Philips (Israel)

Theoretical considerations predicted the feasibility of K-edge x-ray computed tomography (CT) imaging using energy discriminating detectors with more than two energy bins. This technique enables material-specific imaging in CT, which in combination with high-Z element based contrast agents, opens up possibilities for new medical applications. In this paper, we present a CT system with energy detection capabilities, which was used to demonstrate the feasibility of quantitative K-edge CT imaging experimentally. A phantom was imaged containing PMMA, calcium-hydroxyapatite, water and two contrast agents based on iodine and gadolinium, respectively. Separate images of the attenuation by photoelectric absorption and Compton scattering were reconstructed from energy-resolved projection data using maximum-likelihood basis-component decomposition. The data analysis further enabled the display of images of the individual contrast agents and their concentrations, separated from the anatomical background. Measured concentrations of iodine and gadolinium were in good agreement with the actual concentrations. Prior to the tomographic measurements, the detector response functions for monochromatic illumination using synchrotron radiation were determined in the energy range 25 keV-60 keV. These data were used to calibrate the detector and derive a phenomenological model for the detector response and the energy bin sensitivities.

Automatic image processing methods are a prerequisite to efficiently analyze the large amount of image data produced by computed tomography (CT) scanners during cardiac exams. This paper introduces a model-based approach for the fully automatic segmentation of the whole heart (four chambers, myocardium, and great vessels) from 3-D CT images. Model adaptation is done by progressively increasing the degrees-of-freedom of the allowed deformations. This improves convergence as well as segmentation accuracy. The heart is first localized in the image using a 3-D implementation of the generalized Hough transform. Pose misalignment is corrected by matching the model to the image making use of a global similarity transformation. The complex initialization of the multicompartment mesh is then addressed by assigning an affine transformation to each anatomical region of the model. Finally, a deformable adaptation is performed to accurately match the boundaries of the patient's anatomy. A mean surface-to-surface error of 0.82 mm was measured in a leave-one-out quantitative validation carried out on 28 images. Moreover, the piecewise affine transformation introduced for mesh initialization and adaptation shows better interphase and interpatient shape variability characterization than commonly used principal component analysis.

Background Spatial resolution, soft-tissue contrast, and dose-efficient capabilities of photon-counting CT (PCCT) potentially allow a better quality and diagnostic confidence of coronary CT angiography (CCTA) in comparison to conventional CT. Purpose To compare the quality of CCTA scans obtained with a clinical prototype PCCT system and an energy-integrating detector (EID) dual-layer CT (DLCT) system. Materials and Methods In this prospective board-approved study with informed consent, participants with coronary artery disease underwent retrospective electrocardiographically gated CCTA with both systems after injection of 65–75 mL of 400 mg/mL iodinated contrast agent at 5 mL/sec. A prior phantom task-based quality assessment of the detectability index of coronary lesions was performed. Ultra-high-resolution parameters were used for PCCT (1024 matrix, 0.25-mm section thickness) and EID DLCT (512 matrix, 0.67-mm section thickness). Three cardiac radiologists independently performed a blinded analysis using a five-point quality score (1 = insufficient, 5 = excellent) for overall image quality, diagnostic confidence, and diagnostic quality of calcifications, stents, and noncalcified plaques. A logistic regression model, adjusted for radiologists, was used to evaluate the proportion of improvement in scores with the best method. Results Fourteen consecutive participants (12 men; mean age, 61 years ± 17) were enrolled. Scores of overall quality and diagnostic confidence were higher with PCCT images with a median of 5 (interquartile range [IQR], 2) and 5 (IQR, 1) versus 4 (IQR, 1) and 4 (IQR, 3) with EID DLCT images, using a mean tube current of 255 mAs ± 0 versus 349 mAs ± 111 for EID DLCT images (P < .01). Proportions of improvement with PCCT images for quality of calcification, stent, and noncalcified plaque were 100%, 92% (95% CI: 71, 98), and 45% (95% CI: 28, 63), respectively. In the phantom study, detectability indexes were 2.3-fold higher for lumen and 2.9-fold higher for noncalcified plaques with PCCT images. Conclusion Coronary CT angiography with a photon-counting CT system demonstrated in humans an improved image quality and diagnostic confidence compared with an energy-integrating dual-layer CT. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by Sandfort and Bluemke in this issue.

