74878ournal ofNeurology, Neurosurgery, and Psychiatry 1994;57:748-750 SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.6.748 on 1 June 1994. Downloaded from Hemiballism-hemichorea and non-ketotic hyperglycaemia Juei-Jueng Lin, Ming-Key Chang Abstract patients, comprises a spectrum ranging from Three patients with hemiballism-hemi- a mild degree of hyperosmolality with mini- chorea caused by non-ketotic hypergly- mal symptoms to severe hyperosmolality caemia are presented, two of whom had accompanied by coma.'2 To the best of our hyperosmolar non-ketotic hypergly- knowledge, there have been no reports of this caemic syndrome. In two of the three type of hyperkinesia being caused by HNKS. patients, the hyperkinesia was the initial presenting symptom of their diabetes mellitus. The hypersensitivity of the Methods postmenopausal dopamine receptor, From January 1986 to December 1992, a decreased y-aminobutyric acid in the total of 20 patients with hemiballism-hemi- brain in non-ketotic hyperglycaemia, chorea were registered at the special clinic for coexisting lacunar infarct in the basal movement disorders in the neurological ganglion, and pre-existing metabolic department of our hospital; in three of these dysfunction in the basal ganglion may all the disease was caused by non-ketotic hyper- have played a part in the pathogenesis of glycaemia. Two of the three had HNKS this movement disorder. (table 1), based on the fact that patients with HNKS have hyperglycaemia, an absence of (7 Neurol Neurosurg Psychiatry 1994;57:748-750) ketonaemia, and a plasma osmolality greater than 320 mmol/kg.13 Various structural lesions have been associ- ated with hemiballism-hemichorea. Lesions Case reports in the contralateral subthalamic nucleus and PATIENT 1 pallidosubthalamic pathways seem to play a Patient 1 was a 74 year old woman without a critical part,1A and acute vascular stroke is the history of diabetes mellitus, but with hyper- http://jnnp.bmj.com/ most common pathological process.4 tension and cirrhosis of the liver for several Department of Metabolic disorders such as thyrotoxicosis, years. She suddenly developed continuous, Neurology, Tri-Service General Hospital and systemic lupus erythematosis, and dysfunc- arhythmic, and purposeless choreiform invol- National Defense tion of glucose metabolism also cause this untary movements, mainly affecting her left Medical Center, hyperkinaesia.4-'0 arm. The symptoms were aggrevated by talk- Taipei, Taiwan, Republic of China Disorders of glucose metabolism alone ing, or when asked to extend the left arm; J-J Lin rarely cause hemiballism-hemichorea. Non- were less prominent when relaxed; and non- M-K Chang ketotic hyperglycaemia, as one of complica- existent during sleep. Neurological examina- on October 2, 2021 by guest. Protected copyright. Correspondence to: tions of diabetes mellitus, may be associated tion was normal, except for reduced deep Juei-Jueng Lin, Department of Neurology, Tri-Service with various neurological abnormalities, tendon reflexes and hemichorea in the left General Hospital, No 8, including delirium and coma, focal and arm. Blood glucose concentration was 30 5 Sec 3, Ting-Chow Rd, 107, Taipei, Taiwan, Republic generalised seizures, focal neurological mmol/l and the estimated blood osmolality of China. deficits and stroke-like syndromes, and hallu- was 332 mmol/kg. No ketones were detected. Received 13 August 1993 cination." Hyperosmolar non-ketotic hyper- She was dehydrated. Insulin was given intra- and in revised form 25 October 1993. glycaemic syndrome (HNKS), often an acute venously and symptoms disappeared within Accepted 12 November 1993 complication of diabetes mellitus in older two days. At this time her blood glucose con- centration was 13-8 mmol/I and blood osmo- lality was 290 mmol/kg. A brain CT showed of bilateral Table 1 Clinical data of three patients with hemiballism-hemichorea caused by cortical atrophy and calcification hyperosmolar non-ketotic hyperglycaemia basal ganglion. No Age(y)/ Blood sugar Osmolality Site of involuntary Sex (mmolll) (mmollkg) movement DM history Brain CTfindings PATIENT 2 Patient 2, a 78 year old man with a history of 1 74/F 30 5 322 Left upper limb - Cortical atrophy Calcication of BG** hypertension but without diabetes mellitus, 2 78/M 61-4 367 Right upper limb - Cortical atrophy had polydipsia, polyuria, and weight loss, Face 3 75/F 36-3 390 Right upper limb + Lower density in right accompanied for two weeks by involuntary BG, periventricular region writhing movements over his face and right DM = diabetes mellitus; BG = basal ganglion forearm. Examination revealed uncontrolled Hemiballism-hemichorea and non-ketotic hyperglycaemia 749 and purposeless choreiform movements, initial presenting