Unilateral Basal Ganglia Hyperdensity in a Previously Undiagnosed Diabetic Patient Presenting with Hemichorea-Hemiballism (Hchb)

Abstract

Non-ketotic hyperglycemia induced hemichorea-hemiballism (HCHB) is a rare condition with characteristic neuroimaging findings in a diabetic patient. This paper reports a 61-year-old male previously undiagnosed diabetic patient presenting with HCHB as the first presentation of diabetes.

Journal of Nobel Medical College
Volume 5, Number 1, Issue 8, January-July 2016, 66-68

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ISSN: 2091-2331 (PRINT) 2091-234X (ONLINE)
*Corresponding Author: Dr.Manoj Bhattarai, Lecturer | E-mail: drbhattaraimanoj@gmail.com 666
Journal of Nobel Medical College
Available Online: www.nepjol.info, www.nobelmedicalcollege.com.np
Volume 5, Number 1, Issue 8, January-July 2016, 66-68
Case Report
Unilateral Basal Ganglia Hyperdensity in a Previously Undiagnosed
Diabetic Patient Presenting with Hemichorea-Hemiballism (Hchb)
*Manoj Bhattarai
Department of Radiodiagnosis and Imaging, Nobel Medical College Teaching Hospital, Biratnagar
Received 20th June, 2016; Revised after peer-review: 12th July, 2016; Accepted 28th July, 2016
Abstract
Non-ketotic hyperglycemia induced hemichorea-hemiballism (HCHB) is a rare condition with
characteristic neuroimaging findings in a diabetic patient. This paper reports a 61-year-old
male previously undiagnosed diabetic patient presenting with HCHB as the first
presentation of diabetes.
Key Words: Basal ganglia hyperdensity, Computed tomography (CT) scan, Diabetes
mellitus, Hemichorea-hemiballism, Non-ketotic hyperglycemia.
Introduction
Hemichorea-hemiballism associated with
hyperglycemia is a rare and potentially
reversible condition in a patient with non-
ketotic hyperglycemia [1] and was first
described by Bedwell in 1960 [2].
Literature describes this condition occurring
most frequently in elderly patients with
type II diabetes mellitus and majority are
female and Asians [3, 4].
Neuroimaging studies of these patients
show characteristic findings of
contralateral basal ganglia hyperdensity in
non-contrast Computed tomography (CT)
scan, corresponding with T1 shortening in
contralateral basal ganglia in Magnetic
resonance imaging (MRI) [3-5].
However, HCHB as a rare presenting
manifestation of type II diabetes mellitus in
patients with no previous history of
diabetes has been described and should
always be thought of in elderly patients
presenting with HCHB [5-9].
Case Presentation
A 61-year-old male presented to
emergency department of our hospital with
two days history of involuntary movements
of right upper and lower limbs. There was
no past history of hypertension, diabetes
mellitus, trauma, stroke, movement
disorders or intake of neuroleptic drugs,
with no family history of similar illness or
other movement disorders. On examination
he was fully conscious and oriented. HCHB
of right upper and lower limb was present.
Muscle strength and tone were normal.
Blood glucose level was 494 mg/dl and
HbA1c was 7.8 %. On urinalysis no
ketones were detected. Diagnosis of type II
diabetes mellitus with non-ketotic
hyperglycemia was made and patient was
referred for CT scan of brain. Non-contrast
CT scan of brain showed hyperdensity of
left lentiform and caudate nucleus with
sparing of anterior limb of left internal
capsule. No surrounding edema or mass
effect was seen. There was no change in
appearance of lesion on post contrast
images. No other significant intracranial
abnormality was seen. (Figure 1).

Manoj Bhattarai, Journal of Nobel Medical College
Journal of Nobel Medical College Vol. 5, No.1 Issue 8 676
Figure 1: Non-contrast CT scan of brain showing
hyperdensity of left lentiform and caudate nucleus.
Patient was treated with insulin and oral
hypoglycemic agents for elevated blood
sugar level. Haloperidol was given for
involuntary movements. With treatment
blood sugar level was restored too normal
and marked symptomatic improvement
was seen. After a week of treatment HCHB
completely disappeared.
Discussion
Even though HCHB associated with non-
ketotic hyperglycemia is mostly seen in
previously diagnosed diabetic patients with
poor glycemic control, there are literatures
describing HCHB as a rare presenting
manifestation of diabetes [5-9], as in this
case. In these patients, the characteristic
neuroradiological findings are seen in basal
ganglia contralateral to the side of the
patient’s symptoms as seen in present
case, with putamen involved in all cases
and no isolated involvement of caudate
nucleus or globus pallidus [3]. CT scan of
brain shows non-enhancing hyperdensity in
contralateral basal ganglia. Similarly, MRI
scan of brain shows hyperintensity in
contralateral basal ganglia in T1 weighted
images, whereas T2 weighted / FLAIR
images show hypo or isointensity.
Although findings are characteristically
seen unilaterally, changes in bilateral basal
ganglia may be noted [3-5, 10, 11].
The exact underlying pathophysiology for
radiological changes seen in non-ketotic
hyperglycemia induced HCHB are not
clearly known. There are various theories
for this, which includes blood
hyperviscosity, depletion of gamma
aminobutyric acid (GABA) and
acetylcholine, ischemia, petechial
haemorrhage, calcium deposition,
metabolic acidosis, myelinosis, gliosis with
abundant gemistocytes and manganese
accumulation on gemistocytes [6, 12, 13].
Various differential diagnosis depending
upon clinical and imaging findings of
chorea, basal ganglia hyperdensity and T1
basal ganglia hyperintensity are described,
that includes intracranial hemorrhages,
cerebral ischemia, vasculitis, drugs, central
nervous system lupus, multiple sclerosis,
basal ganglia calcifications, chronic hepatic
encephalopathy, manganese toxicity
(following long term parenteral nutrition),
hypoglycemic coma, neurofibromatosis,
Wilson disease, Fahr disease and carbon
monoxide poisoning, which can be
excluded depending upon other clinical and
imaging findings in these conditions [5, 13,
14].
The clinical improvement and resolution of
symptoms in these patients with HCHB
occurs once blood glucose level returns to
normal. Also the radiological abnormalities
in basal ganglia will resolve over a period
of time [13].
Conclusion
Non-ketotic hyperglycemia induced HCHB
may be the first presenting manifestation
of type II diabetes mellitus and should be
considered in differential diagnosis of
patients, especially elderly presenting with
involuntary movements. It has
characteristic neuroimaging findings, and
together with clinical findings early
recognition of this condition is important as
it is easily treatable and correction of
hyperglycemia leads to spontaneous
resolution of symptoms.
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