Previous positron emission tomography (PET) studies have shown that nonmanifesting carriers of the DYT1 dystonia mutation express an abnormal pattern of resting glucose metabolism. To determine whether motor behavior is impaired in these subjects, we compared movement and sequence learning in 12 clinically unaffected DYT1 carriers with 12 age-matched controls. Regional differences in brain function during task performance were assessed with simultaneous H(2) (15)O/PET. We found that motor performance was similar in the DYT1 and control groups, with no significant differences in movement time and spatial accuracy measured during each of the tasks. In contrast, sequence learning was reduced in gene carriers relative to controls (p < 0.01). PET imaging during motor execution showed increased activation in gene carriers (p < 0.001, uncorrected) in the left premotor cortex and right supplementary motor area, with concomitant reduction in the posterior medial cerebellum. During sequence learning, activation responses in DYT1 carriers were increased in the left ventral prefrontal cortex, and lateral cerebellum. These findings suggest that abnormalities in motor behavior and brain function exist in clinically nonmanifesting DYT1 carriers. Although localized increases in neural activity may enable normal movement execution in these subjects, this mechanism may not compensate for their defect in sequence learning.

Rationale Current hepatic locoregional therapies are limited in terms of effectiveness and toxicities. Given promising pre-clinical results, a first in-human trial was designed to assess the technical effectiveness and safety profile of histotripsy, a noninvasive, non-thermal, non-ionizing focused ultrasound therapy that creates precise, predictable tissue destruction, in patients with primary and secondary liver tumors.Methods A multicenter phase I trial (Theresa Study) was performed in a single country with 8 weeks of planned follow-up. Eight of fourteen recruited patients were deemed eligible and enrolled in the study. Hepatic histotripsy, was performed with a prototype system (HistoSonics, Inc., Ann Arbor, MI). Eleven tumors were targeted in the 8 patients who all had unresectable end-stage multifocal liver tumors: colorectal liver metastases (CRLM) in 5 patients (7 tumors), breast cancer metastases in 1 (1 tumor), cholangiocarcinoma metastases in 1 (2 tumors), and hepatocellular carcinoma (HCC) in 1 (1 tumor). The primary endpoint was acute technical success, defined as creating a zone of tissue destruction per planned volume assessed by MRI 1-day post-procedure. Safety (device-related adverse events) through 2 months was a secondary endpoint.Results The 8 patients had a median age of 60.4 years with an average targeted tumor diameter of 1.4 cm. The primary endpoint was achieved in all procedures. The secondary safety profile endpoint identified no device-related adverse events. Two patients experienced a continuous decline in tumor markers during the eight weeks following the procedure.Conclusions This first-in-human trial demonstrates that hepatic histotripsy effectively destroys liver tissue in a predictable manner, correlating very well with the planned histotripsy volume, and has a high safety profile without any device-related adverse events. Based on these results, the need for more definitive clinical trials is warranted. Trial Registration: Study to Evaluate VORTX Rx (Theresa). NCT03741088. https://clinicaltrials.gov/ct2/show/NCT03741088 KEY POINTSHistotripsy, a new noninvasive, non-thermal, non-ionizing focused ultrasound therapy, safely created a zone of tissue destruction in the liver that correlated very well with the pre-defined planned tissue destruction volume.In this first human trial histotripsy was well tolerated with no histotripsy device-related adverse events and its primary endpoint of acute technical success was achieved in all 8 enrolled patients with primary or secondary liver tumors.This new locoregional therapy for patients with liver tumors is safe and effective, warranting further trials.

