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Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia

Background: Hemi chorea and hemiballismus interestingly, are rare presentations of severe chronic persistent hyperglycemia which resolves with correction of hyperglycemia in most cases. Clinical Case: Here we report a case of a 67 YO woman with a PMH of prolonged uncontrolled DM evidenced by average...

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Autores principales: Karam, Jocelyne Georges, Eze-Raphael, Chinenye Uchenna, Calliste, Chrystel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8089970/
http://dx.doi.org/10.1210/jendso/bvab048.783
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author Karam, Jocelyne Georges
Eze-Raphael, Chinenye Uchenna
Calliste, Chrystel
author_facet Karam, Jocelyne Georges
Eze-Raphael, Chinenye Uchenna
Calliste, Chrystel
author_sort Karam, Jocelyne Georges
collection PubMed
description Background: Hemi chorea and hemiballismus interestingly, are rare presentations of severe chronic persistent hyperglycemia which resolves with correction of hyperglycemia in most cases. Clinical Case: Here we report a case of a 67 YO woman with a PMH of prolonged uncontrolled DM evidenced by average baseline HbA1c of 14.6% (n<5.7%) presenting with dance-like movements of the left arm and mouth deviation. Initially the patient presented as a stroke code. Vital signs were unremarkable. On Physical exam, there was mild L face weakness and obvious abnormal hand movement. Other neurological exams were unremarkable for stroke. MRI showed the typical decreased T2/flair signal within the right frontal lobe, white matter, right caudate head, and bilateral lenticular nucleus with mild increased T1 signal of the right caudate and lenticular nucleus compatible with hyperglycemic nonketotic hemichorea hemiballismus. Her significant admission lab values of random blood glucose of 306mg/dl (n 74-200mg/dl), HbA1c of 18.7% (n<5.7%), urine sugar >1000 (n neg mg/dl), absence of ketonuria and acidosis also support the diagnosis. With improvement in her blood sugar control, there was a significant decrease in her abnormal hand movements. However, sugar control was not enough as there was the need for additional medications to improve the chorea such as risperidone and tetrabenazine. Eventually over the course of many months of tight glucose control, there was significant symptomatic improvement. While the exact etiology is unclear, the hyperintensity usually seen on head CT scan and the pathognomonic T1 hyperintensity of the basal ganglia on MRI is postulated to be related to metabolic abnormalities caused by hyper viscosity of the blood in the end arteries supplying the basal ganglia. Many case reports associated movement disorders to chronic persistent hyperglycemia but there are many other cases that described hemiballismus in acute cases of hyperglycemia or persisting despite adequate glycemic correction. Most of the patients described had an underlying markedly elevated HbA1c as a common factor which suggests that a prolonged period of uncontrolled hyperglycemia may be necessary to produce hemiballismus. When thinking of complications of diabetes, we often think of CAD, ESRD, CVD, DKA and HHS. We aim to create an awareness of this rare association and to recognize hemiballismus as one of the varying presentations of severe hyperglycemia in order to ensure early diagnosis and treatment of this condition. Conclusion: Hemichorea and hemiballismus are increasingly becoming popular as one of the many complications of severe chronic or acute hyperglycemia. This warrants recognition in literature.
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spelling pubmed-80899702021-05-06 Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia Karam, Jocelyne Georges Eze-Raphael, Chinenye Uchenna Calliste, Chrystel J Endocr Soc Diabetes Mellitus and Glucose Metabolism Background: Hemi chorea and hemiballismus interestingly, are rare presentations of severe chronic persistent hyperglycemia which resolves with correction of hyperglycemia in most cases. Clinical Case: Here we report a case of a 67 YO woman with a PMH of prolonged uncontrolled DM evidenced by average baseline HbA1c of 14.6% (n<5.7%) presenting with dance-like movements of the left arm and mouth deviation. Initially the patient presented as a stroke code. Vital signs were unremarkable. On Physical exam, there was mild L face weakness and obvious abnormal hand movement. Other neurological exams were unremarkable for stroke. MRI showed the typical decreased T2/flair signal within the right frontal lobe, white matter, right caudate head, and bilateral lenticular nucleus with mild increased T1 signal of the right caudate and lenticular nucleus compatible with hyperglycemic nonketotic hemichorea hemiballismus. Her significant admission lab values of random blood glucose of 306mg/dl (n 74-200mg/dl), HbA1c of 18.7% (n<5.7%), urine sugar >1000 (n neg mg/dl), absence of ketonuria and acidosis also support the diagnosis. With improvement in her blood sugar control, there was a significant decrease in her abnormal hand movements. However, sugar control was not enough as there was the need for additional medications to improve the chorea such as risperidone and tetrabenazine. Eventually over the course of many months of tight glucose control, there was significant symptomatic improvement. While the exact etiology is unclear, the hyperintensity usually seen on head CT scan and the pathognomonic T1 hyperintensity of the basal ganglia on MRI is postulated to be related to metabolic abnormalities caused by hyper viscosity of the blood in the end arteries supplying the basal ganglia. Many case reports associated movement disorders to chronic persistent hyperglycemia but there are many other cases that described hemiballismus in acute cases of hyperglycemia or persisting despite adequate glycemic correction. Most of the patients described had an underlying markedly elevated HbA1c as a common factor which suggests that a prolonged period of uncontrolled hyperglycemia may be necessary to produce hemiballismus. When thinking of complications of diabetes, we often think of CAD, ESRD, CVD, DKA and HHS. We aim to create an awareness of this rare association and to recognize hemiballismus as one of the varying presentations of severe hyperglycemia in order to ensure early diagnosis and treatment of this condition. Conclusion: Hemichorea and hemiballismus are increasingly becoming popular as one of the many complications of severe chronic or acute hyperglycemia. This warrants recognition in literature. Oxford University Press 2021-05-03 /pmc/articles/PMC8089970/ http://dx.doi.org/10.1210/jendso/bvab048.783 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Diabetes Mellitus and Glucose Metabolism
Karam, Jocelyne Georges
Eze-Raphael, Chinenye Uchenna
Calliste, Chrystel
Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia
title Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia
title_full Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia
title_fullStr Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia
title_full_unstemmed Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia
title_short Hemi Chorea and Hemi Balllismus: A Rare Presentation of Prolonged Uncontrolled Hyperglycemia
title_sort hemi chorea and hemi balllismus: a rare presentation of prolonged uncontrolled hyperglycemia
topic Diabetes Mellitus and Glucose Metabolism
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8089970/
http://dx.doi.org/10.1210/jendso/bvab048.783
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