A novel case of concurrent occurrence of demyelinating-polyneuropathy-causing PMP22 duplication and SOX10 gene mutation producing severe hypertrophic neuropathy.

BACKGROUND: Hereditary motor and sensory neuropathy, also referred to as Charcot-Marie-Tooth disease (CMT), is most often caused by a duplication of the peripheral myelin protein 22 (PMP22) gene. This duplication causes CMT type 1A (CMT1A). CMT1A rarely occurs in combination with other hereditary neuromuscular disorders. However, such rare genetic coincidences produce a severe phenotype and have been reported in terms of "double trouble" overlapping syndrome. Waardenburg syndrome (WS) is the most common form of a hereditary syndromic deafness. It is primarily characterized by pigmentation anomalies and classified into four major phenotypes. A mutation in the SRY sex determining region Y-box 10 (SOX10) gene causes WS type 2 or 4 and peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, WS, and Hirschsprung disease. We describe a 11-year-old boy with extreme hypertrophic neuropathy because of a combination of CMT1A and WS type 2. This is the first published case on the co-occurrence of CMT1A and WS type 2. CASE PRESENTATION: The 11-year-old boy presented with motor developmental delay and a deterioration in unstable walking at 6 years of age. In addition, he had congenital hearing loss and heterochromia iridis. The neurological examination revealed weakness in the distal limbs with pes cavus. He was diagnosed with CMT1A by the fluorescence in situ hybridization method. His paternal pedigree had a history of CMT1A. However, no family member had congenital hearing loss. His clinical manifestation was apparently severe than those of his relatives with CMT1A. In addition, a whole-body magnetic resonance neurography revealed an extreme enlargement of his systemic cranial and spinal nerves. Subsequently, a genetic analysis revealed a heterozygous frameshift mutation c.876delT (p.F292Lfs*19) in the SOX10 gene. He was eventually diagnosed with WS type 2. CONCLUSIONS: We described a patient with a genetically confirmed overlapping diagnoses of CMT1A and WS type 2. The double trouble with the genes created a significant impact on the peripheral nerves system. Severe phenotype in the proband can be attributed to the cumulative effect of mutations in both PMP22 and SOX10 genes, responsible for demyelinating neuropathy.

The Motor Network Reduces Multisensory Illusory Perception.

Observing mouth movements has strikingly effects on the perception of speech. Any mismatch between sound and mouth movements will result in listeners perceiving illusory consonants (McGurk effect), whereas matching mouth movements assist with the correct recognition of speech sounds. Recent neuroimaging studies have yielded evidence that the motor areas are involved in speech processing, yet their contributions to multisensory illusion remain unclear. Using functional magnetic resonance imaging (fMRI) and transcranial magnetic stimulation (TMS) in an event-related design, we aimed to identify the functional roles of the motor network in the occurrence of multisensory illusion in female and male brains. fMRI showed bilateral activation of the inferior frontal gyrus (IFG) in audiovisually incongruent trials. Activity in the left IFG was negatively correlated with occurrence of the McGurk effect. The effective connectivity between the left IFG and the bilateral precentral gyri was stronger in incongruent than in congruent trials. The McGurk effect was reduced in incongruent trials by applying single-pulse TMS to motor cortex (M1) lip areas, indicating that TMS facilitates the left IFG-precentral motor network to reduce the McGurk effect. TMS of the M1 lip areas was effective in reducing the McGurk effect within the specific temporal range from 100 ms before to 200 ms after the auditory onset, and TMS of the M1 foot area did not influence the McGurk effect, suggesting topographical specificity. These results provide direct evidence that the motor network makes specific temporal and topographical contributions to the processing of multisensory integration of speech to avoid illusion.SIGNIFICANCE STATEMENT The human motor network, including the inferior frontal gyrus and primary motor cortex lip area, appears to be involved in speech perception, but the functional contribution to the McGurk effect is unknown. Functional magnetic resonance imaging revealed that activity in these areas of the motor network increased when the audiovisual stimuli were incongruent, and that the increased activity was negatively correlated with perception of the McGurk effect. Furthermore, applying transcranial magnetic stimulation to the motor areas reduced the McGurk effect. These two observations provide evidence that the motor network contributes to the avoidance of multisensory illusory perception.

Detectability of Malignant Lesions by Whole-Body Magnetic Resonance Imaging Using Whole-Body Integrated Positron Emission Tomography/Magnetic Resonance Imaging.

