Chorea Hyperglycemia Basal Ganglia Syndrome—A Rare Case of Bilateral Chorea-Ballismus in Acute Non-Ketotic Hyperglycemia

Ilić, Aleksandra; Semnic, Marija; Stefanović, Dušan; Galic, Vladimir; Živanović, Željko

doi:10.4103/aian.aian_519_20

Cited by 3 publications

(5 citation statements)

References 12 publications

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“…Our patient had long-standing poor metabolic control, with an HbA1c of 7.8% and nonketotic hyperglycemia of 2.25 g/dL. Although the disease is usually unilateral, in less than 10% of cases, the basal ganglia are affected bilaterally [ 4 , 10 ], as was the case in our patient. We thoroughly investigated potential autoimmune, infectious, and toxic triggers, all of which were ruled out.…”

Section: Discussionmentioning

confidence: 57%

Bilateral Chorea-Ballism Associated With Non-ketotic Hyperglycemia: A Case Report

Abbassi,

Ali Kako,

Mebrouk

2024

Cureus

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The intricate workings of uncontrolled diabetes and its effects on the nervous system are not fully understood. However, it is known that this condition can lead to various neurological manifestations, including altered consciousness and epileptic seizures. In this case study, a 66-year-old woman presented with abnormal ballistic movements and chorea due to severe hyperglycemia. The results of her brain MRI suggested diabetic striatopathy. Fortunately, her symptoms improved significantly with the normalization of blood glucose levels and appropriate medical intervention. While the exact pathophysiology remains elusive, there is speculation that dysfunction of dopaminergic activity may play a role. The management of these complications involves stabilizing blood glucose levels and providing symptomatic relief. Despite the limited understanding of the underlying mechanisms, these neurological complications generally have a positive prognosis.

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Section: Discussionmentioning

confidence: 57%

Bilateral Chorea-Ballism Associated With Non-ketotic Hyperglycemia: A Case Report

Abbassi,

Ali Kako,

Mebrouk

2024

Cureus

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“…Typical findings in neuroimaging by brain magnetic resonance (MRI) include high-intensity T1-weighted and low-intensity T2-weighted lesions of the contralateral basal ganglia. Positron emission tomography (PET) with 2-deoxy-2-[fluorine-18] fluoro-D-glucose (18F-FDG) is also an option, showing a reduction in cerebral glucose metabolism in the basal ganglia on the affected side [ 3 - 5 ]. Our patient had a long history of poor metabolic control, with an HbA1c of 14% and non-ketonic hyperglycemia of 541 mg/dL.…”

Section: Discussionmentioning

confidence: 99%

“…Our patient had a long history of poor metabolic control, with an HbA1c of 14% and non-ketonic hyperglycemia of 541 mg/dL. Although the usual presentation entails unilateral involvement, less than 10% of these cases have bilateral basal ganglia affliction [ 5 ]. Autoimmune, infectious, and toxic causes were ruled out, and there was no family history of chorea-like symptoms.…”

Section: Discussionmentioning

confidence: 99%

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Hyperglycemic Hemichorea: A Case Report

Lopes¹,

Antunes²,

Oliveira³

et al. 2023

Cureus

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Chorea is a hyperkinetic movement disorder characterized by a continuous flow of rapid, random, and involuntary bodily contractions, usually affecting the distal limbs. When these movements are more proximal or assume a larger amplitude with a flinging or kicking character, they’re referred to as ballism. These disorders can be associated with several causes, ranging from genetic and neurovascular to toxic, autoimmune, and metabolic. Non-ketotic hyperglycemic hemichorea-hemiballismus is a rare consequence of decompensated diabetes mellitus with a poorly understood pathogenesis but with characteristic MRI T1 and T2 hyperintense abnormalities in the contralateral basal ganglia. We present the case of a 74-year-old woman with a history of poorly controlled type 2 diabetes mellitus, dyslipidemia, and arterial hypertension who was admitted to the emergency room due to a two-day history of rapid non-stereotypical involuntary movements of the left side of her body. A neurological exam showed large amplitude and repetitive left-side body movements. Glycemia was 541 mg/dL with no ketosis. Her glycosylated hemoglobin was 14%. A brain CT excluded acute abnormalities. Brain MRI showed a discrete T1 hyperintense signal involving the right corpus striatum, compatible with non-ketotic hyperglycemic hemichorea-hemiballism syndrome. After metabolic optimization with insulin and haloperidol, the movements resolved. Early recognition and metabolic control are essential to the resolution of choreiform movements. Our aim is to raise awareness for hyperglycemic hemichorea-hemiballismus, in which decompensated diabetes is the early sign of diagnosis.

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“…Cases reported in the literature with basal ganglia CT hyperdensities usually describe patients with severe ketotic or non-ketotic hyperglycaemic and contralateral hemiballismus and/or hemichorea 2–4. There are also other cases in which the patient presents atypically, with loss of consciousness 5.…”

Section: Descriptionmentioning

confidence: 99%