Uncontrollable Movements in Patient with Diabetes Mellitus Teaching

Practice

Teaching Case Report

Uncontrollable movements in patient with diabetes mellitus

The Case: A 63-year-old woman pre- sented to the emergency department after experiencing uncontrollable, irregular jerking movements of her right arm for 1 week. The movements were increasingly painful and were making it difficult for her to sleep; however, they disappeared during sleep. She reported no other symptoms. The patient had type 2 diabetes mellitus for 23 years, hypertension and arthritis. Cancer of the right breast had been treated with radiation and mas- tectomy 2 years before the current presentation. Her medications includ- ed tamoxifen, oxycodone, fentanyl, triazolam, enalapril, ranitidine, lasix, amitriptyline and indomethacin. She started taking insulin 10 years ago, with poor glycemic control. She checked her glucose infrequently and followed up with her family physician rarely. Blood glucose levels on test strips ranged from 20–30 mmol/L. On examination the patient’s vital signs were stable. Results of language testing, and cranial nerve and motor examinations were normal. There were Fig 1: CT scan, showing hyperdensity of left lentiform nucleus of basal ganglia. continuous and irregular jerking movements of her right arm and hand and, to a lesser extent, her right leg. The complete blood count and elec- order, and the patient was discharged to She could not willfully suppress the trolyte, blood urea nitrogen, creatinine the referring hospital. The patient was movements nor appreciably minimize and calcium levels were normal. The called back for a formal neurologic as- them forcibly with the other hand. random glucose level was elevated, at sessment after the emergency depart- There was infrequent grimacing of the 17.2 (normal 3.3–11.0) mmol/L. Also el- ment physician subsequently reviewed right side of her face (see video clip, evated were the hemoglobin A1c con- the history and CT scan with the neurol- available at www.cmaj.ca/cgi/content centration (13.8% [normal 4.3%– ogy team. A diagnosis of hemichorea– /full/175/8/871/DC1). Deep-tendon re- 6.1%]), creatine kinase level (668 [nor- hemiballismus as a complication of

1 flexes were absent in all limbs, and mal < 200] U/L) and cholesterol and nonketotic hyperglycemia was made. 4 5

0 there was no Babinski sign. Vibratory triglyceride levels. A CT scan of the Multiple medications in different 6 0 . j a sense was bilaterally diminished to the head showed hyperdensity of the left combinations were tried, including m c / ankles. There was dysmetria in the lentiform nucleus of the basal ganglia haloperidol, olanzapine, quetiapine, 3 0 5

1 right arm and leg and an unsteady (Fig. 1). clonazepam and tetrabenazine, with . 0 1

: gait, in keeping with the degree of in- The preliminary impression of the minimal immediate benefit. Severe I O

D voluntary movements. emergency staff was of a functional dis- pain from the continuous right arm

jerking was treated with morphine. tory and examination do not provide MRI scans. In most cases, these imaging Long-term tetrabenazine therapy for sufficient information.1 features completely reverse after therapy.2 the hemichorea was continued, to Our patient had poorly controlled dia- The prognosis of HC–HB as a com- which the patient had a satisfactory betes, and her HC–HB was a rare complication of nonketotic hyperglycemia response. Subsequent testing for Wil- plication of nonketotic hyperglycemia. is excellent.2 In a meta-analysis, 97% of son’s and thyroid disease yielded nega- Between 1985 and 2001, only 53 case re- patients had resolution of the abnor- tive results. An endocrinologist was ports of this condition were published.2 mal movements within 6 months.3 consulted to optimize diabetic control. The underlying pathophysiology leading Tight blood glucose control is some- to basal ganglia dysfunction is multi- times sufficient to treat the hemi- factorial. Recent imaging studies have chorea. More often, targeted mono- Hemichorea–hemiballismus (HC–HB) shown reduced cerebral glucose metabo- therapy or combination therapy with is a spectrum of involuntary, continu- lism on positron emission tomography neuroleptics is also required.2 Rarely ous, nonpatterned movements of one (PET) scans, with concomitant hyperper- surgical interventions, such as thalam- side of the body. It can be caused by a fusion in the affected basal ganglia seen otomy and deep brain stimulation, are focal lesion of the contralateral basal on single photon emission computed to- considered. ganglia or a diffuse systemic process mography (SPECT). These findings may Our case illustrates the diagnostic (Box 1). Other abnormal movements suggest a local failure in vascular au- challenges of movement disorders and must be distinguished from HC–HB toregulation in the setting of pre-existing an association of one with a common (Table 1). Also, the possibility of con- microangiopathic disease and recurrent medical condition. Unfamiliarity with tinuous focal seizures (epilepsia par- metabolic derangements.3 these disabling conditions may result tialis continua [EPC]) causing unilat- The basal ganglia in HC–HB appears in their attribution to psychological or eral movements must be considered. hyperdense without mass effect on CT psychiatric disturbances. A high index EPC can occur secondary to hyper- scans and hyperintense on T1-weighted of suspicion is warranted for neuro- glycemia; however, the movements are logic consultation and investigation of usually regular and rhythmic and often patients with abnormal movements. have corresponding electroencephalo- Table 1: Classification of hyperkinetic movement disorders gram abnormalities. EPC is often asso- Haley Block ciated with cortical lesions rather than Disorder Definition James Scozzafava subcortical (basal ganglia) lesions. As a S. Nizam Ahmed result, electroencephalography and Dystonia Sustained muscle Sanjay Kalra brain MRI scanning are helpful in dis- contraction with Division of Neurology abnormal posturing tinguishing these 2 entities, when his- Department of Medicine Athetosis Slow, writhing University of Alberta movements Edmonton, Alta. Chorea Irregular, unpredictable, Box 1: Causes of hemichorea– brief, jerky movements hemiballismus Competing interests: None declared. Ballismus Large-amplitude flailing Focal (contralateral basal ganglia) movements • Ischemic or hemorrhagic stroke Myoclonus Rapid, shock-like jerks REFERENCES • Infection of central nervous system Tremor Regular, rhythmic 1. Zumsteg D, Wennberg RA. A 61-year-old man with • Neoplasm oscillations at fixed continuous clonic jerks of his right leg. CMAJ interval 2005;173(7):754-5. Diffuse systemic process 2. Oh SH, Lee KY, Im JH, et al. Chorea associated Tic Abrupt, irregular, • Nonketotic hyperglycemia with non-ketotic hyperglycemia and hyperintensity suppressible movements basal ganglia lesion on T1-weighted brain MRI • Systemic lupus erythematosus that may mimic gestures study: a meta-analysis of 53 cases including four present cases. J Neurol Sci 2002;200:57-62. • Wilson’s disease Akathisia Inability to remain still 3. Hsu JL, Wang HC, Hsu WC. Hyperglycemia- • Thyrotoxicosis with a subjective feeling induced unilateral basal ganglion lesions with and of restlessness without hemichorea. A PET study. J Neurol 2004; 251:1486-90.

CMAJ • October 10, 2006 • 175(8) | 872