Category: Choreas (Non-Huntington's Disease)
Objective: Localized structural damages could impair the threshold of activation and/or inhibition of the nigrostriatal pathway with a mirrored effect on the healthy and pathological basal ganglia side, respectively.
Background: Hemichorea syndrome secondary to non ketotic hyperglycemia also called “Diabetic striatopathy” (DS) is characterized by the presence of choreic unilateral involuntary movements and striatal hyperintensity on T1-weighted Magnetic Resonance Imaging (MRI)1.
Method: An 81-year-old male patient and a 71-year-old woman with type II diabetes mellitus in poor glycemic compensation were admitted to our department for the acute onset of left upper and lower limb choreic involuntary movements. On admission their Glycated Hemoglobin level was 76 and 105 mmol/mol, respectively. T1-weighted brain MRI showed area of altered signal in the right putaminal region in both patients2 [Figure 1]; DaT-scan showed a slight but non-significant reduction of the radiotracer uptake in the right putamen [Figure 2]. After 2 months from starting treatment with Haloperidol up to 2-3 mg/day3 they both complained of clumsiness with the right upper limb and a tendency to crawl the homolateral foot on the ground. On neurological examination a right-sided marked bradykinesia with moderate rigidity was noted. Therapy with Haloperidol had been gradually reduced until suspended, with marked improvement of the hypokinetic disorder.
Results: As it occurs in Parkinson Disease (PD), progressive degeneration of dopaminergic neurons alters the susceptibility of the basal ganglia pathway lowering the threshold for Levodopa-induced Dyskinesias (LIDs) 4. Degeneration is usually bilateral, but it progresses faster in the first involved basal ganglia side where LIDs occur earlier. We would expect that even in a context of a hyperkinetic disorder, DIP should involve first the most affected side due to an altered dopaminergic receptors sensitivity.
Conclusion: Therefore we’ve assumed that in these cases a structural damage of the striatal pathway may lead to a higher sensitivity threshold, making neuroleptic drugs rapidly efficacious against the hyperkinetic disorder, hiding any potential parkinsonian side effects. A mirrored condition in the healthy basal ganglia side explained the early appearance of iatrogenic parkinsonism, despite of low drug levels and a few weeks of treatment.
References: [1] Chua CB, Sun CK, Hsu CW, Tai YC, Liang CY, Tsai IT. “Diabetic striatopathy”: clinical presentations, controversy, pathogenesis, treatments, and outcomes. Sci Rep. 2020 Jan 31;10(1):1594. doi: 10.1038/s41598-020-58555-w.
[2] Cherian A, Thomas B, Baheti NN, Chemmanam T, Kesavadas C. Concepts and controversies in nonketotic hyperglycemia-induced hemichorea: further evidence from susceptibility-weighted MR imaging. J Magn Reson Imaging. 2009 Mar;29(3):699-703. doi: 10.1002/jmri.21672.
[3] Maia M, Moreira AP, Gonçalves AI, Espírito Santo J, Araújo J. Hemichorea-Hemiballism as a Manifestation of Hyperglycemia. Cureus. 2021 Nov 7;13(11): e19330. doi: 10.7759/cureus.19330. PMID: 34909293;
[4] Calabresi P, Di Filippo M, Ghiglieri V, Tambasco N, Picconi B. Levodopa-induced dyskinesias in patients with Parkinson’s disease: filling the bench-to-bedside gap. Lancet Neurol. 2010 Nov;9(11):1106-17. doi: 10.1016/S1474-4422(10)70218-0. Epub 2010 Sep 27. PMID: 20880751.
To cite this abstract in AMA style:
C. Santoro, S. Landolfo, V. Velucci, G. Iliceto. Hemichorea syndrome secondary to diabetic striatopathy with contralateral drug-induced parkinsonism: a different threshold for drug induced movement disorders? [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/hemichorea-syndrome-secondary-to-diabetic-striatopathy-with-contralateral-drug-induced-parkinsonism-a-different-threshold-for-drug-induced-movement-disorders/. Accessed December 10, 2024.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/hemichorea-syndrome-secondary-to-diabetic-striatopathy-with-contralateral-drug-induced-parkinsonism-a-different-threshold-for-drug-induced-movement-disorders/