipsia, and unin- ■ FIGURE 1. Head CT scan shows for profit, and the work is not altered. See http:// tentionally weight loss of 10 lb. On creativecommons.org/licenses/by-nc-nd/3.0 chronic small-vessel ischemic disease. for details. admission, his vital signs included a

VOLUME 35, NUMBER 3, SUMMER 2017 183 CASE STUDIES cephalogram and lumbar puncture The exact pathophysiology on imaging is not uncommon. were ordered to rule out seizures of HCHB remains unclear (4). • Prompt diagnosis and treatment of and cerebrospinal fluid infection, Hyperglycemia induces cerebral hyperglycemia leads to complete respectively, and both yielded normal hypoperfusion and activates the resolution of symptoms. results. anaerobic pathway, causing a decrease Conclusion Within a few days, the patient’s in the level of gamma-aminobutyric This case underscores the importance glucose decreased to 170 mg/dL acid (GABA) in basal ganglia of recognizing atypical manifestations and his left forearm movements had neurons, which in turn causes a of diabetes to initiate prompt evalua- almost disappeared; by day 5, the HC reduction in acetylcholine synthesis. tion, avoid long-term complications, was completely resolved. GABA is the main inhibitory neu- and decreased hospital-related health One-month follow-up occurred rotransmitter in the basal ganglia costs. via a telephone call because the (4,5). Hyperviscosity induced by hyperglycemia causes a disruption of patient had decided to go to a dif- Duality of Interest ferent provider closer to his home for the blood-brain barrier, triggering a transient ischemic event in vulnerable No potential conflicts of interest relevant to future care. He reported that he had this article were reported. striatal neurons. This could cause the continued to be asymptomatic. astrocytic hypertrophy and edema References Questions that result in the characteristic MRI 1. Bedwell SF. Some observations on hemib- 1. Why is it important to screen changes (6,7). Putaminal petechial allismus. Neurology 1960;10:619–622 patients who present with move- hemorrhage has been also suggested 2. Prabhu S, Ramya N. Movement disor- ment disorders for diabetes? as a possible mechanism for HCHB, ders and diabetes: a study of South India. Internet J Neurol 2012;14 2. What radiological clues help given the findings of hyperintensity 3. Song CG, Yang X, Xing GH, Zhao in identifying HC as part of found on brain CT scans (8). CS. Hemichorea associated with nonke- HHNS, and are those finding Overall, the prognosis of HHNS- totic hyperglycemia in a female. Neuro necessary to make a diagnosis? induced chorea is excellent, with Endocrinol Lett 2012;33:489–492 3. What could explain the link rare exceptions (9), and depends on 4. Chang CV, Felicio AC, Godeiro CDO Jr, proper identification of undiagnosed et al. Chorea-ballism as a manifestation of between HHNS and HCHB? decompensated type 2 diabetes mellitus. diabetes and adequate control of Am J Med Sci 2007;333:175–177 Commentary blood glucose. Additionally, typical 5. Nath J, Jambhekar K, Rao C, Armitano Chorea secondary to hyperglycemia neuroleptic drugs and sometimes E. Radiological and pathological changes was first reported in 1960 (1). Most benzodiazepines are useful in the in hemiballism-hemichorea with striatal hyperintensity. J Magn Reson Imaging of the cases reported to date have treatment of choreic movements 2006;23:564–568 been secondary to HHNS in type 2 (4). A follow-up brain MRI after 6 6. Slabu H, Savedia-Cayabyab S, Senior diabetes patients, and some have been months will usually show resolu- P, Arnason T. Permanent haemichorea the first indication of hyperglycemia tion of the initial findings, but these associated with transient hyperglycemia. BMJ Case Rep 2011 (DOI: 10.1136/ (2). The average age of onset for HC abnormalities may persist for years. bcr.08.2011.4641) secondary to HHNS is 71 years, Clinical Pearls 7. Shan DE, Ho DM, Chang C, Pan HC, with a female-to-male ratio of 1.8:1. Teng MM. Hemichorea-hemiballism: an • HC associated with HHNS is a explanation for MR signal changes. Am J Most cases occur in Asian women rare manifestation of type 2 dia- Neuroradiol 1998;19:863–870 and the elderly (2,3). The mean A1C betes that usually occurs in female 8. Chang MH, Chian HT, Lai PH, Sy on presentation is 14%. Typically, in Asian and elderly individuals. CG, Lee SS, Lo YY. Putaminal petechial patients with HHNS chorea, hyper- haemorrhage as the cause of chorea: a • Patients who present with move- neuroimaging study. J Neurol Neurosurg intensities of the contralateral basal ment disorders should be screened Psychiatry 1997;63:300–303 ganglia are found on T1-weighted for diabetes. 9. Oh SH, Lee KY, Im JH, Lee MS. Chorea MRI with hypointensities found on • Brain imaging generally shows associated with non-ketotic hyperglycemia T2-weighted MRI. However, the lack contralateral basal changes to and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-anal- of these findings does not rule out the side of neurological findings. ysis of 53 cases including four present cases. this syndrome. However, a lack of abnormalities J Neurol Sci 2002;200:57–62

184 CLINICAL.DIABETESJOURNALS.ORG