Unraveling Nonketotic Hyperglycemia Hemichorea-Hemiballismus Syndrome: A Case Report of Diagnosis and Management

Cureus. 2024 Jul 22;16(7):e65094. doi: 10.7759/cureus.65094. eCollection 2024 Jul.

Abstract

Nonketotic hyperglycemia hemichorea-hemiballismus syndrome (NHH) is an uncommon neurological condition linked to poorly managed diabetes mellitus (DM). It presents with spontaneous, erratic movements that impact just one side of the body. Our case of NHH was of a 76-year-old female with uncontrolled type 2 DM, ischemic heart disease, and dilated cardiomyopathy. Despite previous treatment for similar symptoms, the patient developed left-sided choreo-ballistic movements. Despite difficulties obtaining clear magnetic resonance imaging (MRI) due to involuntary movements, the image revealed T1 hyperintense signals in the right lentiform nucleus and subtle signals in the left lentiform nucleus and external capsule. Management included insulin, tetrabenazine, haloperidol, lorazepam, and other adjunctive therapies, resulting in symptom resolution by the fourth day. This case underscores the importance of considering NHH in patients with uncontrolled DM presenting with abnormal movements, highlighting the challenges in imaging due to involuntary movements and emphasizing the need for aggressive glycemic control and treatment strategies.

Keywords: chorea and hemiballismus; diabetic hemi-chorea; hemichorea-hemiballismus; nonketotic hyperglycemia; type-2 diabetes mellitus.

Publication types

  • Case Reports