Purpose This study aimed to quantitate the accuracy of the determination of electron density ( ED ), effective atomic number (Z eff ), and iodine concentration, directed for more accurate radiation therapy planning, with a new dual‐layer dual‐energy computed tomography ( DL ‐ DECT ) system. The dependence of the accuracy of these values on the scan and reconstruction parameters, as well as on the phantom size, was also examined. Methods Measurements were performed on a commercial DECT system with a DL detector ( IQ on Spectral CT , Philips Healthcare), using phantoms with various tissue‐equivalent inserts as well as iodine and calcium inserts of different concentrations. The expected values of ED and Z eff for the insert materials were derived from the chemical compositions provided by the vendors. The nominal scan condition for the accuracy measurements was 120 kV p, 20 mG y CTDI vol, 0.812 pitch, 16 × 0.625 mm collimation, and 0.33‐second gantry rotation. Results The median deviation of ED ranged from −0.1% to 1.1% for all Gammex tissue inserts. The median deviation of Z eff ranged from −2.3% to 1.7% for soft tissue and bone inserts and was ≤7% for lung inserts. The absolute deviations for ED and Z eff in lung inserts were within 1% of the ED of water and 1 a.u., respectively. For two different phantom sizes, the ED values agreed to within 0.7% and the Z eff values agreed to within 2%, except for the lung inserts. When the scan parameters were changed from 120 kV p/20 mG y to 140 kV p/30 mG y, the ED differed within [−0.51%, 0.65%] and the Z eff differed within [−1.1%, 0.23%] for all materials except lungs, in which Z eff increased by 2.4%. The accuracy of ED and Z eff measurement at 120 kV p was no worse than that at 140 kV p. For iodine quantitation, the median absolute deviations from the nominal values were up to 0.3 mg/ mL for iodine concentrations of 2–20 mg/ mL , with an overall median deviation of −0.1 mg/ mL . Iodine and calcium were well separated on the ED ‐Z eff scatter plot, even at the lowest concentrations (2 mg/ mL for iodine and 50 mg/ mL for calcium). Conclusions The accuracy of ED measurement, Z eff determination, and iodine quantitation derived from DL ‐ DECT was demonstrated with phantom measurements. The accuracies were not sensitive to scan and reconstruction parameters, namely tube potential, dose, rotation time, and spectral reconstruction level, especially in the case of electron density.

A new prototype spectral photon-counting computed tomography (SPCCT) based on a modified clinical CT system has been developed. SPCCT analysis of the energy composition of the transmitted x-ray spectrum potentially allows simultaneous dual contrast agent imaging, however, this has not yet been demonstrated with such a system. We investigated the feasibility of using this system to distinguish gold nanoparticles (AuNP) and an iodinated contrast agent. The contrast agents and calcium phosphate were imaged in phantoms. Conventional CT, gold K-edge, iodine and water images were produced and demonstrated accurate discrimination and quantification of gold and iodine concentrations in a phantom containing mixtures of the contrast agents. In vivo experiments were performed using New Zealand White rabbits at several times points after injections of AuNP and iodinated contrast agents. We found that the contrast material maps clearly differentiated the distributions of gold and iodine in the tissues allowing quantification of the contrast agents' concentrations, which matched their expected pharmacokinetics. Furthermore, rapid, repetitive scanning was done, which allowed measurement of contrast agent kinetics with high temporal resolution. In conclusion, a clinical scale, high count rate SPCCT system is able to discriminate gold and iodine contrast media in different organs in vivo.

Purpose To investigate the feasibility of using spectral photon-counting computed tomography (CT) to differentiate between gadolinium-based and nonionic iodine-based contrast material in a colon phantom by using the characteristic k edge of gadolinium. Materials and Methods A custom-made colon phantom was filled with nonionic iodine-based contrast material, and a gadolinium-filled capsule representing a contrast material–enhanced polyp was positioned on the colon wall. The colon phantom was scanned with a preclinical spectral photon-counting CT system to obtain spectral and conventional data. By fully using the multibin spectral information, material decomposition was performed to generate iodine and gadolinium maps. Quantitative measurements were performed within the lumen and polyp to quantitatively determine the absolute content of iodine and gadolinium. Results In a conventional CT section, absorption values of both contrast agents were similar at approximately 110 HU. Contrast material maps clearly differentiated the distributions, with gadolinium solely in the polyp and iodine in the lumen of the colon. Quantitative measurements of contrast material concentrations in the colon and polyp matched well with those of actual prepared mixtures. Conclusion Dual-contrast spectral photon-counting CT colonography with iodine-filled lumen and gadolinium-tagged polyps may enable ready differentiation between polyps and tagged fecal material. © RSNA, 2016

= 0.93). TEM results were in agreement with the imaging and ICP-OES in that much higher concentrations of AuNPs were observed in the liver, spleen, bone marrow and lymph nodes (mainly in macrophages). In conclusion, we found that SPCCT can be used for repetitive and non-invasive determination of the biodistribution of gold nanoparticles in vivo.

