Hemiballism-hemichorea induced by ketotic hyperglycemia: case report with PET study and review of the literature

Abstract

Hemiballism-hemichorea (HB-HC) is commonly used to describe the basal ganglion dysfunction in non-ketotic hyperglycemic elderly patients. Here we report two elderly female patients with acute onset of involuntary movements induced by hyperglycemia with positive urine ketones. We described the computed tomography and magnetic resonance imaging findings in these two patients, which is similar to that of non-ketotic hyperglycemic HB-HC patients. FDG-PET was performed and the glucose metabolism in the corresponding lesion in these two patients was contradictory with each other. We tried to clarify the underlying mechanisms of HB-HC and explain the contradictory neuroradiological findings in FDG-PET as being performed at different clinical stages.

Hemichorea with unilateral MRI striatal hyperintensity in a Saudi patient with diabetes

“…Trials of lorazepam,10 chlorpromazine (12.5 mg/day), clonazepam (5 mg/day), reserpine (1.5 mg/day),8 and tetrabenazine (50 mg/day)2 lacked sufficient evidence of their effectiveness. Trihexyphenidyl (6 mg/day), olanzapine, perphenazine, and biperiden were of no benefit 7,9. Our experience supports the effectiveness of haloperidol, and the potential effectiveness of clonazepam while discouraging the use of lorazepam.…”

“…Some patients were newly diagnosed diabetics, and their HH was the first manifestation of the disorder 3,4. Others, as in our patient, had longstanding diabetes 3,5,8,9…”

“…Haloperidol (1-15 mg/day) frequently showed good efficacy in managing HH,2-4,9 but dose escalation in refractory patients can be limited by side effects, such as tardive dyskinesia or hemiparkinsonism 7. Moreover, risperidone (3 mg/day) may be effective 4.…”

Reversible striatal hypermetabolism in chorea associated with moyamoya disease: a report of two cases

“…Striatal hypermetabolism has been reported in various types of chorea, including chorea caused by group A streptococcal infection (Sydenham's chorea) [5,10,20], hyperthyroidism [3,9], and hyperglycemia [12,19,24]. As of this writing, however, no reports have surfaced in relation to moyamoya disease.…”

“…Although the prospect of a causal relationship between chorea and striatal hypermetabolism remains a subject of discussion [8,10], striatal hypermetabolism could represent activation of a direct pathwayan inhibitory signal pathway to the internal segment of the globus pallidus and substantia nigra, both of which negatively regulates thalamocortical projection [4]resulting in activation of the motor circuit. Several possible mechanisms have been suggested to explain striatal hypermetabolism observed in chorea: immunological reaction in Sydenham's chorea and hyperthyroidism [3,8,9,13,20], and the failure of vascular autoregulation in hyperglycemia [12,19,24].…”