Published on February 19, 2014
Movement disorders: A complication of chronic Hyperglycemia? A case report
a p o l l o m e d i c i n e x x x ( 2 0 1 4 ) 1 e3 Available online at www.sciencedirect.com ScienceDirect journal homepage: www.elsevier.com/locate/apme Case Report Movement disorders: A complication of chronic hyperglycemia? A case report R. Ben Othman a,*, A. Ben Mahmoud b, A. Mankai a, N. Trabelsi a, F. Hentati b, F. Ben Mami a a Department (C) of Diabetology, Nutrition and Metabolic Diseases, The National Institute of Nutrition, Avenue Jbel Lakhdhar, 1007 Tunis, Tunisia b Department of Neurology, National Institute Mongi Ben Hmida of Neurology, Tunis, Tunisia article info abstract Article history: Introduction: The association of chorea with a speciﬁc lesion on brain imaging is described Received 29 March 2013 as an atypical manifestation of chronic hyperglycemia. Accepted 9 October 2013 Case report: Patient of 77 years, hospitalized for bilateral choreic movements predominant Available online xxx on the right with dystonic movements, appeared a month ago. At admission: Hyperglycemia at 2.4 g/L, without ketosis, a type 2 diabetes (HbA1c 17.3%) on Keywords: oral antidiabetic agents. CT scan shows hyperdensity of putamen and lenticular nucleus Diabetes mellitus that eliminates an ischemic attack. Evolution after glycemic reequilibration and treatment Chorea by haloperidol is favorable, with regression of chorea. Dyskinesia Discussion: This is a rare syndrome, affecting more elderly, presenting a diabetes poorly Hyperglycemia controlled. There is hyperglycemia without ketosis and moderate hyperosmolarity. The Nonketotic hyperglycemia symptoms usually regress after glycemic control and neuroleptic treatment (recurrence in 13% of cases). The appearance of this syndrome seems to be related to the achievement of the control system GABAergic of the basal ganglia leading to an excitation of the cerebral cortex, causing involuntary movements. The etiopathogenic hypotheses advanced are multiple: impaired striatal neurotransmission associated with metabolic disturbances of hyperglycemia, dysfunction of the hematoencephalic barrier and abnormalities of the cerebral vascularization. Conclusion: Chorea secondary to nonketotic hyperglycemia is a rare complication and little known of type 2 diabetes. Transitional neuroleptic treatment and glycemic control make the symptoms regress in most cases with the disappearance of the lesion imaging. Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved. 1. Introduction The uncontrolled diabetes can be responsible for different neurological symptoms such as epileptic seizures and disorders of consciousness. Transient chorea or ballism provoked by an episode of nonketotic hyperglycemia has been reported by numerous authors over the past couple of decades.1,2 In this case, the choreiform movements have resolved within days after normalization of blood glucose. In the vast majority of cases, the chorea/ballism triggered by hyperglycemia has * Corresponding author. Tel.: þ216 97291139. E-mail address: email@example.com (R. Ben Othman). 0976-0016/$ e see front matter Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved. http://dx.doi.org/10.1016/j.apme.2013.10.006 Please cite this article in press as: Ben Othman R, et al., Movement disorders: A complication of chronic hyperglycemia? A case report, Apollo Medicine (2014), http://dx.doi.org/10.1016/j.apme.2013.10.006
2 a p o l l o m e d i c i n e x x x ( 2 0 1 4 ) 1 e3 been unilateral. Several recent reports have documented characteristic brain imaging ﬁndings consisting of high density on computed tomography (CT) scans. We describe a case of chorea developing subacutely during an episode of reversible nonketotic hyperglycemia. 2. Observation A patient, aged 77 years, followed for type II diabetes on oral antidiabetic agents for three months and for hypertension since two years, presents to the emergency for abnormal movements of the upper limbs, the head and neck. These movements are type of ﬂexion-extension and rotation. They are abrupt, irregular and of large amplitude, exaggerating to emotion and disappearing during sleep. There is no remarkable family history or a prior history of dopamine antagonist or estrogen medication use and no history of rheumatic fever/Sydenham’s chorea. At admission, the patient was conscious, afebrile and well oriented in time and space. Neurological examination objectify chorea in both upper limbs, the head and neck predominant at right without oral or lingual