Non-ketotic Hyperglycemia Chorea-Ballismus and Intracerebral Hemorrhage: A Case Report and Literature Review

Abstract

Non-ketotic hyperglycemia chorea-ballismus (NKH-CB) is a rare metabolical syndrome secondary to the hyperglycemic condition, which is characterized by a triad of acute or subacute hemichorea-hemiballismus, hyperglycemic state, and unique abnormalities limited to the striatum on neuroimaging. Several related case studies on this disorder have been reported previously, but NKH-CB had never been associated with intracerebral hemorrhage (ICH). Herein, we report an uncommon case of NKH-CB and ICH that occurred simultaneously in one patient, which provides a challenge for clinicians in making a correct diagnosis. An 88-year-old woman with a long-term history of poor-controlled type 2 diabetes mellitus and hypertension, who presented with a sudden-onset headache, restlessness, severe bilateral choreiform and ballistic movements, elevated levels of glucose and osmolality in the serum, an increased white blood cell count, and two-type hyperdense signs on CT imaging, was finally diagnosed with NKH-CB and ICH. Despite administrated active treatments, the patient's clinical status did not improve and ultimately passed away. This case is reported to remind clinicians to consider the possibility of NKH-CB when patients present sudden-onset choreiform and ballistic movements. It is also the first entity with two-type hyperdense signs on CT imaging simultaneously, which helps us distinguish NKH-CB from ICH more intuitively.

Diabetic Striatopathy Complicated With Acute Ischemic Stroke: A Case Report

“…Diabetic striatopathy (DS) is a rare complication of diabetes mellitus (DM), characterized by a prominently increased blood glucose level, unilateral striatal hyper-density on CT, and hyperintensity on T1-weighted MRI, as well as contralateral choreiform movement (Dong and Zhang, 2021 ). Bilateral striatal lesions were rarely (9.7%) seen in patients with DS, which help distinguish DS from metabolic disorders, infectious, drugs, or toxics-induced striatal lesions that prominently affect the bilateral striatum (Chua et al, 2020 ; Dong and Zhang, 2021 ). DS prominently occurs in Asian women, particularly these patients with newly diagnosed or poorly controlled diabetes.…”

“…A few patients developed persistent chorea even under a backdrop of well-controlled diabetes mellitus or complete resolution of abnormal striatum signals, suggesting the presence of irreversible damage (Ahlskog et al, 2001 ; Wu et al, 2014 ; Roy et al, 2016 ; Lucassen et al, 2017 ; Chatterjee et al, 2022 ). Severe or uncontrolled hyperglycemia is associated with chorea relapse and the attacks of acute cerebrovascular events, such as acute cerebral infarction and hemorrhage, leading to a poor prognosis (Carrion and Carrion, 2013 ; Lucassen et al, 2017 ; Chua et al, 2020 ; Dong and Zhang, 2021 ). Carrion et.…”

Hemichorea-Hemiballismus Syndrome in Acute Non-ketotic Hyperglycemia

“…Non-ketotic hyperglycemia hemichorea/hemiballismus is a rare entity that occurs in the context of diabetes or acute hyperglycemia [ 7 ]. It is relatively more common in older female patients with type 2 diabetes [ 2 ].…”

Non-ketotic Hyperglycemic Hemichorea-Hemiballismus: A Case of a Male With Diabetes Mellitus and Speech Disturbances

“…Non-ketotic hyperglycemic chorea-ballismus, also known as chorea hyperglycemia basal ganglia syndrome, is a rare manifestation of poorly controlled diabetes mellitus most commonly reported in women of eastern Asian descent with a mean age of 71 years [1]. It is characterized by a triad of acute or subacute choreaballismus, non-ketotic hyperglycemic state, and hyperintense striatopathy on magnetic resonance imaging (MRI) or computerized tomography scan (CT), most commonly the putamen [2]. Hemichorea and hemiballismus are hyperkinetic movements that affect the contralateral side to striatal hyperintensity on neuroimaging.…”

“…The third hypothesized mechanism is that the non-ketotic state decreases the availability of acetoacetate for conversion to gamma-aminobutyric acid (GABA) and thus limits the amount of GABA (the chief inhibitory neurotransmitter in the central nervous system) in the thalami/striatum [4]. Non-ketotic hyperglycemic chorea-ballismus is reversible by treatment with optimal glycemic control [2][3][4][5][6]. Most patients recover within days to months.…”