Clinical features, neuroimaging findings and treatment outcomes in non ketotic hyperglycemic hemichorea-hemiballismus

Abstract

Introduction: Nonketotic hyperglycemic hemichorea-hemiballism (NKHH) is an uncommon hyperkinetic movement disorder. It is the second most common etiology after stroke affecting basal ganglion. Only a few hundred cases are reported so far in literature. Objectives: The study attempts to systematically highlight clinical profile and neuroimaging findings in 15 patients of Nonketotic hyperglycemic hemichorea-hemiballism. Materials and Methods: Patients with hemichorea/ hemiballism associated with hyperglycemia were included in the study. The period of the study was from January 2008 to September 2018. Neurological examination, Demographic data, clinical, biochemical, and neuroimaging findings were captured from the medical records. Details of the involuntary hyperkinetic movements such as type and side(s) involved were noted. Treatment was aimed to control glycemic status and neuroleptic drugs. Descriptive statistics were used wherever necessary. Results: During the study period, 52 patients had hemichorea/ hemiballismus.27 patients had stroke affecting basal ganglia.6 patients had granuloma affecting basal ganglia. 4 patients had Wilson’s disease.15 patients had NKHH. Among 15 patients of NKHH, 9 patients were females and 6 were males. (Female to male 1.5:1).The mean age at the onset was 70.2 years (Range 58 -85). The mean serum glucose levels measured after the onset of NKHH was 439.93 mg/dL (Range 342-532).The mean glycated haemoglobin was 10.98% (Range 9.1-18.5.Neuroimaging showed involvement of striatum in 13 patients. Conclusion: NKHH is a rare hyperkinetic movement disorder. Elderly poorly controlled diabetics and female sex are most commonly affected. Neuroimaging findings are distinct. Further studies are needed to elucidate pathogenesis of this disease and for determining prognosticating factors. Keywords: Non ketotic, hyperglycemia, Hemichorea-hemiballism, Putamen, Striatum.