|Year : 2021 | Volume
| Issue : 1 | Page : 39-41
A reversible lentiform nucleus T1 hyperintensity in hypoglycemic chorea
Dinesh Chouksey, Pankaj Rathi, Nitisha Goyal, Ajoy K Sodani
Department of Neurology, SAMC and PGI, Indore, MP, India
|Date of Submission||02-Sep-2020|
|Date of Decision||23-Oct-2020|
|Date of Acceptance||18-Mar-2021|
|Date of Web Publication||17-Apr-2021|
Dr. Dinesh Chouksey
Department of Neurology, SAMC and PGI, A2, Flat 204, Maple Woods, Piplya Kumar, Nipania Main Road, Indore, MP.
Source of Support: None, Conflict of Interest: None
Acute to subacute onset chorea is commonly due to stroke, metabolic, post-infectious, and autoimmune disorders. Chorea in diabetes (DM) patient is mostly due to hyperglycemia, and few cases were reported due to hypoglycemia. The mechanism at the cellular level is still evolving. The MRI changes in diabetic chorea patients are variably reported. It is important to discuss our patient with hypoglycemic hemichorea because of unique features like low BMI, recently diagnosed DM on sulphonylurea with a history of hypoglycemic episodes, and acute left hemichorea that was controlled with neuroleptics, and her MRI brain showed reversible lentiform nucleus T1 hyperintensity without diffusion restriction and blooming on SWI.
Keywords: Acute hemichorea, hypoglycemic chorea, lentiform nucleus in chorea, MRI changes in chorea, T1 hyperintensity in basal ganglia
|How to cite this article:|
Chouksey D, Rathi P, Goyal N, Sodani AK. A reversible lentiform nucleus T1 hyperintensity in hypoglycemic chorea. Ann Mov Disord 2021;4:39-41
|How to cite this URL:|
Chouksey D, Rathi P, Goyal N, Sodani AK. A reversible lentiform nucleus T1 hyperintensity in hypoglycemic chorea. Ann Mov Disord [serial online] 2021 [cited 2021 Jun 15];4:39-41. Available from: https://www.aomd.in/text.asp?2021/4/1/39/313943
| Introduction|| |
Chorea is a hyperkinetic movement disorder of involuntary, continual, abrupt, rapid, brief, unsustained, irregular, semi purposeful movements that flow randomly from one body part to another. Chorea can be induced by structural, neurochemical, or metabolic disorders involving the basal ganglia. Hyperglycemia is a very well known cause of chorea, hemiballismus in a diabetic patient. Hypoglycemia as a reversible cause of chorea in a diabetic patient with a complete reversal of MRI brain imaging findings after the treatment is rarely described. Hypoglycemic patients may present with hemichorea- hemiballismus, seizures, ataxia, paresis, aphasia, and coma.
| Case|| |
A 60-year-old female, with a body mass index of 15.2 was diagnosed with diabetes mellitus recently. She was started on oral hypoglycemic drugs glimepiride 1mg and Metformin 500mg twice a day as a fixed-dose combination for 2 months. She occasionally used to experience hypoglycemic symptoms such as confusion, difficulty in finding words, sweating, and facial flushing, more frequently in early mornings which was precipitated by irregular diet and medications. She presented to us with left upper and lower limb involuntary, rapid, semi purposeful, non-stereotyped, flowing movements involving proximal as well as distal muscles. At admission, her neurological examination revealed normal power in both upper and lower limbs and her blood sugar was 52mg/dL. The laboratory blood parameters are mentioned in [Table 1].
