Unilateral hemichorea/hemiballism (HH) associated with contralateral neuroimaging abnormalities of the basal ganglia, which is characterized by T1 hyperintensity on magnetic resonance imaging (MRI) and is secondary to diabetic nonketotic hyperglycemia, is a rare and unique complication of poorly controlled diabetes mellitus (DM). Although almost all prior reports have documented rapid resolution of HH within days after normalization of blood glucose levels, medically refractory persistent HH has been noted. The experience of surgical intervention for persistent HH is limited. A 46-year-old, right-handed female patient with type 2 DM presented with refractory diabetic HH on the left side of 6 months’ duration despite DM control and neuroleptic medication usage. Image-guided deep brain stimulation (DBS) on the right globus pallidus internus (GPi) was performed. A mechanical micropallidotomy effect was observed and chronic stimulation of GPi was quite effective in symptomatic control of diabetic HH until a 16-month follow-up visit. DBS of the GPi can be an effective treatment for medically refractory diabetic HH.
Hemichorea/hemiballism (HH) is a hyperkinetic movement disorder characterized by acute or subacute onset of high-amplitude, involuntary movements affecting one side of the body [
The clinical course of diabetic HH is favorable and the symptoms tend to resolve rapidly with normalization of hyperglycemia in patients who develop diabetic HH secondary to nonketotic hyperglycemia [
A 46-year-old, right-handed female patient with an 11-year history of type 2 diabetes mellitus (DM) presented with a continuous, violent choreic/ballistic movement of the left arm, leg, and trunk of 6 months’ duration. At the beginning, she experienced vague discomfort in her left arm after a brief episode of heavy lifting. An involuntary choreic movement gradually developed in her arm within several hours. Owing to cramping pain associated with a flinging movement in her left arm and hand, she was initially treated with nonsteroidal anti-inflammatory drugs and physical therapy. However, the choreic movement progressively worsened over the following 2 weeks and eventually became ballistic and involved her left arm and leg and had a high amplitude. She was admitted to another hospital via the emergency department for evaluation. Neurologic examination showed facial dyskinesis (grimacing) and a ballistic movement in her left trunk, arm, and legs. The movements could not be suppressed voluntarily but ceased during sleep. Muscle tone and strength in the upper and lower extremities were normal bilaterally. There was no sensory impairment and her cranial nerves were normal. Laboratory studies revealed a fasting blood glucose level of 536 mg/dl, a serum osmolarity of 335 mOsm/kg, and an HbA1c count of 15.7%. Urinalysis was negative for ketones. There was no history of dopamine agonist or estrogen medication use or rheumatic fever/Sydenham’s chorea.
A T1-weighted MRI image revealed a region of increased signal intensity restricted to the right putamen and globus pallidus, which was isointense on T2-weighted images (Figure
Magnetic resonance imaging (MRI) findings of diabetic hemichorea/hemiballism.
A T1-weighted MRI image showing hyperintensity involving the right putamen and globus pallidus. The caudate nucleus was spared in this case
An irregular area of hypointense lesion within the isointense putamen and globus pallidus was found on a T2-weighted MRI image
A T1-weighted MR image taken 16 months after the onset of diabetic HH. T1 hyperintensity in the right putamen was still identified but much attenuated, compared to the initial MRI. Note the hypointense metallic artefact of the DBS lead (arrow)
The patient underwent a stereotactic MRI scan (1.5 tesla Archieva®, Philips, Best, The Netherlands), and volumetric T1-weighted three-dimensional images, T2-weighted images, and proton density images were obtained. The images were transferred to a Framelink® planning station (version 4.1, Medtronic, Minneapolis, MN, USA) so that we could determine the coordinates of the image-guided GPi target and were reformatted for extraventricular trajectory planning (Figure
Image-guided deep brain stimulation (DBS) of the globus pallidus internus (GPi) for refractory diabetic HH.
An image in the planning software showing target localization and trajectory planning for right GPi DBS. The target was directed to the posterolateral portion of the GPi which was easily identified owing to hyperintensity in the volumetric T1-weighted MRI images
An image captured from the planning software after fusion of an intraoperative O-arm CT scan with preoperative volumetric T1 images to verify the location of the implanted lead, which was taken with the patient under general anesthesia. Although the electrode was found to be about 1 mm posterior and medial to the initial target point, it was accepted
An image captured from the planning software after fusion of the postoperative CT scan with preoperative volumetric T1 images. This image corresponds to the center of the 2nd contact (number 2), which was used as an anode for chronic stimulation
A prominent mechanical effect with significant reduction (50% reduction in intensity) of the choreic and proximal ballistic movements was observed. Bipolar electrical stimulation (100–130 Hz, 1–3 mA, 130 usec, up to 3.5 mA) further suppressed HH, and the hyperkinetic movements became grossly undetectable after 7 days of external stimulation. No stimulation-related side effects such as dysarthria, dysphasia, or motor contraction were observed. A pulse generator (Libra®, St. Jude Medical, Plano, TX, USA) was implanted via a transaxillary subpectoral route after 7 days of external stimulation [
Transient chorea/ballism provoked by an episode of nonketotic hyperglycemia has repeatedly been reported over the past couple of decades and is the second most common cause of hemiballism [
High intensity in the basal ganglia on T1-weighted MRI is a characteristic finding in diabetic HH [
The putamen has been suggested to play a central role in diabetic HH [
Diabetic HH generally resolves over time, and often no treatment is necessary. Determining the etiology of the condition is a matter of paramount importance and correcting nonketotic hyperglycemia along with long-term diabetic control is the mainstay of treatment [
Summary of stereotactic surgery for diabetic hemichorea/hemiballism.
Study/year | Number of patients | Age/sex | Timing of surgery | Surgery (lesion/DBS) | Results | follow-up period | Remarks |
---|---|---|---|---|---|---|---|
Takamatsu et al. [ |
1 | 57/f | 1.5 mo. | VL lesion | Excellent | 4 years | |
Nakano et al. [ |
1 | 65/m | 5 mo. | VO (Voa, Vop) DBS | Effective | 9 mo. | Persistent HH in off stimulation |
Goto et al. [ |
1 | 78/f | N/A | GPi lesion | Excellent | 12 mo. | HH immediately disappeared |
Current case, 2017 | 1 | 46/f | 6 mo. | GPi DBS | Effective | 16 mo. | Persistent HH in off stimulation |
GPi: globus pallidus internus; HH: hemichorea/hemiballism; mo: months; VL: ventral lateral nucleus; VO: ventralis oralis; Voa: ventralis oralis anterior; Vop: ventralis oralis posterior.
Owing to the limited reports on surgical intervention against diabetic HH, the timing and type of surgery, lesioning, or DBS are difficult to determine. However, despite the limited evidence, both lesioning and stimulation in the VL or VO thalamus and GPi showed good results [
We report the long-term effectiveness of GPi DBS in the treatment of chronic, disabling diabetic HH.
The authors declare that they have no conflicts of interest regarding this manuscript.