A Case of Non-ketotic Hyperglycemic Hemichorea and Fahr Syndrome

TL;DR

A patient with diabetes and hypoparathyroidism developed a rare movement disorder linked to high blood sugar and brain calcifications, highlighting the need for careful diagnosis.

Contribution

This case report adds to the understanding of NHH and Fahr syndrome co-occurrence and emphasizes systematic diagnostic approaches.

Findings

The patient's bilateral chorea was linked to abrupt diabetes regimen discontinuation and brain calcifications.

Brain imaging showed diffuse calcifications consistent with Fahr syndrome.

Treatment with tetrabenazine and diabetes management improved symptoms.

Abstract

Non-ketotic hyperglycemic hemichorea (NHH) denotes acute hemichorea or hemiballism in patients with poorly controlled diabetes with striatal abnormalities seen on brain MRI. Here, we describe a case with diabetes mellitus and primary hypoparathyroidism who developed NHH with bilateral chorea due to the abrupt stopping of her diabetic regimen. She presented with subacute and progressive bilateral asymmetric chorea. Over the prior six months, she stopped following her diabetic regimen. Brain imaging showed features of diffuse brain calcifications suggestive of Fahr syndrome. Extensive blood investigations including genetic testing for causes of basal ganglia calcifications were unremarkable. Treatment with tetrabenazine and resumption of her diabetes medications slowly improved her chorea. This case highlights the importance of interpreting imaging findings in the context of the nature and time course of the chorea presentation. In addition, it emphasizes a systematic approach to interpreting diffuse brain calcifications with the appropriate investigations.

Figures