Category: Choreas (Non-Huntington's Disease)
Objective: To demonstrate that metabolic derangement can lead to choreiform movement disorder.
Background: Hemichorea are involuntary, non-patterned movement disorders caused by lesions of the contralateral striatum. Hyperosmolar, non-ketotic hyperglycemia (NKH) consists of severe hyperglycemia with intracellular dehydration but without ketoacidosis and presents with typical lesions in the contralateral subthalamic nucleus and pallido-subthalamic pathways, with the spectrum of neurological deficit. Chorea hyperglycemia basal ganglia syndrome (C-H-BG) was initially described in 1960 and is defined by the sudden occurrence of hemichorea and in the more severe cases, hemiballismus.
Method: A 49 years old male patient with history of 10 years of diabetes on alternative therapy presented to hospital with hemichoreiform movement of left upper limb and parapresis in bilateral lower limbs with weight loss. Patient routine biochemical and hematological parameter revealed hypoglycemia which was managed accordingly. Routine hematological and biochemical investigations with with HbA1c of 16% with no ketones in urine routine and microscopy with serum osmolarity of 301 mosmol/l. MRI Brain screen which showed ill defined FLAIR hyperintensity with no diffusion restriction or magnetic susceptibility in the right basal ganglia and right anterior and medial temporal lobes suggestive of hyperglycemia related changes. Serum Lipid profile, Thyroid profile, Serum Electrophoresis were within normal limits. Lumbar puncture done in which CSF showed albuminocytological dissociation. Serum ceruloplasmin and serum copper are within normal limits. Serum MOG antibody, Serum Autoimmune encephalitis panel , Serum paraneoplastic panel, Serum NMO antibody was negative. On the basis of this diagnosis of diabetic lumbosacral radiculoplexus neuropathy (DRLPA) with chorea hyperglycemic basal ganglia syndrome was made and patient was managed conservatively on injection IVIg (2gm/kg body weight) for DRLPA, Injection with strict blood sugar control. Patient improved both in parapresis and movement disorder.
Results: Patient respond to strict hyperglycemic control.
Conclusion: Metabolic cause has been considered for hemichorea if presented with background of diabetes after ruling out other cause of hemichorea.
To cite this abstract in AMA style:S. Pandey, M. Varma, C. Gaba, S. Jain, A. Goswami. Chorea Hyperglycemic Basal Ganglia syndrome(CHBG) with Diabetic Lumbosacral Radiculoplexus Neuropathy – A rare case presentation [abstract]. Mov Disord. 2022; 37 (suppl 2). https://www.mdsabstracts.org/abstract/chorea-hyperglycemic-basal-ganglia-syndromechbg-with-diabetic-lumbosacral-radiculoplexus-neuropathy-a-rare-case-presentation/. Accessed September 23, 2023.
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