abnormal movements. In addition, the review found hypotonia in all four limbs. The remainder of the examination shows no motor or sensory deﬁcit, nor parkinsonism nor cerebellar nor cranial disorder. A specialized neuropsychological examination did not show signiﬁcant cognitive disorders (mini-mental state ¼ 28/30) except an alteration of executive functions (frontal score ¼ 8/18). In addition, a psychomotor instability was noted as well as irritability. A cerebral CT scan was performed in emergency, which objectiﬁed a spontaneous hyperdensity of putamen and lenticular nuclei (Fig. 1a et b). Our patient did not beneﬁt of brain MRI. Urinalysis was strongly positive for glucose and negative for ketones. The following laboratory data were notable: fasting blood glucose 13.2 mmol/l (240 mg/dl), glycosylated hemoglobin A1c 17.3%, creatinine 140 mmol/l, clearance 37 ml/min. The patient was put on insulin and small doses of haloperidol (3 mg/day in three divided doses). A signiﬁcant reduction in abnormal movements was noted, these disappeared within four days, which motivated progressive stopping of haloperidol .The patient was admitted in diabetology for initiation of insulin 6UI at morning due to contraindication for the use of oral antidiabetic .The patient is still asymptomatic after six months. Control brain CT scan was not performed. 3. Discussions Chorea and ballism are hyperkinetic movement disorders which are characterized by the occurrence of uncontrollable, sudden, irregular, high amplitude and short duration movements of all or a part of the body.3,4 Chorea and/or ballism have been reported for the ﬁrst time, as a complication of nonketotic hyperglycemia by Bedwell in 1960.5 These abnormal movements are rarely revealing of diabetes.2 The symptomatology is made from hemichorea and/or hemiballismus in most cases correlated to unilateral scannographic images.6 For our case the damage is bilateral predominantly to the right in relation to imagery and in accordance with some similar cases reported in the literature where the lesion is bilateral.7 Physiopathology subtending the development of these movements during hyperglycemia without ketosis is still unclear.3 Several hypotheses have been evoked. Brain structures implicated in the genesis of chorea are represented by the basal ganglia, mainly the subthalamic nucleus, the way pallido-subthalamic, striatum and to a lesser degree the cerebral cortex and the thalamus.8 These cores are susceptible to ATP and cellular energy depletion which probably explain the predilection of lesions at their level.8 Indeed, during hyperglycemia, there is an alteration of the bloodebrain barrier (BBB) leading to, on one hand, the reduction of regional cerebral blood ﬂow and on the other hand, the passage of the cerebral metabolism to anaerobic, resulting in an inhibition of the tricarboxylic acid cycle (Krebs).9,10 Fig. 1 e (a and b) A CT scan showing a spontaneous hyperdensity in the putamen and the lenticular nucleus. Please cite this article in press as: Ben Othman R, et al., Movement disorders: A complication of chronic hyperglycemia? A case report, Apollo Medicine (2014), http://dx.doi.org/10.1016/j.apme.2013.10.006
a p o l l o m e d i c i n e x x x ( 2 0 1 4 ) 1 e3 The brain then uses the gamma-aminobutyric acid (GABA) as an alternative source of energy. In patients without ketosis,9e12 the amount of GABA and acetate is rapidly depleted.11,13 Because of this deﬁciency of acetate, acetylcholine synthesis may also be decreased. The depletion of these two principal neurotransmitters (GABA and acetylcholine) in the basal ganglia, in combination with loss of energy and metabolic acidosis is the cause of the appearance of movement disorders.11,13,14 Hyperglycemia has also a role in the extravasation of red blood cells that is the cause of hemorrhagic suffusions found in cerebral imaging of the majority of these patients.8 Other indirect factors have been incriminated in the genesis of chorea, in fact most cases of chorea during nonketotic hyperglycemia have been reported in elderly patients with hypertension associated with diabetes.14,15 Indeed, these two vascular risk factors are often responsible for cerebral vascular lesions especially lacunar mainly at the basal ganglia.16 These lesions are usually not visible on standard neuroimagery, but responsible for clinical manifestations such as chorea.14,17 Finally, the acanthocytosis as a predictor of the onset of chorea during diabetes has been reported.12 Several hypotheses have suggested