Neurol Sci (2008) 29:289–290
DOI 10.1007/s10072-008-0984-4

LETTER TO THE EDITOR

A case report on pituitary macroadenoma presented as hemichorea
hemiballism syndrome
Sevda Erer • Selcuk Yilmazlar • Mehmet Zarifoglu • Metin Guclu

© Springer-Verlag 2008

Sir,
A 36 year old woman was referred to the emergency room
with a preliminary diagnosis of pituitary macroadenoma
presenting as hemichorea-hemiballismus (HC-HB). She
reported an acute onset of abnormal movement in the left
extremities. An initial physical examination showed an
acromegalic appearance. Neurological examination
revealed severe HC-HB, especially prominent in the left
upper extremity.
Laboratory and endocrine evaluation showed: elevated
blood glucose, 471 mg/dL (normal range, 70–110); glycosuria; and elevated GH, 40.00 ng/mL (normal range,
0.06–5.00). Ketones and serum were not detected, and no
electrolyte imbalance was observed. MRI revealed a
4x4x5 cm homogeneous intrasellar-suprasellar mass
extending into the sphenoid and cavernous sinuses bilaterally. T1-weighted hyper intensity of the bilateral caudate

S. Erer · M. Zarifoglu
Uludag University, School of Medicine
Department of Neurology
Bursa, Turkey
S. Yilmazlar (ì¾·)
Uludag University, School of Medicine
Department of Neurosurgery
Gorukle kampus, Bursa, 16059 Turkey
e-mail: selsus@uludag.edu.tr
M. Guclu
Uludag University, School of Medicine
Department of Endocrinology
Bursa, Turkey

and right lentiform nuclei was observed (Fig. 1a, b). The
patient was diagnosed non-ketotic hyperglycaemia with
HC-HB. While the patient received insulin therapy treatment, the abnormal movements continued for three
weeks. Such patients typically have elevated serum glucose levels, but are unaware of existing diabetes mellitus.
Initially, our patient underwent conventional MRI with
gadolinium enhancement. T1- and T2-weighted MR
images showed high signal intensities in the right and left
heads of the caudate nucleus and in the putamen. At the
beginning of therapy, symptoms were treated with, but not
controlled by, insulin alone. The addition of a dopamine
receptor blocking agent quetiapine (25 mg twice a day)
was effective over time. Diffusion-weighted (DW)-MRIs
were obtained during the persistence of symptoms. DWMRIs did not show high signal intensities reflecting
ischaemia in the corresponding lesions. The movement
disorders improved gradually followed by satisfactory
regulation of blood glucose levels within two months.
Transphenoidal surgery was planned to remove the
tumour.
Sudden onset HC-HB syndrome caused by hyperglycaemia often presents in elderly patients who have a history of diabetes mellitus. MR images from these patients
often reveal contralateral putaminal hyperintensity [1].
The main reason of non-ketotic hyperglycaemia induced
HC-HB remains unknown. It is thought that reduction of
regional blood flow in basal ganglia caused by hyperglycaemia leads the metabolism into an anaerobic pathway
which damages GABA-inhibitory neurons in the striatum
that disinhibits the external segment of the globus pallidus
(GPe) and subthalamic nucleus resulting in hyperkinetic
abnormal movements expressed as HC-HB. It has also
been reported that metabolic acidosis causes reversible
ischemia in basal ganglia without developing any infarction [2]. All these circumstances give rise to a hyperin-

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a

Neurol Sci (2008) 29:289–290
Fig. 1 Coronal T1-weighted magnetic resonance imaging (a) reveals
a 4x4x5 cm enhancing homogeneous intrasellar-suprasellar mass
infiltrating into the sphenoid and
cavernous sinuses bilaterally. The
axial image (b) displays increased
intensity involving the bilateral caudate and right lentiform nuclei, partially sparing the left lentiform
nucleus

b

tense image of cranial MR T1 sequence as it also reported in this presented case.
Considering all these former reports along with clinical and radiological findings we speculate that the diagnosis of our case is hyperglycaemia-induced HC-HB.
There are many previous reports of uncontrolled diabetes mellitus first presenting as HC-HB [1, 3–6]. This is
the first report of a GH secreting, invasive pituitary
macroadenoma causing HC-HB through long lasting
hyperglycaemia
Extrinsic or intrinsic tumours may involve structures
of basal ganglia, or their connections, by interruption of
neuronal circuits as the possible mechanism for movement disorders. Our observations, however, support a
mechanism that contrasts with the one postulated by
Bhatoe [7]. We concluded that HB-HC may be an initial
presentation of long lasting GH secreting pituitary adenoma by hormonal hyperfunction, but not by direct compression.

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