Abstract:Non-ketotic hyperglycaemia (NKH) is the most common metabolic cause of hemichorea-hemiballismus (HC-HB) and an often-reversible condition. A 68-year-old man presented to the emergency department with a severe hyperglycaemic episode and altered mental status. He was treated appropriately and discharged home after his blood glucose levels were normal with an improvement of mental status. Four weeks after the discharge, he returned with flailing movements of bilateral upper and lower limbs. MRI of the brain revea… Show more
“…Many variations of this presentation have been described, including bilateral MRI changes [ 15 16 17 18 19 20 21 22 23 24 ], ipsilateral MRI changes [ 25 ], bilateral chorea [ 23 26 ], recurrent chorea [ 27 28 ] and persistent chorea [ 25 29 30 ]. Cases have been described in the pediatric population [ 31 32 33 34 35 36 ] as well as in both children and adults presenting with ketotic hyperglycemia [ 22 37 38 39 ].…”
Background:
Chorea can be due to a large number of etiologies. Unilateral chorea is classically related to a contralateral structural lesion, e.g. of the putamen or subthalamic nucleus, however, based upon personal impressions, we have observed that systemic disease, in particular metabolic or autoimmune conditions, can also lead to a unilateral or markedly asymmetric presentations. We sought to investigate this impression by reviewing the literature.
Methods:
A PubMed search was conducted using the terms asymmetric” AND “chorea” OR “hemichorea” OR “unilateral” AND “chorea” OR “monochorea” OR “right greater than left” AND “chorea” OR “left greater than right” AND “chorea” OR “right more than left” AND “chorea” OR “left more than right” AND “chorea” as well as “hemiballismus” NOT “stroke” NOT “infarct” NOT “dyskinesia. A total of 243 sources were felt to meet criteria and were reviewed.
Results:
The most common etiology of reported hemi- or asymmetric chorea was diabetic non-ketotic hyperglycemic hemichorea/hemiballismus. Other common diagnoses were Sydenham’s disease, antiphospholipid syndrome and drug-induced chorea. The vast majority of patients with hemi- or asymmetric chorea had acquired rather than genetic, degenerative or congenital causes.
Conclusion:
Despite the potential limitations of our literature review, the evidence presented here supports the observation that the vast majority of asymmetric or unilateral chorea presentations are due to acquired causes, and in this situation an exhaustive search for reversible etiology should be undertaken. However, presentation with symmetric, generalized chorea does not exclude reversible causes, and investigations should address these in addition to genetic and neurodegenerative etiologies.
“…Many variations of this presentation have been described, including bilateral MRI changes [ 15 16 17 18 19 20 21 22 23 24 ], ipsilateral MRI changes [ 25 ], bilateral chorea [ 23 26 ], recurrent chorea [ 27 28 ] and persistent chorea [ 25 29 30 ]. Cases have been described in the pediatric population [ 31 32 33 34 35 36 ] as well as in both children and adults presenting with ketotic hyperglycemia [ 22 37 38 39 ].…”
Background:
Chorea can be due to a large number of etiologies. Unilateral chorea is classically related to a contralateral structural lesion, e.g. of the putamen or subthalamic nucleus, however, based upon personal impressions, we have observed that systemic disease, in particular metabolic or autoimmune conditions, can also lead to a unilateral or markedly asymmetric presentations. We sought to investigate this impression by reviewing the literature.
Methods:
A PubMed search was conducted using the terms asymmetric” AND “chorea” OR “hemichorea” OR “unilateral” AND “chorea” OR “monochorea” OR “right greater than left” AND “chorea” OR “left greater than right” AND “chorea” OR “right more than left” AND “chorea” OR “left more than right” AND “chorea” as well as “hemiballismus” NOT “stroke” NOT “infarct” NOT “dyskinesia. A total of 243 sources were felt to meet criteria and were reviewed.
Results:
The most common etiology of reported hemi- or asymmetric chorea was diabetic non-ketotic hyperglycemic hemichorea/hemiballismus. Other common diagnoses were Sydenham’s disease, antiphospholipid syndrome and drug-induced chorea. The vast majority of patients with hemi- or asymmetric chorea had acquired rather than genetic, degenerative or congenital causes.
Conclusion:
Despite the potential limitations of our literature review, the evidence presented here supports the observation that the vast majority of asymmetric or unilateral chorea presentations are due to acquired causes, and in this situation an exhaustive search for reversible etiology should be undertaken. However, presentation with symmetric, generalized chorea does not exclude reversible causes, and investigations should address these in addition to genetic and neurodegenerative etiologies.
“…She had increased T1 signal in her left putamen and HbA 1c of 8.9% (74mmol/mol) 18 . The other cases had recent significant hyperglycaemia with blood glucose of 64.4mmol/L (case A) 19 and 54.6mmol/L (case B) 20 treated with intravenous insulin followed by subcutaneous switch on discharge. The cases presented with hemiballismus at four and 12 weeks post‐discharge, respectively, in the setting of euglycaemia.…”
Section: Discussionmentioning
confidence: 99%
“…There are only three other case reports of diabetes‐related hemiballismus in the setting of euglycaemia. They all describe improved glycaemic control at presentation in the setting of recent suboptimal glycaemia 18–20 . One case had a previous stroke (which is itself a risk factor for hemiballismus) and only mild hyperglycaemia of 11–14mmol/L.…”
“…Besides an abnormal metabolic panel, it is associated with noticeable changes in neuroimaging. CT and MRI scans of several affected patients show hyperdensities and hyperintense signals respectively on the contralateral striatum (7). A meta-analysis of 53 cases conducted by Oh et al showed that 89% of the cases had hemichorea with contralateral putaminal hyperintensity and 11% had generalised chorea with bilateral putaminal changes on MRI(8).…”
Background: Hemichorea-hemiballismus is a spectrum of involuntary, continuous non-patterned movement involving one side of the body. Possible causes of hemichorea-hemiballismus include haemorrhagic or ischemic stroke, neoplasm, systemic lupus erythematosus, NHH (non-ketotic hyperglycaemic hemichorea), Wilson’s disease, and thyrotoxicosis. Amongst the metabolic causes, chorea associated with NHH is noteworthy and is mainly reported in elderly Asian women. The pathophysiology of this syndrome remains controversial. It is likely that a combination of hyperglycaemia induced basal ganglia metabolic derangement and failure of cerebral blood flow autoregulation contribute to the syndrome.Case presentation: A 45-year-old Malay gentleman presented to our Emergency Department with right upper and lower limb weakness associated with hemichorea for 3-4 days. His initial blood glucose level was 22 mg/dl with normal serum ketone and bicarbonate levels. CT brain showed a hyperdensity in the left caudate nucleus and globus pallidus region. Subsequent brain MRI revealed an asymmetric T1 hyperintensity of the left putamen. This specific finding was compatible with hyperglycaemia-induced hemichorea-hemiballismus syndrome. The hemiballismus/hemichorea improved rapidly within the next day. Conclusions: This unusual clinical presentation is often accompanied by severe hyperglycaemia. Appropriate blood glycaemic control is important because it is reversible with correction of hyperglycaemia. Thus, prompt recognition and treatment is essential to avoid adverse outcomes.
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