Hemichorea-hemiballism in a nonketotic diabetic patient.

Hemichorea-hemiballism can be the solely presentation of a wide range of non-neurological clinical pictures, such as metabolic or hydro-electrolyte derange-ments. Hemichorea-hemiballism as the first presentation of type 2 diabetes mellitus has been described. The case depicted herein reinforces this association highlighting that especially in elder patients with new-ly diagnosed hemichorea-hemiballism, non-ketotic hyperglycemia should promptly be recognized.

Introduction

Diabetes is common all over the world. It leads to complications related to every system of the body. Movement disorders namely chorea, hemiballismus-hemichorea, and choreoathetosis can be induced by hyperglycemia. Early recognition of hyperglycemia induced movement disorders is important, as hyperglycemia is an easily treatable disorder and these carries a good prognosis.[1] It should also be noted that movement disorders can be one of the presenting feature of diabetes mellitus. Hence, screening all patients who present with involuntary movements for hyperglycemia, even in patients without a previous history of diabetes is important.[1] We hereby report an interesting case of hemichorea-hemiballism due to hyperglycemia with complete remission of symptoms after control of blood sugar level.

Case Report

A 67-year-old male normotensive, nonsmoker, and diabetic patient presented with involuntary movements of left side of the body. He was known diabetic for 10 years and on good control with oral gliclazide preparation. Recently, he discontinued the medication for 5 days without any reason. His family members mentioned that the patient was reasonably well till the evening he presented. There was no history of fever or intake of any other drugs. Patient was oriented, conscious, and vital signs were normal. Patient was violently moving his left sided limbs [Video 1]. Routine investigations were in normal limit except blood glucose was 35 mmol/l. There were no ketone bodies in urine. Computed tomography (CT) brain showed nonenhancing hyperdensities in the right sided basal ganglia region. Controlling of blood glucose reduced the intensity of movements. Oral tetrabenazine added prior to discharge from hospital. A follow-up visit after 4 weeks showed no residual movements.

Discussion

Movement disorders have been documented with diabetes mellitus.[12345678] These involuntary movements can be due to hereditary reasons, drugs, metabolic causes-hyperglycemia, thyroid/parathyroid disorders, infections, immunological, and perinatal hypoxia.[1] Metabolic cause is one of the easily treatable and completely reversible cause of involuntary movements. Among the metabolic disorders, diabetes mellitus has a high prevalence.[1] Diabetes and impaired glucose tolerance showed increasing trend with age.[11] Stroke and diabetes mellitus remain as the major causes of movement disorders.[12] Majority of the patients who reported with movement disorders caused by nonketotic hyperglycemia were Asians, due to possible genetic predisposition.[7912] It can also be a presenting symptom of diabetes.[23410] Proposed hypothesis for hyperglycemia as a cause of these movement disorders in diabetes are a) hyperglycemia induces mild ischemia in the putamen via hypoperfusion and b) it induces anaerobic metabolism which leads on to gamma-aminobutyric acid (GABA) depletion.[8] It mostly occurs in females of 50-80 years of age.[1] Brain CT shows hyperdensity of putamen/caudate nucleus, resolves eventually with resolution of symptoms. The cause of hyperdensity is due to protein hydration inside the cytoplasm of swollen gemistocytes.[57] Elder female diabetic patients from East Asian origin are more prone to develop hemichorea-hemiballism, but whether this is related to a particular genetic predisposition is still a matter of debate. Recently, it was suggested that the presence of acanthocytes in circulating peripheral blood might render diabetic patients prone to develop hemichorea-hemiballism.[6] In our case, we did not search for acanthocytes on the peripheral blood since a good relief of symptoms was obtained with glycemia control and the use of neuroleptics. The prognosis of nonketotic hyperglycemia-induced hemichorea-hemiballism is favorable and depends on the prompt identification of undiagnosed diabetes or the compensation of previously diagnosed patients. Additionally, typical neuroleptics and sometimes benzodiazepines are useful in the management of the choreic movements. Topiramate, levetiracetan, and tetrabenazine have also been tried in selected cases with favorable outcomes.[8]

In conclusion, this case report underscores the nonketotic hyperglycemia-induced hemichorea-hemiballism syndrome as an unusual presentation of type 2 diabetes mellitus, highlighting that its recognition and stringent glycemia control associated to the use of neuroleptics hasten the patient recovery. Moreover, hemichorea-hemiballism should be regularly included in the differential diagnosis of acute movement disorders, especially in the elder population.