A brain MRI was performed on admission which revealed asymmetrical right lentiform nucleus T1 hyperintense signal, isointense on T2 and FLAIR, without diffusion restriction and no blooming in SWI images suggesting absence of acute infarct and blood, respectively [Figure 1].
|Figure 1: (A) T1 hyperintensity in right lentiform nucleus, (B) T2 image, (C) Diffusion image|
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The patient was diagnosed to have unilateral hemichorea induced by hypoglycemia. She was started on IV Dextrose with hourly monitoring of blood sugar. She was also started on oral Haloperidol for symptomatic relief of chorea. During the period of her stay in the hospital, for 7 days, choreiform movements resolved significantly. Her oral antidiabetic drugs dosage was modified, and patients and family members were counseled about regular blood sugar monitoring at home. On follow up after 2 months, her MRI brain was repeated, which showed significant reduction of right lentiform T1 hyperintensity [Figure 2].
|Figure 2: (A) Disappeared T1 hyperintensity in right lentiform nucleus, (B) T2 image, (C) Diffusion image|
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| Discussion|| |
Repetitive episodes of hypoglycemia can lead to chorea and dyskinesias. These symptoms can be temporary or apermanent sequelae. Focal disorders can occur in the absence of an identifiable structural lesion. Of these focal disorders, transient neurological deficit simulating stroke is the most common. Nonketotic hyperosmolar coma may be associated with tonic spasms and paroxysmal choreoathetosis. The basal ganglia require high energy input for motor control and maybe selectively damaged by systemic processes that decrease cerebral metabolism. Because the neurons have a high metabolic rate and can neither generate nor store significant amounts of glucose, a rapid decline in plasma glucose can jeopardise neuronal homeostasis over minutes. Neuronal uptake of glucose is independent of insulin and mediated through glucose transporter 1(GLUT 1). When capillary endothelial cells in the brain are deprived of glucose, there is an associated increase in the transcription and translation of GLUT 1 protein and the energy metabolism could potentially be maintained in the presence of lower plasma glucose concentrations. Hypoglycemia results in the accumulation of oxaloacetate, which leads to the generation of excess aspartate. Aspartate is a known neurotoxin, and it has been shown to cause preferential neuronal necrosis in the cerebral cortex, neostriatum, and hippocampus.
The thalamus, brain stem, and cerebellum are invariably spared, and this may help to differentiate hypoglycemia from hypoxic injury, which often involves the thalamus. Schmidt et al. recently showed that short-term severe hypoglycemia by itself might not show any visible diffusion abnormalities. They observed neither hypoglycemia with a blood glucose level of <2.5 mmol/L for 20min nor severe hypoglycemia of <2.0 mmol/L for 10min induce visible changes in DWI of the human brain assuming that on a cellular level, the metabolic transpositions are still compensable. Our patient also did not have any diffusion abnormalities, and that suggests the absence of neuronal necrosis. T1 hyperintensity in basal ganglia is also seen in hepatocerebral degeneration, hyperglycemia, manganese toxicity, Wilson disease, abnormal calcium metabolism, neurofibromatosis, hypoxia, and hemorrhage. The T1 hyperintensity in hypoglycemic patients is described only in a few reports.,, The possible mechanism of T1 hyperintensity could be due to subacute blood (Petechial Haemorrhage) and deposition of calcium in the lentiform nucleus. Still, SWI images have to show blooming in both situation, so these two mechanisms are unlikely in our case. The substrate deficiency leading to neuronal loss and proliferation of astrocytic glial cells may be one of the factors in the pathogenesis as described by Wolz et al. and Fujioka et al. The other possible theory could be that vascular compromise may lead to subacute T1 hyperintensity, a study in 1999 by Fujioka et al. reported hyperintensity on T1-weighted images which appeared 7 days after brief ischemia with preservation of the macroscopic structure of brain.,
Single-photon emission computed tomography imaging in patients with chorea has shown reduced blood flow in basal ganglia and increased perfusion of thalamus contralateral to the side of chorea.