that erythrocyte membrane proteins can undergo, secondary to a particular metabolic state (diabetes), functional and morphological changes, with acanthocytes formation.18 So hyperviscosity induced by hyperglycemia may cause a transient malfunction in striatal neurons in predisposed individuals. This hypothesis is in agreement with the concept that hyperglycemia worsens the evolution of stroke. For our patient, the search for acanthocytes came back negative. Chorea in the context of hyperglycemia without ketosis is generally good prognosis. Most patients improve when normalization of blood glucose, sometimes without the use of neuroleptics or with very low doses.10,14 Radiological abnormalities may persist longer and disappear for days or weeks after normalization of glycemia.12 Neuroleptics is the treatment of choice. Benzodiazepines can also be used. 4. Conclusion Chorea during uncontrolled diabetes is an unusual complication, however it remains favorable prognosis in most cases, which makes necessary the knowledge of this association since therapeutic implications arising. Conﬂicts of interest All authors have none to declare. 3 references 1. Chang CV, Felicio AC, Godeiro-Junior CO, et al. Choreaballism as a manifestation of decompensated type 2 diabetes mellitus. Am J Med Sci. 2007;333:175e177. 2. Lai PH, Tien RD, Chang MH, et al. Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus. Am J Neuroradiol. 1996;17:1057e1064. 3. Mihai CM, Catrinoiu D, Stoicescu RM. Atypical onset of diabetes in a teenage girl: a case report. Cases J. 2008;1:425. 4. Vingerhoets F, Russmann H, Carruzzo A, et al. Mouvements ´ ´ anormaux (dystonie, athetose, choree, ballisme). EMC. 2004, 17:007-B-10. 5. Bedwell SF. Some observations on hemiballismus. Neurology. 1960;10:619e622. ´ ´ ´ 6. Rafai MA, Gynerane M, et al. Hemichoree-hemiballisme et ´ ´ hyperglycemie non cetonique. NPG. 2010;10:229e232. 7. Nakagawa T, Mitani K, et al. Chorea-ballism associated with nonketotic hyperglycemia and presenting with bilateral hyperintensity of the putamen on MR T1-weighted imagesea case report. Rinsho Shinkeigaku. 1994;34(1):52e55. 8. Felicio AC, Chang CV, Godeiro-Junior C, et al. Hemichoreahemiballism as the ﬁrst presentation of type 2 diabetes mellitus. Arq Neuropsiquiatr. 2008;66(2-A):249e250. 9. Low PA, Ward K, Schmelzer JD, et al. Ischemic conduction failure and energy metabolism in experimental diabetic neuropathy. Am J Physiol. 1985;248:E457eE462. 10. Oerlemans WGH, Moll LC. Non ketotic hyperglycemia in a young woman presenting as hemiballism-hemichorea. Acta Neurol Scand. 1999;100:411e414. 11. Guisado R, Arieff AI. Neurological manifestations of diabetic comas: correlation with biochemical alterations in the brain. Metabolism. 1975;24:665e679. 12. Pisani A, Diomedi M, Rum A. Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism. J Neurol Neurosurg Psychiatry. 2005;76:1717e1719. 13. Rector WG Jr., Herlong HF, Moses H 3rd. Nonketotic hyperglycemia appearing as choreoathetosis or ballism. Arch Intern Med. 1982;142:154e155. 14. Lin JJ, Chang MK. Hemiballism-hemichorea and non ketotic hyperglycemia. J Neurol Neurosurg Psychiatry. 1994;57:748e750. 15. Nagai C, Kato T, Katagiri T, et al. Hyperintense putamen on T1-weighted images in a case of chorea with hyperglycemia. Am J Neuroradiol. 1995;16:1243e1246. 16. Weisberg LA. Diagnostic classiﬁcation of stroke, especially lacunes. Stroke. 1988;19:1071e1073. 17. Cherian A, Thomas B, Baheti NN, et al. Concepts and controversies in nonketotic hyperglycemia-induced hemichorea: further evidence from susceptibility-weighted MR imaging. J Magn Reson Imaging. 2009;29(3):699e703. 18. Rampoldi L, Danek A, Monaco AP. Clinical features and molecular bases of neuroacanthocytosis. J Mol Med. 2002;80:475e491. Please cite this article in press as: Ben Othman R, et al., Movement disorders: A complication of chronic hyperglycemia? A case report, Apollo Medicine (2014), http://dx.doi.org/10.1016/j.apme.2013.10.006
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Case Report Movement disorders: A complication of chronic hyperglycemia? A case report R. Ben Othmana,*, A. Ben Mahmoudb, A. Mankaia, N. Trabelsia,
The association of chorea with a specific lesion on brain imaging is described as an atypical manifestation of chronic hyperglycemia.Patient of 77 years ...
Abstract Introduction. The association of chorea with a specific lesion on brain imaging is described as an atypical manifestation of chronic hyperglycemia ...
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