symptoms of diabetes melli- occasionally accompanied by rapid uncon- tus are rare; only Hann et al'4 and Sanfield et J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.6.748 on 1 June 1994. Downloaded from trolled flinging ballistic movements of his al'0 have reported cases of generalised chorea right arm. Involuntary twitching and grimac- and hemichorea. ing of the right side of his face were also seen. A total of nine cases of hemiballism-hemi- Neurological examination was otherwise nor- chorea caused by non-ketotic hyperglycaemia mal. His blood glucose concentration was have been reported, mostly in older women 61-4 mmol/l and the osmolality was 367 (table 2). Oestrogen can decrease the mmol/kg. No ketonaemia was noted. He was dopamine (DA) function of the nigrostriatal rehydrated and treated with insulin and system, and subsequently increase the density antibiotics intravenously. The next morning, of the DA receptors.'5 16 Concentrations of the involuntary movements had disappeared, oestrogen decrease in women after the but asterixis of both hands was seen later. menopause, which contributes to the devel- Treatment with insulin was continued, and opment of supersensitivity in the striatal DA the patient's blood glucose concentration was receptor. Studies have also shown the con- controlled around 7-2-13.9 mmol/l. Three centration of DA in the striatum and substan- days later, the asterixis also disappeared. A tia nigra to be normal or slightly increased in CT of the brain showed only cortical atrophy. patients with Huntington's chorea,'7 and that hemichorea can be suppressed by neuroleptic PATIENT 3 drugs.418 We postulate that DA hypersensitiv- Patient 3 was a 75 year old woman with a his- ity in postmenopausal women is the reason tory of diabetes mellitus and hypertension. for their predisposition for developing this She suddenly developed a right sided weak- type of hyperkinesia with non-ketotic hyper- ness with bouts of drowsiness. Hemichorea of glycaemia. her right arm was also noted. Neurological In hyperglycaemia, cellular energy demand examination showed her to be drowsy with a shifts towards anaerobic metabolism, which right hemiparesis. The deep tendon reflexes inhibits the function of the tricarboxylic acid were symmetrically reduced, with flexor plan- cycle and causes the brain to metabolise y- tar responses. Her blood glucose was 36-6 aminobutyric acid as an alternative energy mmol/l and estimated blood osmolality was source.'9 Patients with ketosis would have an 390 mmol/kg. She was rehydrated and abundant source of acetoacetate from which treated with insulin and antibiotics. The next y-aminobutyric acid might be resynthesised, morning, the hyperkinesia and weakness had but non-ketotic patients would be rapidly resolved. Her blood glucose concentration depleted. In animal studies, y-aminobutyric was 16'4 mmol/l and blood osmolality was acid antagonists can cause a lesion in the sub- 329 mmol/kg. A brain CT revealed an old thalamic nucleus, inducing contralateral infarct lesion in the left basal ganglion, with hemichorea.20 We therefore propose that the low density in the periventricular region. depletion of y-aminobutyric acid in the brain may play an important part in the pathogene- sis of the non-ketotic hyperglycaemia, and Discussion HNKS induced hemiballism-hemichorea. Hemiballism-hemichorea caused by non- Most cases of hemiballism-hemichorea ketotic hyperglycaemia was first reported by associated with non-ketotic hyperglycaemia http://jnnp.bmj.com/ Bedwell in 1960. He described a 65 year old have occurred in elderly patients with hyper- woman who developed ballistic movements in tension or diabetes mellitus. Hypertension all four limbs during three episodes of hyper- and diabetes mellitus greatly increase the risk glycaemia.7 Patients with hyperkinetic move- of stroke, as well as increase the incidence of ments caused by non-ketotic hyperglycaemia lacunar infarction of the brain.2' Deep lacu- have been reported by Rector et al,8 Tortoritis nar infarctions may not be visible on CT, so et al,9 and Sanfield et al,'0 but none of the that coexisting lacunar infarction in the con- on October 2, 2021 by guest. Protected copyright. patients had HNKS. In our study, two of the tralateral basal ganglion might be the cause of three cases (patients 2 and 3) satisfied the cri- this type of hyperkinesia in patients with non- teria for HNKS. ketotic hyperglycaemia. Two of our three Patients with diabetes mellitus often ini- patients showed calcification in the basal gan- tially present with symptoms including glia and multiple infarcts on CT. The acute polyphagia, polydipsia, weight loss, gener- metabolic effect of non-ketotic hypergly- alised weakness, and fatigue.'2 In our study,