The DYT1 dystonia mutation is associated with an abnormal metabolic brain network characterized by hypermetabolism of the basal ganglia, supplementary motor area, and the cerebellum. In this study, we quantified the activity of this network in carriers of other dystonia mutations to determine whether this functional abnormality is linked to genotype. The findings suggest that the DYT1 metabolic topography is not genotype specific and may be present in carriers of other dystonia mutations.

OBJECTIVES: The aim of this study is to compare the image quality of in vivo coronary stents between an energy integrating detectors dual-layer computed tomography (EID-DLCT) and a clinical prototype of spectral photon counting computed tomography (SPCCT). MATERIALS AND METHODS: In January to June 2021, consecutive patients with coronary stents were prospectively enrolled to undergo a coronary computed tomography (CT) with an EID-DLCT (IQon, Philips) and an SPCCT (Philips). The study was approved by the local ethical committee and patients signed an informed consent. A retrospectively electrocardiogram-gated acquisition was performed with optimized matching parameters on the 2 scanners (EID-DLCT: collimation, 64 × 0.625 mm; kVp, 120, automatic exposure control with target current at 255 mAs; rotation time, 0.27 seconds; SPCCT: collimation, 64 × 0.275 mm; kVp, 120; mAs, 255; rotation time, 0.33 seconds). The injection protocol was the same on both scanners: 65 to 75 mL of Iomeron (Bracco) at 5 mL/s. Images were reconstructed with slice thickness of 0.67 mm, 512 matrix, XCB (Xres cardiac standard) and XCD (Xres cardiac detailed) kernel, iDose 3 for EID-DLCT and 0.25-mm slice thickness, 1024 matrix, Detailed 2 and Sharp kernel, and iDose 6 for SPCCT. Two experienced observers measured the proximal and distal external and internal diameters of the stents to quantify blooming artifacts. Regions of interest were drawn in the lumen of the stent and of the upstream coronary artery. The difference (Δ S-C) between the respective attenuation values was calculated as a quantification of stent-induced artifacts on intrastent image quality. For subjective image quality, 3 experienced observers graded with a 4-point scale the image quality of different parameters: coronary wall before the stent, stent lumen, stent structure, calcifications surrounding the stent, and beam-hardening artifacts. RESULTS: Eight patients (age, 68 years [interquartile range, 8]; all men; body mass index, 26.2 kg/m2 [interquartile range, 4.2]) with 16 stents were scanned. Five stents were not evaluable owing to motion artifacts on the SPCCT. Of the remaining, all were drug eluting stents, of which 6 were platinum-chromium, 3 were cobalt-platinum-iridium, and 1 was stainless steel. For 1 stent, no information could be retrieved. Radiation dose was lower with the SPCCT (fixed CT dose index of 25.7 mGy for SPCCT vs median CT dose index of 35.7 [IQ = 13.6] mGy; P = 0.02). For 1 stent, the internal diameter was not assessable on EID-DLCT. External diameters were smaller and internal diameters were larger with SPCCT (all P < 0.05). Consequently, blooming artifacts were reduced on SPCCT (P < 0.05). Whereas Hounsfield unit values within the coronary arteries on the 2 scanners were similar, the Δ S-C was lower for SPCCT-Sharp as compared with EID-DLCT-XCD and SPCCT-Detailed 2 (P < 0.05). The SPCCT received higher subjective scores than EID-DLCT for stent lumen, stent structure, surrounding calcifications and beam-hardening for both Detailed 2 and Sharp (all P ≤ 0.05). The SPCCT-Sharp was judged better for stent structure and beam-hardening assessment as compared with SPCCT-Detailed 2. CONCLUSION: Spectral photon counting CT demonstrated improved objective and subjective image quality as compared with EID-DLCT for the evaluation of coronary stents even with a reduced radiation dose.

Spectral photon-counting computed tomography (SPCCT) is a rapidly emerging imaging modality that provides energy-dependent information on individual x-ray photons, leading to accurate material decomposition and simultaneous quantification of multiple contrast generating materials. Development of SPCCT-specific contrast agents is needed to overcome the issues with currently used iodinated contrast agents, such as difficulty in differentiation from calcified structures, and yield SPCCT's full promise. In this study, the contrast generation of different elements is investigated using a prototype SPCCT scanner based on a modified clinical CT system and suitable elements for novel contrast agent development for SPCCT imaging are identified. Furthermore, nanoparticles were synthesized from tantalum as a proof of concept spectral photon-counting CT agent and tested for their in vitro cytotoxicity and contrast generation to provide insight into the feasibility of nanoparticle contrast agent development from these elements. We found that gadolinium, ytterbium and tantalum generate high contrast in spectral photon-counting CT imaging and may be suitable elements for contrast agent development for this modality. Our proof of concept results with tantalum-based nanoparticles underscore this conclusion due to their detectability with spectral photon-counting CT, as well as their biocompatibility.

