|Year : 2012 | Volume
| Issue : 2 | Page : 82-84
Hemichorea-hemiballismus in nonketotic hyperglycemia
Pawan Dhull1, Vimal Upreti2, K. V. S. Hari Kumar3, SK Patnaik4
1 Department of Neurology, Command Hospital (AF), Bangalore, Karnataka, India
2 Department of Endocrinology, Command Hospital (AF), Bangalore, Karnataka, India
3 Department of Endocrinology, Command Hospital (CC), Lucknow, Uttar Pradesh, India
4 Department of Paediatrics, Command Hospital (AF), Bangalore, Karnataka, India
|Date of Web Publication||21-Sep-2013|
Department of Neurology, Command Hospital (AF), Bangalore, Karnataka
Source of Support: None, Conflict of Interest: None
Hemichorea-hemiballismus (HC-HB) is a rare, but dramatic complication of nonketotic hyperglycemia specially in elderly Asian females. We report a case of HC-HB syndrome with emotional lability in a poorly-controlled diabetic female. MRI brain showed T1 and FLAIR hyperintensities in the right caudate and hippocampus.
Keywords: Caudate nucleus and hippocampus, hemichorea-hemiballismus, nonketotic hyperglycemia
|How to cite this article:|
Dhull P, Upreti V, Kumar KH, Patnaik S K. Hemichorea-hemiballismus in nonketotic hyperglycemia. J Acad Med Sci 2012;2:82-4
|How to cite this URL:|
Dhull P, Upreti V, Kumar KH, Patnaik S K. Hemichorea-hemiballismus in nonketotic hyperglycemia. J Acad Med Sci [serial online] 2012 [cited 2020 Jan 21];2:82-4. Available from: http://www.e-jams.org/text.asp?2012/2/2/82/118667
| Introduction|| |
Hemichorea-hemiballismus (HC-HB) is an unilateral involuntary movement disorder involving both proximal and distal groups of muscles.  Lesions of the contralateral subthalamic nucleus or its connections due to vascular lesions, tumors, multiple sclerosis, Huntingtons chorea, HIV infection, toxins, systemic lupus erythematosus, and thyrotoxicosis are common associations.  Uncommonly HC-HB occurs in diabetic patients with hyperosmolar hyperglycemia state (HHS) due to ischemic changes in the striatum associated with hyperglycemia and hyperviscosity with characteristic neuroradiological findings. ,, We report occurrence of HC-HB in a poorly-controlled female diabetic patient.
| Case Report|| |
A 54-year-old lady with type 2 diabetes mellitus on oral antidiabetic drugs, presented with acute onset abnormal involuntary writhing and twisting movements of left upper and lower limbs of three days duration. She was conscious, oriented, and normotensive. There was HC with occasional ballismic component and mild hypotonia in left upper and lower limbs [Video 1]. Movements were particularly noticeable during voluntary effort. There was associated emotional lability and impaired recall. No precipitating cause for HHS was evident on clinical examination. Investigations revealed poorly-controlled hyperglycemia (random blood glucose 548 mg/dl; HbA1c: 14.4%) and nonketoacidotic hyperosmolar state (calculated plasma osmolality 329.8 mOsm/L, blood pH 7.32, bicarbonate 19.8 mmol/l, negative urinary ketones, Serum sodium 144 mmol/l, serum potassium 5.2 mmol/l). Blood counts, liver, and renal functions, chest radiograph, ultrasonography of abdomen, blood and urine cultures, and ECG were normal. MRI of brain showed T1, T2, and FLAIR hyperintensity in right caudate and hippocampus with diffusion deficits on Diffusion weighted sequences [Figure 1], [Figure 2], [Figure 3], [Figure 4]. She was managed with insulin and clonazepam. Ballismus subsided within one week after she became euglycemic. At 3 months follow up, she has minimal HC with a better controlled glycemic state (Fasting/postprandial blood glucose: 118/176 mg/dl and HbA1c: 9.6%).
| Discussion|| |
HC-HB is common in older diabetic women of Asian descent like our patient. Nonketotic hyperglycemia remains an underecognized cause of HC-HB probably due to lack of awareness of this entity as well as reversibility in euglycemic state. , Neuroimaging findings in our case were similar to those earlier reported in HC-HB and HHS.  Hyperintensities on T1-weighted MRI and hyperdensities in noncontrast CT of the brain involve contralateral putamen and sometimes caudate nucleus and globus pallidus. ,, Hippocampal involvement in our case, a hitherto unreported finding in literature, explains the memory impairment.
Etiopathogenesis of HC-HB associated with HHS remains speculative. Hyperglycemia-induced regional cerebral blood flow impairment in the absence of ketoacidosis leads to attenuation of Kreb's cycle in brain and increases gamma-aminobutyric acid depletion via the succinic semialdehyde pathway.  Disinhibition of dopamine pathways in basal ganglia causes dopaminergic hyperactivity. Older women are predisposed due to postmenopausal estrogen deficiency causing dopamine hypersensitivity. , Petechial hemorrhages due to hyperglycemia and cerebral ischemia as well as diabetic microangiopathy also contribute.  Resolution occurs within few months after hyperglycemia correction.  Persistent movements warrant use of antipsychotics, benzodiazepines, anticonvulsants such as topiramate, and tetrabenazine. Our patient showed a satisfactory recovery with clonazepam. Awareness of HC-HB with HHS is important - it may be initial presentation of diabetes mellitus. In diabetics presenting with movement disorders, this reversible entity should be considered.
| References|| |
|1.||Dewey RB Jr, Jankovic J. Hemiballism-hemichorea. Clinical and pharmacologic findings in 21 patients. Arch Neurol 1989;46:862-7. |
|2.||McCullen MK, Miller J, Jabbour S, Furlong K, Shirodakar M, Ahmed I, et al. Chorea in the setting of hyperglycemia: A case report and review of the literature. Pract Neurol 2010;16-9. |
|3.||Chu K, Kang DW, Kim DE, Park SH, Roh JK. Diffusion- weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: A hyperviscosity syndrome? Arch Neurol 2002;59:448-52. |
|4.||Mestre TA, Ferreira JJ, Pimentel J. Putaminal petechial haemorrhage as the cause of non-ketotic hyperglycaemic chorea: A neuropathological case correlated with MRI findings. J Neurol Neurosurg Psychiatry 2007;78:549-50. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]