Blood brain barrier destruction in hyperglycemic chorea in a patient with poorly controlled diabetes

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Abstract

A case of hemichorea in a patient with poorly controlled diabetes is reported. T1-weighted magnetic resonance imaging (MRI) showed an unusual homogeneous high-intensity area in the corpus striatum. Of interest in the case was the fact that the globus pallidus, which was enhanced with gadolinium at the onset of hemichorea, showed homogeneous high-intensity on a subsequent T1-weighted image. This indicated that blood brain barrier destruction preceded the signal intensity change in the basal ganglia. As far as the authors could determine, this is the first reported case showing such enhancement during the course of diabetic hemichorea.

Introduction

A case of hemichorea in a patient with poorly controlled diabetes is reported. T1-weighted magnetic resonance imaging (MRI) showed an unusual homogeneous high-intensity area in the corpus striatum. Of interest in the case was the fact that the globus pallidus, which was enhanced with gadolinium at the onset of hemichorea, showed homogeneous high-intensity on a subsequent T1-weighted image. This indicated that blood brain barrier destruction preceded the signal intensity change in the basal ganglia. As far as the authors could determine, this is the first reported case showing such enhancement during the course of diabetic hemichorea.

Hemichorea has been reported as a rare manifestation of nonketotic hyperglycemia [1], [2], [3]. In some reports, high signal intensity in the basal ganglia is observed on T1-weighted magnetic resonance (MR) images [4], [5], [6], [7], [8]. The lesion in hemichorea is now regarded as a petechial hemorrhage from small vessels [9] and hyperglycemia is said to play some role in the evolution of such diapedesis, but the nature of the lesion remains controversial. Here we present a case of diabetic hemichorea that showed enhancement in the globus pallidus with gadopentetate dimeglumine (Gd-DTPA) on T1-weighted MR image. The enhanced area showed T1 shortening 40 days later. This indicates that blood brain barrier (BBB) destruction preceded formation of the high-intensity lesion. The finding supports the hypothesis that diapedesis is due to BBB destruction resulting from hyperosmotic state caused by hyperglycemia.

A 64-year-old-man was admitted to Kanto Teishin Hospital on August 22, 1996, due to sudden onset of left hemichorea. He had been diagnosed with diabetes mellitus during an annual check-up 1 year prior to admission (random blood glucose 22.2 mmol/l (400 mg/dl)), but he refused medication, and even dietary control. He smoked one pack of cigarettes and consumed one can of beer every day. There was no family history of involuntary movements. On admission, his blood pressure was 130/69 mmHg and the pulse was 66/min and regular; he weighed 58 kg and was 157 cm tall. He was alert and the mental state was normal.

Neurological examination revealed left hemiballism–hemichorea with left-sided pyramidal signs. There was no cranial nerve involvement except for left hemifacial choreic movements. Muscle tone was decreased in the left extremities, but there was no weakness. Sensation was intact and no cerebellar sign was observed. Involuntary movement disappeared during sleep and increased with emotional tension.

The following laboratory data were notable: fasting blood glucose 31.5 mmol/l (567 mg/dl), serum osmolarity 301 mmol/kg, glycosylated hemoglobin A1c 17.3%. On blood gas analysis, there was no metabolic acidosis. Urinalysis was strongly positive for glucose and ketones and negative for protein.

Brain CT scan on admission (about 26 hours after the onset) showed a slight high-density area in the right putamen and the caudate head in accordance with the structures without any mass effect or edema. On MRI (Signa HORIZON HiSpeed 1.5T (General Electric, Milwaukee MI)) performed 11 days after admission, the entire right putamen and caudate head showed high-intensity on T1-weighted images (Fig. 1A) and slightly low intensity on T2-weighted images (Fig. 1B). The right globus pallidus was enhanced using Gd-DTPA (Fig. 1C). Also on day 11, a brain CT scan was performed again, and it revealed disappearance of the high-density area observed on the initial CT scan and globus pallidus showed slight low-density. Single photon emission computed tomography (SPECT) with 99mTc-ethyl cysteinate dimer (ECD) performed 32 days after admission showed reduced accumulation in the right basal ganglia. On day 58, the entire right corpus striatum and the globus pallidus, which were previously enhanced, revealed high signal intensity on T1-weighted MRI (Fig. 1D). At this time, no enhancement was observed. On day 100 after admission, the right corpus striatum showed moderate atrophy as well as the CT scan, but the high-intensity on the T1-weighted image had not changed.

The patient’s involuntary movement gradually improved with administration of haloperidol and tiapride.

Section snippets

Discussion

Although the putamen is the most common site for primary intracerebral hemorrhage, hemichorea is a relatively rare symptom. Compared to diabetic hemichorea, which does not affect the internal capsule, the hypertensive putaminal hemorrhage commonly does affect it, causing some degree of hemiparesis. Jones et al. reported a 42-year-old man with apparent putaminal hypertensive hemorrhage (hematoma with mass effect) associated with hemichorea [10]. Unlike usual putaminal hemorrhage, the lesion

Acknowledgments

The authors would like to thank Dr Makoto Iwata, Department of Neurology, Tokyo Women’s Medical University, for his critical comments.

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