Paediatric non-ketotic hyperglycaemic hemichorea–hemiballismus

• Published in: BMJ case reports 2018-04, Vol.2018, p.bcr-2017-223429
• Main Authors: Suratos, Cezar Thomas Reyes, Benitez, James Albert Edward Lim, Urquiza, Sheen Corvera, Sacro, Cheryl Anne Lubaton
• Format: Article
• Language: English
• Subjects: Adolescent; Anti-Dyskinesia Agents - therapeutic use; Blood Glucose; Chorea - blood; Chorea - diagnosis; Chorea - drug therapy; Chorea - etiology; Clonazepam - therapeutic use; Diabetes Mellitus, Type 1 - blood; Diabetes Mellitus, Type 1 - complications; Diabetes Mellitus, Type 1 - physiopathology; Dyskinesias - blood; Dyskinesias - diagnosis; Dyskinesias - drug therapy; Dyskinesias - etiology; Female; Haloperidol - therapeutic use; Humans; Hypoglycemic Agents - therapeutic use; Insulin - blood; Insulin - therapeutic use; Neuroimaging; Patient Compliance; Rare Disease; Tomography, X-Ray Computed; Treatment Outcome
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2017-223429

Non-ketotic hyperglycaemic hemichorea–hemiballismus (NHHH) is commonly seen among elderly Asian women with type 2 diabetes mellitus. Here, we present a case of a 16-year-old Filipina with type 1 diabetes mellitus who is poorly compliant to her medications and subsequently developed right hemichorea–hemiballismus (HH). She was initially admitted with hyperglycaemia but was negative for ketonuria or metabolic acidosis. Neuroimaging showed bilateral lentiform nuclei and left caudate hyperdensities on CT and T1-weighted hyperintensity on MRI. Blood glucose was controlled with insulin. Haloperidol and clonazepam were started for the HH with gradual resolution of symptoms in 6 weeks. This is the fifth reported case of NHHH seen among the paediatric age group. NHHH in the paediatric population is clinically and radiographically similar to NHHH seen among adults. Correction of hyperglycaemia results in clinical improvement and radiographic resolution of lesions but persistent cases may necessitate specific treatment targeted towards the abnormal movements.