Abstract:
We report a rare case of left-sided choreoathetosis in a 60-year-old renal transplant recipient associated with a recent hyperglycemic episode and multiple chronic lacunar infarcts involving the basal ganglia. This case highlights a complex interplay between metabolic dysregulation, vascular compromise and immunosuppression, presenting as a hyperglycemia-related movement disorder in a post-transplant setting.
Introduction:
Chorea-hyperglycemia-basal ganglia (CHBG) syndrome is an uncommon complication of poorly controlled diabetes mellitus, predominantly affecting elderly patients, especially females of Asian origin. It typically manifests with unilateral choreiform movements (involuntary, dance-like movements), correlating with T1 hyperintensities in the contralateral basal ganglia on Brain MRI. This condition is even rarer in immunocompromised individuals, such as renal transplant recipients, where the overlap with ischemic and metabolic encephalopathies complicates diagnosis and management.
Case Presentation:
A 60-year-old male, with a renal transplant (Madurai, 2011), Coronary Artery Disease (post-PTCA, 2013), Pulmonary Tuberculosis (2017/2020), and left total
knee replacement, presented with involuntary, dance-like movements involving the left upper and lower limbs. He had a history of raising creatinine levels and
chronic kidney graft dysfunction was confirmed by a recent biopsy showing chronic active cell-mediated rejection (Banff 1A, Dec 2024).
The patient experienced a severe hyperglycemic episode (CBG 1000 mg/dL) in February 2025(treatment done elsewhere). First episode of hyperglycemia ever and
diagnosis of Diabetes Mellitus. While he was treated for extreme hyperglycemia, severe choreiform movements of head, neck, left upper and lower limbs were
observed. There was orofacial dyskinesia too. There was one hypoglycemic episode (CBG 30 mg/dL) after an insulin overdose during initial treatment. After
initial management at Madurai, patient presented to our hospital with ongoing choreo athetoid movements preventing him from resting in bed, chair and even
eating or drinking
Though his sugars were under control for the past 2 weeks there was no improvement in the movement disorder. There is no family history of movement disorder and no history of any offending drug history. MRI brain done at Kauvery Hospital , Alwarpet revealed chronic ischemic infarcts in bilateral basal ganglia
(BG), caudate nuclei, thalami, and left occipital lobe.
Based on clinical features and imaging, a diagnosis of chorea-hyperglycemia-basal ganglia syndrome with underlying chronic ischemia was considered.
He was initiated on Procyclidine ,Tetrabenazine and Clonazepam following which his involuntary movements gradually reduced. His sugar levels were closely
monitored and maintained at optimal levels.
Discussion:
CHBG syndrome, first described by Oh et al. (2002), is strongly associated with non-ketotic hyperglycemia. Imaging typically shows hyperdensity on CT or T1
hyperintensity in the contralateral BG. This presentation has been linked to metabolic dysfunction of GABAergic and dopaminergic systems, microvascular
ischemia, and astrocytic dysfunction.
Commonly used medicines are Procyclidine, Tetrabenazine and Clonazepam.
Procyclidine – The mechanism of action is unknown. It is thought that Procyclidine acts by blocking central cholinergic receptors, and thus balancing cholinergic and dopaminergic activity in the basal ganglia.
Tetrabenazine – Its anti-chorea effect is believed to be due to a reversible depletion of monoamines such as dopamine, serotonin, norepinephrine, and histamine from nerve terminals.
Clonazepam enhances the activity of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA) in the central nervous system
In a meta-analysis of 53 cases (Oh et al., 2002), 85% were elderly females, with unilateral chorea and BG changes being the hallmark. Similarly, Mittal et al.
(2012) and Dwarak et al. (2019) reported symptom resolution with glycemic control, emphasizing the importance of early metabolic correction.
Our patient’s case is unique due to:
- Post-transplant status and chronic rejection
- Coexisting chronic ischemic changes on MRI
- Acute fluctuations in glucose (hyper- and hypoglycemia)
- Male gender and atypical demographic for CHBG
The combination of ischemic injury and metabolic insult likely exacerbated basal ganglia vulnerability, resulting in persistent choreiform movements despite glucose normalization.
Conclusion:
This case underscores the necessity of a high index of suspicion for CHBG in transplant recipients with new-onset movement disorders, especially when
associated with acute glycemic derangements. Brain imaging and early multidisciplinary intervention are key to diagnosis and management.
References:
- Oh SH, Lee KY, Im JH, Lee MS. Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI
study: A meta-analysis of 53 cases. J Neurol Sci. 2002;200(1-2):57-62. PMID: 12127677 - Mittal P, et al. Dyskinesia Associated with Hyperglycemia and Basal Ganglia Hyperintensity: Report of a Rare Diabetic Complication. J Clin Diagn Res.
2012;6(8):1424-1425. PMID: 23154926 - Dwarak S, et al. Chorea, Hyperglycemia, Basal Ganglia Syndrome in Diabetes – Two Rare Case Reports. Maedica (Bucur). 2019;14(3):271-274. PMID: 31123515
- Choi S, Rhee SY, Kim HS. CHBG Syndrome in a Young Patient with Type 1 Diabetes Mellitus: a Case Report. Brain & Neurorehabil. 2019. PMID: 36744185
Dr. Yashica Priya R.,MD
Internal Medicine
Kauvery Hospital, Chennai
Dr. Sivarajan Thandeswaran, MBBS, MRCP (UK)
Senior Consultant
Kauvery Hospital, Chennai
Dr. Subhasri, MBBS,
Kauvery Hospital, Chennai