technetium-tc-99m-exametazime and Chorea

Left-sided hemichorea developed suddenly in a 73-year-old male. Computed tomography revealed a left subdural hematoma (SDH) and infarction in the right corona radiata and temporo-occipital region. Hemichorea subsided completely after removal of the SDH. Postoperative single photon emission computed tomography with technetium-99m-hexamethyl-propyleneamine oxime revealed a global low-perfusion area in the right cerebral hemisphere. Right carotid angiography demonstrated severe stenosis of the trunk of the right middle cerebral artery. The cerebral blood flow in the right cerebral hemisphere had probably already decreased to nearly the critical level and was reduced further by the left SDH, inducing the left-sided hemichorea due to dysfunction of the right cerebral hemisphere. This case shows that when hemichorea ipsilateral to a SDH is present, it is important to ascertain whether there is a pre-existing ischemic lesion in the contralateral cerebral hemisphere, particularly in the basal ganglia, thalamus, or corona radiata.

Topics: Aged; Cerebral Infarction; Chorea; Chronic Disease; Dominance, Cerebral; Hematoma, Subdural; Humans; Magnetic Resonance Imaging; Male; Organotechnetium Compounds; Oximes; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

The objective of this study was to determine whether technetium-99m hexamethyl-propyleneamine oxime (HMPAO) brain single photon emission computed tomography (SPECT) imaging is capable of detecting perfusional abnormalities in Sydenham's chorea (SC) patients and contributing to diagnosis of SC.. In this study, 17 SC patients were evaluated. HMPAO SPECT was performed on all patients at the acute phase and six of them at the recovery phase. Magnetic resonance imaging (MRI) of the brain was performed to 13 patients. SPECT images of basal ganglia and thalamus were evaluated both visually and by measuring the radioactivity uptakes. Ten subjects constituted the control group.. HMPAO SPECT was visually evaluated as indicating hyperperfusion in the basal ganglia and thalamus in 16 patients, and evaluated as normal in one patient at the acute phase. The radioactivity uptakes of basal ganglia and thalamus of the patients at the acute phase were found statistically higher than those of the patients at the recovery phase, and also higher than those of the control group (P < 0.05). A significant difference between the radioactivity uptake of the patients at the recovery phase and those of the control group was not found (P > 0.05). Only one patient did not show any abnormality in both acute and recovery phases. MRI study did not show any abnormality in the basal ganglia and thalamus.. It is suggested that brain SPECT can contribute to the diagnosis of SC as an objective tool. Resolving of the hyperperfusion at the recovery phase provides further support for the diagnosis of SC.

Topics: Adolescent; Basal Ganglia; Brain; Child; Chorea; Humans; Magnetic Resonance Imaging; Radiopharmaceuticals; Sensitivity and Specificity; Technetium Tc 99m Exametazime; Thalamus; Tomography, Emission-Computed, Single-Photon

Functional imaging studies in patients with Sydenhams chorea have suggested brain perfusional abnormalities. In this study, we aimed to investigate the cerebral perfusion patterns of the cortical/subcortical structures by using Tc-99m hexamethylpropylenamine oxime single photon emission computed tomography in children with Sydenhams chorea, accompanied with magnetic resonance imaging and cranial Doppler studies. Brain MRI and Doppler studies of the all cases were normal. In the brain SPECT study, six patients were determined to have cerebral perfusion abnormalities. Although six patients responded well to the therapy, two girls who had hypoperfusion in the right frontal region as well as hypo- / hyperperfusion in the basal ganglia did not respond to therapy. While the chorea form of the patients who have cerebral perfusional abnormality was generalized, the clinical picture of the patients with normal cerebral perfusion was in the form of hemichorea.

Topics: Adolescent; Basal Ganglia; Cerebrovascular Circulation; Child; Chorea; Female; Humans; Magnetic Resonance Imaging; Male; Radiopharmaceuticals; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon; Ultrasonography, Doppler

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare and benign disorder with its onset in childhood. PKC generally improves with age, and its pathophysiology has not been revealed. We recorded both ictal and interictal SPECT in a 14-year-old girl with PKC. Ictal SPECT showed a significant decrease in blood flow in the caudate nucleus contralateral to the limb showing an involuntary movement. We also examined paired-pulse stimuli somatosensory evoked potential (SEP) of the same patient. Recovery pattern of P25 and N33 components was normal and comparable to 5 healthy volunteers, suggesting the absence of cortical hyperexcitability. These results suggest dysfunction or immaturity of the indirect pathway of basal ganglia in PKC, as well as the hyperexcitability of the descending pathway.