Dual-energy CT is known to enable possible improvement of material separation over regular CT. However, in clinical implementation most of the dual-energy techniques show limited results mainly due to their sensitivity to noisy data. We simulate data acquisition by a dual-layer CT based on two scintillation layers one on top of the other with which the data is acquired simultaneously. We map the results of the reconstruction into a plane created from the Hounsfield units (HU) of the upper-layer image versus the HU of the lower-layer image. We find that different scanned materials end up in different definable regions in the HU-plane. Application of a special correction on the reconstructed images achieves stability on the HU-plane despite beam-hardening effects. In order to assess the practical material separation capabilities, part of the simulations were done with exact noise calculations. We analyze the material separation capabilities with such a configuration and conclude that the combination of the dual-layer CT with the classification analysis in the HU-plane is a practical and robust method that significantly improves clinical applications, in particular those involving iodine-calcium separation such as analysis and classification of coronary artery calcifications and soft plaques.

Cone beam computed tomography scanners in combination with heart rate adaptive reconstruction schemes have the potential to enable cardiac volumetric computed tomography (CT) imaging for a larger number of patients and applications. In this publication, an adaptive scheme for the automatic and patient-specific reconstruction optimization is introduced to improve the temporal resolution and image quality. The optimization method permits the automatic determination of the required amount of gated helical cone beam projection data for the reconstruction volume. It furthermore allows one to optimize subvolume reconstruction yielding an increased temporal resolution. In addition, methods for the assessment of the temporal resolution are given which enable a quantitative documentation of the reconstruction improvements. Results are presented for patient data sets acquired in low pitch helical mode using a 16-slice cone beam CT system with parallel ECG recording.

In modern computer tomography (CT) systems, the fast rotating gantry and the increased detector width enable 3D imaging of the heart. Cardiac volume CT has a high potential for non-invasive coronary angiography with high spatial resolution and short scan time. Due to the increased detector width, true cone beam reconstruction methods are needed instead of adapted 2D reconstruction schemes. In this paper, the extended cardiac reconstruction method is introduced. It integrates the idea of retrospectively gated cardiac reconstruction for helical data acquisition into a cone beam reconstruction framework. It leads to an efficient and flexible algorithmic scheme for the reconstruction of single- and multi-phase cardiac volume datasets. The method automatically adapts the number of cardiac cycles used for the reconstruction. The cone beam geometry is fully taken into account during the reconstruction process. Within this paper, results are presented on patient datasets which have been acquired using a 16-slice cone beam CT system.

To evaluate the feasibility of multicolour quantitative imaging with spectral photon-counting computed tomography (SPCCT) of different mixed contrast agents. Phantoms containing eleven tubes with mixtures of varying proportions of two contrast agents (i.e. two selected from gadolinium, iodine or gold nanoparticles) were prepared so that the attenuation of each tube was about 280 HU. Scans were acquired at 120 kVp and 100 mAs using a five-bin preclinical SPCCT prototype, generating conventional, water, iodine, gadolinium and gold images. The correlation between prepared and measured concentrations was assessed using linear regression. The cross-contamination was measured for each material as the root mean square error (RMSE) of its concentration in the other material images, where no signal was expected. The contrast-to-noise ratio (CNR) relative to a phosphate buffered saline tube was calculated for each contrast agent. The solutions had similar attenuations (279 ± 10 HU, mean ± standard deviation) and could not be differentiated on conventional images. However, a distinction was observed in the material images within the same samples, and the measured and prepared concentrations were strongly correlated (R2 ≥ 0.97, 0.81 ≤ slope ≤ 0.95, -0.68 ≤ offset ≤ 0.89 mg/mL). Cross-contamination in the iodine images for the mixture of gold and gadolinium contrast agents (RMSE = 0.34 mg/mL) was observed. CNR for 1 mg/mL of contrast agent was better for the mixture of iodine and gadolinium (CNRiodine = 3.20, CNRgadolinium = 2.80) than gold and gadolinium (CNRgadolinium = 1.67, CNRgold = 1.37). SPCCT enables multicolour quantitative imaging. As a result, it should be possible to perform imaging of multiple uptake phases of a given tissue/organ within a single scan by injecting different contrast agents sequentially.

