Category: Choreas (Non-Huntington's Disease)
Objective: Late onset hemichorea in a diabetic patient could be either due to a vascular cause or Chorea Hyperglycemia Basal Ganglia Syndrome. The distinction between the two is possible by the clinical presentation and imaging characteristics.
Background: Vascular chorea is a rapid onset involuntary, purposeless non-stereotypical movement disorder that presents as hemichorea with the pathology in the contralateral cerebral hemisphere. Ischemic or hemorrhagic infarction will be demonstrated by the imaging commonly localizing to the thalamus, lentiform nucleus and less commonly sub thalamic nucleus. Most have vascular risk factors. Chorea Hyperglycemia Basal Ganglia Syndrome is rare and manifests in the background of uncontrolled non ketotic diabetes mellitus. The possible pathology of latter is attributed to the impaired central nervous system autoregulation leading to striatal dysfunction in the background of poorly controlled diabetes. Imaging localizes the pathology to the basal ganglia. Cases with CHBG syndrome have been reported even in normoglycemia as well.
Method: We followed a patient with acute onset of hemichorea in the background of diabetes for three months.
Results: A forty-six years old female patient with recent onset diabetes presented with acute onset right hemichorea which had been progressive over the first 24 hours. She didn’t have a diagnosis of connective tissue disease and there was no family history of any neurological disorder. On examination she had the involuntary movements predominantly involving right upper limb more than the lower limb without head and neck involvement. The limb power, reflexes and the sensory examination was normal. Chorea disappeared during sleep. Her serum blood sugar measurement remained within normal limits and the HbA1C was 6.5. Non contrast CT brain revealed faint hypodensity in left posterolateral thalamus whereas MRI brain revealed hyperintensity in the same territory with diffusion restriction in DWI in the territory of posterior choroidal artery.Her carotid duplex revealed atheromatous disease involving bilateral internal carotid arteries. Her symptoms got benefitted by low dose clonazepam in addition to usual stroke care and completely resolved at the end of three months.
Conclusion: Chorea is a rare complication of stroke. It manifests with hemichorea and have a benign disease course. Distinction between CHBG syndrome is mandatory for subsequent management.
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Carrion DM, Carrion AF. Non-ketotic hyperglycaemia hemichorea-hemiballismus and acute ischaemic stroke. BMJ Case Rep. 2013 Mar 6;pii: bcr2012008359. | CrossRef | PubMed |
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To cite this abstract in AMA style:N. Premarathna, A. Fernando, S. Bandusena. Late Onset Hemichorea Of Vascular Origin In A Middle Aged Female With Recent Onset Diabetes [abstract]. Mov Disord. 2022; 37 (suppl 2). https://www.mdsabstracts.org/abstract/late-onset-hemichorea-of-vascular-origin-in-a-middle-aged-female-with-recent-onset-diabetes/. Accessed March 4, 2024.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/late-onset-hemichorea-of-vascular-origin-in-a-middle-aged-female-with-recent-onset-diabetes/