These findings have suggested a possible hypothesis that decreased pallidal inhibitory input to thalamus, resulting in increased thalamocortical outflow, might explain hyperkinesia.
| Conclusion|| |
Acute neurological deficits and hyperkinetic movement disorders like acute hemichorea are not always because of stroke or hyperglycemia and hypoglycemia has to be considered in these patients. Treatment with dopamine antagonist for a few months will control the symptoms, and there will be a reversal of T1 hyperintensity in MRI with time. Those patients who do not have diffusion restriction in MRI show better prognosis. The possible mechanism would be transient ischemia of basal ganglia at the cellular level with vanishing pallidal inhibition. This case gives insight for future research in the pathophysiological mechanism of hypoglycemic chorea and typical MRI changes.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Jagota P, Bhidayasiri R, Lang AE. Movement disorders in patients with diabetes mellitus. J Neurol Sci 2012;314:5-11.
Lai SL, Tseng YL, Hsu MC, Chen SS. Magnetic resonance imaging and single-photon emission computed tomography changes in hypoglycemia-induced chorea. Mov Disord 2004;19:475-8.
Meyer JS, Portnoy HD. Localized cerebral hypoglycemia simulating stroke; a clinical and experimental study. Neurology 1958;8:601-14.
Maccario M. Neurological dysfunction associated with nonketotic hyperglycemia. Arch Neurol 1968;19:525-34.
Juryńczyk M, Rozniecki J, Zaleski K, Selmaj K. Hypoglycemia as a trigger for of acute bilateral basal ganglia syndrome lesions in uremia. J Neurol Sci 2010;297:74-5.
Schmidt P, Böttcher J, Ragoschke-Schumm A, Mentzel HJ, Wolf G, Müller UA, et al
. Diffusion-weighted imaging of hyperacute cerebral hypoglycemia. AJNR Am J Neuroradiol 2011;32:1321-7.
Kang EG, Jeon SJ, Choi SS, Song CJ, Yu IK. Diffusion MR imaging of hypoglycemic encephalopathy. AJNR Am J Neuroradiol 2010;31:559-64.
Ma JH, Kim YJ, Yoo WJ, Ihn YK, Kim JY, Song HH, et al
. MR imaging of hypoglycemic encephalopathy: Lesion distribution and prognosis prediction by diffusion-weighted imaging. Neuroradiology 2009;51:641-9.
Lee D, Lee D, Ahn TB, Hong IK, Kim DY. Recurrent hemichorea after a hypoglycemic episode. Parkinsonism Relat Disord 2014;20:676-7. doi:10.1016/j.parkreldis.2014.03.006
Wolz M, Reichmann H, Reuner U, Storch A, Gerber J. Hypoglycemia-induced choreoathetosis associated with hyperintense basal ganglia lesions in T1-weighted brain MRI. Mov Disord 2010;25:966-8.
Paraskevas GP, Vlachos GS, Vassilopoulou S, Anagnostou E, Spengos K, Zis V. Hypoglycemia-induced hemichorea in a patient with fahr’s syndrome. Neurol Sci 2012;33:1397-9.
Shan DE. Hemichorea-hemiballism associated with hyperintense putamen on T1-weighted MR images: An update and a hypothesis. Acta Neurol Taiwan 2004;13:170-7.
Fujioka M, Okuchi K, Hiramatsu KI, Sakaki T, Sakaguchi S, Ishii Y. Specific changes in human brain after hypoglycemic injury. Stroke 1997;28:584-7.
Fujioka M, Taoka T, Hiramatsu KI, Sakaguchi S, Sakaki T. Delayed ischemic hyperintensity on T1-weighted MRI in the caudoputamen and cerebral cortex of humans after spectacular shrinking deficit. Stroke 1999;30:1038-42.
Fujioka M, Taoka T, Matsuo Y, Hiramatsu KI, Sakaki T. Novel brain ischemic change on MRI. Delayed ischemic hyperintensity on T1-weighted images and selective neuronal death in the caudoputamen of rats after brief focal ischemia. Stroke 1999;30:1043-6.
Kim JS, Lee KS, Lee KH, Kim YI, Kim BS, Chung YA, et al
. Evidence of thalamic disinhibition in patients with hemichorea: Semiquantitative analysis using SPECT. J Neurol Neurosurg Psychiatry 2002;72:329-33.
[Figure 1], [Figure 2]