Diagnostic imaging of hepatocellular carcinoma (HCC) requires a liver CT or MRI multiphase acquisition protocol. Patients would benefit from a high-resolution imaging method capable of performing multi-phase imaging in a single acquisition without an increase in radiation dose. Spectral Photon-Counting Computed Tomography (SPCCT) has recently emerged as a novel and promising imaging modality in the field of diagnostic radiology. SPCCT is able to distinguish between two contrast agents referred to as multicolor imaging because, when measuring in three or more energy regimes, it can detect and quantify elements with a K-edge in the diagnostic energy range. Based on this capability, we tested the feasibility of a dual-contrast multi-phase liver imaging protocol via the use of iodinated and gadolinated contrast agents on four healthy New Zealand White (NZW) rabbits. To perform a dual-contrast protocol, we injected the agents at different times so that the first contrast agent visualized the portal phase and the second the arterial phase, both of which are mandatory for liver lesion characterization. We demonstrated a sensitive discrimination and quantification of gadolinium within the arteries and iodine within the liver parenchyma. In the hepatic artery, the concentration of gadolinium was much higher than iodine (8.5 ± 3.9 mg/mL versus 0.7 ± 0.1 mg/mL) contrary to the concentrations found in the liver parenchyma (0.5 ± 0.3 mg/mL versus 4.2 ± 0.3 mg/mL). In conclusion, our results confirm that SPCCT allows in-vivo dual contrast qualitative and quantitative multi-phase liver imaging in a single acquisition.

Dual-energy information in computed tomography can be obtained through different technical approaches. Most available scanner designs acquire examination with two different X-ray spectra. Recently, the first detector-based approach became clinically available. Upfront, physical principles of dual-energy CT are reviewed, including the interaction of photons with matter in terms of the Photoelectric effect and Compton scattering. In addition, available concepts to dual energy computed tomography are described. Afterwards, the spectral detector CT system is described in detail. The design of the of the stacked detector design and its inherent technical advantages and disadvantages are discussed. Further, the principles of image reconstruction, their possibilities and limitations are referred. The increase in reconstructions and data pose some challenges to both, clinical and technological workflow which are hereafter addressed. Finally, the detector-based approach is discussed in light of other, emission-based DECT approaches.

Correct visualization of the vascular lumen is impaired in standard computed tomography (CT) because of blooming artifacts, increase of apparent size, induced by metallic stents and vascular calcifications. Recently, due to the introduction of photon-counting detectors in the X-ray imaging field, a new prototype spectral photon-counting CT (SPCCT) based on a modified clinical CT system has been tested in a feasibility study for improving vascular lumen delineation and visualization of coronary stent architecture. Coronary stents of different metal composition were deployed inside plastic tubes containing hydroxyapatite spheres to simulate vascular calcifications and in the abdominal aorta of one New Zealand White (NZW) rabbit. Imaging was performed with an SPCCT prototype, a dual-energy CT system, and a conventional 64-channel CT system (B64). We found the apparent widths of the stents significantly smaller on SPCCT than on the other two systems in vitro (p < 0.01), thus closer to the true size. Consequently, the intra-stent lumen was significantly larger on SPCCT (p < 0.01). In conclusion, owing to the increased spatial resolution of SPCCT, improved lumen visualization and delineation of stent metallic mesh is possible compared to dual-energy and conventional CT.

Abstract We used [ 18 F]‐fluorodeoxyglucose and positron emission tomography to determine a discrete cerebral pattern of abnormal glucose utilization in dopa‐responsive dystonia. Network analysis demonstrated that dopa‐responsive dystonia is associated with a specific pattern of regional metabolic covariation, characterized by increases in the dorsal midbrain, cerebellum, and supplementary motor area, as well as reductions in motor and lateral premotor cortex and in the basal ganglia. This pattern was not expressed in mutation carriers for primary torsion dystonia. Dopa‐responsive dystonia has a unique metabolic architecture that differs from other inherited forms of dystonia. Ann Neurol 2005;57:596–600