Topics: Adolescent; Athetosis; Chorea; Evoked Potentials, Somatosensory; Female; Humans; Radiopharmaceuticals; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

Paroxysmal kinesigenic choreoathetosis is a rare neurologic disorder characterized by sudden attacks of brief involuntary dyskinetic movement that are precipitated by voluntary movement. A 14-year-old male who presented with frequent brief attacks of hemidystonia triggered by sudden movement is reported. Investigations, including video electroencephalogram and magnetic resonance imaging of brain, were normal. There was excellent and sustained response to carbamazepine. Ictal single-photon emission computed tomography using (99m)Tc ethyl cysteinate dimer revealed increased perfusion of the contralateral basal ganglia, which is associated with onset of choreoathetosis attacks. Our findings provide evidence that hyperactivity of the basal ganglia is associated with the dyskinetic attacks in paroxysmal kinesigenic choreoathetosis.

Topics: Adolescent; Anticonvulsants; Athetosis; Brain; Carbamazepine; Cerebrovascular Circulation; Chorea; Electroencephalography; Humans; Kinesics; Magnetic Resonance Imaging; Male; Radiopharmaceuticals; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

Sydenham's chorea results from group A streptococcus infection and subsequent generation of antineuronal antibodies directed at the caudate nucleus and putamen. Predominantly bilateral, in up to 30% of cases the chorea can be unilaterally restricted. Imaging studies, both structural (magnetic resonance imaging) and functional (positron emission tomography), in patients with bilateral Sydenham's chorea have suggested reversible striatal abnormalities. Two patients with unilateral Sydenham's chorea are presented. Computed tomographic and magnetic resonance imaging were normal in both. However, hexamethylpropylenamine oxime single photon emission tomographic (HMPAO SPECT) studies demonstrated hypermetabolism in the contralateral basal ganglia. Resolution of symptoms in one of the patients coincided with normalization of the SPECT scan. Thus, unilateral striatal hypermetabolism appears to underlie the contralateral chorea observed. A SPECT scan probably should be included in the work-up of new-onset chorea.

Topics: Autoimmune Diseases; Caudate Nucleus; Child, Preschool; Chorea; Dominance, Cerebral; Energy Metabolism; Female; Humans; Male; Putamen; Streptococcal Infections; Streptococcus pyogenes; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

A case of paroxysmal kinesigenic choreoathetosis (PKC) was described. The patient had attacks of paroxysmal choreoathetotic movements lasting 20-30 seconds. The attacks were regularly precipitated by sudden and unintentional movements. There were no metabolic abnormalities. EEG showed no epileptiform discharges. The attacks were subsided after administration of carbamazepine. We studied this patient with single photon emission computed tomography (SPECT) using 99mTc-HMPAO. Ictal SPECT revealed decrease of cerebral blood flow in the basal ganglia on the contralateral side of choreothetotic movements. Although the pathophysiology of PKC is still uncertain, it is hypothesized that motor activities are influenced by the direct pathway (positive feedback) and the indirect pathway (negative feedback) from the basal ganglia to the motor cortecis. Dysfunction of negative feedback is considered to be common underlying mechanisms of hyperkinetic disorders. Our findings support this hypothesis. Dysfunction of basal ganglia is likely relevant to the genesis of choreoathetosis.

Topics: Adolescent; Athetosis; Basal Ganglia; Chorea; Humans; Male; Radiopharmaceuticals; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

Topics: Aged; Aged, 80 and over; Basal Ganglia; Cerebral Infarction; Chorea; Dominance, Cerebral; Humans; Male; Neurologic Examination; Organotechnetium Compounds; Oximes; Parietal Lobe; Regional Blood Flow; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

To study regional blood flow of the striatum in non-ketotic hyperglycaemic choreic patients.. Brain SPECT was performed with intravenous injection of 20 mCi 99mTc hexamethylpropylene amineoxime in six non-ketotic hyperglycaemic choreic patients and 10 age matched patients with a similar level of hyperglycaemia without chorea as a control. The focal perfusion defects were analysed by visual interpretation and semiquantitative determination with reference to homolateral occipital blood flow.. The measured blood flow of striatum on the contralateral side of chorea was significantly decreased.. Hypometabolism of the striatum is seen not only in Huntington's disease, but also in non-ketotic hyperglycaemic chorea. Hypofunction of the striatum is a possible common pathogenesis in the development of contralateral chorea in different diseases. Furthermore, the sensitivity and reliability of SPECT is as good as PET in assessing choreic patients.

Topics: Aged; Case-Control Studies; Cerebrovascular Circulation; Chorea; Corpus Striatum; Female; Humans; Hyperglycemic Hyperosmolar Nonketotic Coma; Male; Middle Aged; Organotechnetium Compounds; Oximes; Sensitivity and Specificity; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

Topics: Aged; Aged, 80 and over; Basal Ganglia Diseases; Blood Flow Velocity; Brain Mapping; Cerebral Infarction; Chorea; Corpus Striatum; Dominance, Cerebral; Humans; Male; Neural Inhibition; Neurons; Organotechnetium Compounds; Oximes; Regional Blood Flow; Technetium Tc 99m Exametazime; Thalamic Nuclei; Thalamus; Tomography, Emission-Computed, Single-Photon