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Abstract Details

Dancing Diabetes: A rare pediatric case of non-ketotic hyperglycemic hemichorea-hemiballismus
Child Neurology and Developmental Neurology
Child Neurology and Developmental Neurology Posters (7:00 AM-5:00 PM)
047
To report the clinical course and MRI findings in a case of pediatric non-ketotic hyperglycemic hemichorea-hemiballismus (HH) in the setting of poorly controlled type 1 diabetes and lactic acidosis without diabetic ketoacidosis.  
Non-ketotic hyperglycemic HH, classically associated with high T1 signal in the basal ganglia, is usually seen in elderly adults with poorly controlled type 2 diabetes. To our knowledge, there are only six other reported cases in children, and these have not reported concurrent lactic acidosis. 
NA
The patient, an eleven-year-old girl with poorly controlled type 1 diabetes mellitus with onset at 18 months of age, presented with right-sided involuntary movements. On initial presentation to another hospital three days after symptom onset, functional movement disorder was suspected, and the patient was discharged. Three days later, she presented to our hospital because movements had spread from the right arm to the right leg. Examination showed right sided HH evidenced by near-constant, irregular low amplitude fidgety movements of the distal right arm with superimposed higher amplitude quick jerks affecting the proximal right extremities. MRI brain demonstrated T1 hyperintense (FLAIR hypointense) signals, associated with blooming artifact, symmetrically within both globus pallidi, lentiform nuclei and thalami, and the left caudate nucleus. Glucose was elevated (317), though urine ketones were absent. HbA1C was 12.7%.  Lactic acid was elevated to 6 despite no evidence of sepsis or dehydration. The HH improved on correction of hyperglycemia. Residual HH was only slight, and medications were not necessary for symptomatic treatment.   
Non-ketotic hyperglycemic HH is exceedingly uncommon in children and may be mistaken for functional disorder. MRI shows characteristic high T1 signal in the striatum which can be unilateral or bilateral. The HH usually improves with treatment of the hyperglycemia. The elevation of lactate without evidence of diabetic ketoacidosis, sepsis, or dehydration reported in our case is unique. 
Authors/Disclosures
Allie C. Toth, MD (University of Michigan Health - West)
PRESENTER 		Dr. Patterson has nothing to disclose.
No disclosure on file