The Journal of Emergency Medicine, Vol. 50, No. 3, pp.
e133e134, 2016
http://dx.doi.org/10.1016/j.jemermed.2015.11.016
Clinical
Communications: Adults
NONKETOTIC HYPERGLYCEMIA PRESENTING AS MONOBALLISM
Amitesh Aggarwal, MD, Nitin Bansal, MD, and Raghav Aggarwal, MD
Department of Medicine, University College of Medical Sciences, Delhi University, Delhi, India
Corresponding Address: Nitin Bansal, MD, Department of Medicine, University College of Medical Sciences, Delhi 110095, India
, AbstractBackground: Monoballismus is rarely seen
clinically, but when observed, it is usually a manifestation
of an acute cerebrovascular accident (CVA). We report a
case of monoballismus observed in a patient without evi-
dence of a CVA. Case Report: We observed a case of mono-
ballismus in a 60-year-old diabetic patient who had not had a
stroke. The movement disorder resolved with improvement
of the patients hyperglycemia. Nonketotic hyperglycemia is
an uncommon cause of ballismus. Why Should an Emergency
Physician be Aware of This?: Emergency physicians should
include the movement disorder of ballismus among the po-
tential clues that a patient may be suffering an acute CVA.
However, noncerebrovascular causes of ballismus exist.
The movements manifest by a patient with ballismus should
also lead the physician to consider the possibility not only of
a CVA, but also neuroleptic malignant and serotonin syn-
dromes. 2016 Elsevier Inc.
, Keywordsballismus; hemichorea; nonketotic hyper-
glycemia
INTRODUCTION
Monoballismus is rarely seen clinically, but when
observed, it is usually a manifestation of an acute cere-
brovascular accident (CVA). Monoballismus manifests
as a violent form of choreiform movement, consisting
of uncontrolled, flinging, large-amplitude movements of
Reprints are not available from the authors.
RECEIVED: 23 September 2015; FINAL SUBMISSION RECEIVED: 24 October 2015;
ACCEPTED: 13 November 2015
e133
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one limb. The most common cause of monoballismus is
an infarction or hemorrhage within the subthalamic nu-
cleus that causes irreversible neurologic damage. Howev-
er, a rare cause of ballismus is nonketotic hyperglycemia
in patients with diabetes mellitus (1). It is more common
among postmenopausal women and can, in rare in-
stances, be the first presentation of diabetes mellitus
(2). It is important for an emergency physician to recog-
nize the significance of this entity because glycemic con-
trol leads to rapid reversal of the abnormal movements.
We report a case of nonketotic hyperglycemiainduced
monoballismus.
CASE REPORT
A 60-year-old woman presented to our emergency
department with a chief complaint of involuntary, jerking
movements initiated by the proximal muscle groups of
her left upper limb. She was diabetic and hypertensive,
with the complication of diabetic nephropathy. Her med-
ications included insulin and amlodipine. She was re-
ported to have been in her normal state of health until
the day before presentation. Her abnormal movement
began the day before presentation, on a day when she
missed her two scheduled doses of insulin because of un-
expected travel. The involuntary movements had sub-
sided during her sleep during the previous night. She
presented to our emergency department with involuntary
movement and a blood glucose level of 512 mg/dL. Her
urine was negative for ketones, and her arterial pH was
7.41. Because of the duration of the symptoms and the
e134
suspicion of an acute ischemic CVA, a noncontrast mag-
netic resonance imaging scan of her brain was performed.
However, there was no evidence of ischemia or hemor-
rhage. Her blood urea nitrogen measurement was
65 mg/dL and her serum creatinine level was 2.5 mg/
dL. Deranged kidney functions were consistent with her
nephropathy, and there was no acute rise in creatinine
levels compared to her old medical records. The
remainder of her routine laboratory investigations was
normal.
We considered the possibility of a simple partial
seizure, and she was given an intravenous loading dose
of phenytoin, but her involuntary movements did not
improve. She subsequently began a course of intravenous
insulin. Her abnormal movements decreased on day 1 and
subsided completely with no neurological sequelae on the
second day as her blood sugar level returned to normal.
She was observed for 48 hours with no recurrence of
the abnormal movements. She was discharged with
advice for strict adherence to her insulin regimen.
DISCUSSION
Hemiballismus is a violent form of chorea comprised of
wild, flinging, large-amplitude movements on one side
of the body. Proximal limb muscles tend to be predomi-
nantly affected. These movements may affect just one
limb (monoballism) or, less commonly, both upper or
lower limbs (paraballism). The most common cause is a
partial lesion (i.e., infarct or hemorrhage) in the subthala-
mic nucleus, but cases of lesions in the putamen, thal-
amus, and parietal cortex have been reported (3).
The exact pathogenesis of ballismus with hyperglyce-
mia is poorly understood. The pathogenesis is thought to
be related to hyperglycemia-induced perfusion changes
in the contralateral striatum and ischemic excitotoxicity
neurons that produce g-aminobutyric acid (i.e., GABA-
ergic neurons) (4). During hyperglycemia, the activity
of Krebs cycle is suppressed and glucose utilization is
altered to alternative mechanisms. In patients with nonke-
totic hyperglycemia, the shift to anaerobic metabolism
causes the brain to use GABA; the levels of GABA are
subsequently depleted. Depletion of GABA causes a
decrease in GABA-ergic activity, thereby decreasing
the inhibition of the thalamus by the medial part of the
globus pallidus. Hyperkinetic movements like hemi-
choreahemiballismus may therefore occur with nonke-
totic hyperglycemia (5).
The computed tomography scan may show an area of
hyperdensity in the basal ganglia when the cause of bal-
lismus is a CVA. The characteristic magnetic resonance
imaging finding is high signal intensity in T1-weighted
A. Aggarwal et al.
images in the putamen, contralateral to the side afflicted
by ballismus (6). The diagnosis is made in the presence
of typical clinical and radiological features in the pres-
ence of nonketotic hyperglycemia.
Infarct and hemorrhage in the subthalamic nucleus are
the most common causes of ballismus, but they cause
irreversible neurological damage. Medications, such as
haloperidol or risperdone, are generally required to
manage ballismus. However, in patients with nonketotic
hyperglycemia and ballismus, the most important aspect
of treatment is aggressive glycemic control, which results
in resolution of the ballismus in the majority of cases.
Emergency physicians must therefore recognize that
easily correctable states like hyperglycemia may lead to
ballismus, and that it usually resolves without any neuro-
logical sequelae. In refractory cases, drugs that block
postsynaptic dopaminergic receptors (e.g., haloperidol
and risperidone) may be used (7). Radiological lesions
are late to resolve and may take months to disappear
completely.
The presentation of our case was typical in that the pa-
tient presented with monoballismus and nonketotic hy-
perglycemia that improved drastically with blood sugar
control. No abnormalities caused by acute cerebral
ischemia were detected in the patients brain imaging.
WHY SHOULD AN EMERGENCY PHYSICIAN BE
AWARE OF THIS?
This case emphasizes that hyperglycemia is an uncom-
mon but reversible cause of hemichoreahemiballismus.
It is important for the emergency physician to consider
hyperglycemia as an easily remedied cause of hemi-
choreahemiballismus.
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