PURPOSE: To assess the diagnostic ability of whole-body magnetic resonance imaging (MRI) using integrated positron emission tomography/MRI(PET/MRI). METHODS: Axial T2-weighted image (T2WI), diffusion-weighted imaging (DWI), coronal T1-weighted image (T1WI), axial volumetric interpolated breath-hold examination in the lung field, and 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG-PET) were evaluated in combination with T2WI alone, T2WI + DWI, T2WI + DWI + T1WI, T2WI + DWI + T1WI + volumetric interpolated breath-hold examination (all MRI images), and all MRI + FDG-PET. RESULTS: A total of 370 lesions were observed in 90 (62.5%) of the 144 patients. The lesion-based sensitivities were 62%, 74%, 74%, 76%, and 94%, and the patient-based sensitivities were 70%, 77%, 77%, 77%, and 81% using T2WI, T2WI + DWI, T2WI + DWI + T1WI, all MRI, and all MRI + FDG-PET, respectively. There were significant differences in the lesion-based sensitivity between T2WI and other sequence combinations and between all MRI and all MRI + FDG-PET. No significant differences were observed between any combinations among the patient-based sensitivities. CONCLUSION: The sensitivity of whole-body MRI was lower when lesion based, but almost equivalent when patient based compared with PET/MRI.

A Case of a Cardiac Resynchronization Therapy-Defibrillator Exhibiting a Lower and Alternately Variable Basic Rate.

A cardiac resynchronization therapy defibrillator (CRT-D) (Medtronic Inc. Protecta XT) was implanted in a 67-year-old man who had cardiac sarcoidosis with extremely low cardiac function. He had ventricular tachycardia which was controlled by catheter ablation, medication and pacing. The programmed mode was DDI, lower rate was 90 beats/minute, paced AV delay was 150 ms, and the noncompetitive atrial pacing (NCAP) function was programmed as 300 ms.After his admission for pneumonia and heart failure, we changed his DDI mode to a DDD mode because he had atrial tachycardia, which led to inadequate bi-ventricular pacing. After a while, there were cycle lengths which were longer than his device setting and alternately varied. We were able to avoid this phenomenon with AV delay of 120 ms and NCAP of 200 ms.NCAP is an algorithm which creates a gap above a certain period after the detection of an atrial signal during the postventricular atrial refractory period of the pacemaker. This is to prevent atrial tachycardia and repetitive non-reentrant ventriculoatrial (VA) synchrony in the presence of retrograde VA conduction. But in this case, NCAP algorithm induced much lower rate than the programmed basic lower rate. This situation produced some arrhythmias and exacerbated symptoms of heart failure. This had to be paid attention to, especially when the device was programmed at high basic heart rate.

[Introduction and Applications of 3D Printing in Radiological Technology].

A 3D printing emerges as a common procedure in clinical radiology practice after installation of a module that converts the digital imaging and communications in medicine (DICOM) dataset into stereolithography (STL) data on medical workstations. However, they did not conventionally provide the appropriate filtering, sculpting, hollowing out, and Boolean (subtraction) operations on STL data. These functions are indispensable to handle the STL data to fabricate the smooth, low-cost, and sophisticated models. Here are some tips for handling the 3D data with three software packages through making a sample lumbar spine model. Because they are all free- and open-source software with the exception of Boolean operations, they could make it easy for anyone to fabricate their 3D model imaged by CT or MRI. We tested the loop subdivision surface algorithms for the smoothing, the sculpting function for removing a sharp prick, and the hollowing function to save the cost. Computer-aided design (CAD) is also used to fabricate the devices in medical research. We designed and developed a cap attached to a glass dosimeter to show the effectiveness of CAD in radiological research. Lastly, we discuss the important matters for 3D printing and examples of the clinical applications.

Normative data of dopaminergic neurotransmission functions in substantia nigra measured with MRI and PET: Neuromelanin, dopamine synthesis, dopamine transporters, and dopamine D2 receptors.

The central dopaminergic system is of major importance in the pathophysiology of Parkinson's disease, schizophrenia, and other neuropsychiatric disorders. In the present study, the normative data of dopaminergic neurotransmission functions in the midbrain, consisting of neuromelanin, dopamine synthesis, dopamine transporters and dopamine D2 receptors, were constructed using magnetic resonance (MR) imaging and positron emission tomography (PET). PET studies with L-[ß-11C]DOPA, [18F]FE-PE2I and [11C]FLB457 and MRI studies were performed on healthy young men. Neuromelanin accumulation measured by MRI was compared with dopaminergic functions, dopamine synthesis capacity, dopamine transporter binding and dopamine D2 receptor binding measured by PET in the substantia nigra. Although neuromelanin is synthesized from DOPA and dopamine in dopaminergic neurons, neuromelanin accumulation did not correlate with dopamine synthesis capacity in young healthy subjects. The role of dopamine transporters in the substantia nigra is considered to be the transport of dopamine into neurons, and therefore dopamine transporter binding might be related to neuromelanin accumulation; however, no significant correlation was observed between them. A positive correlation between dopamine D2 receptor binding and neuromelanin accumulation was observed, indicating a feedback mechanism by dopaminergic autoreceptors. Discrepancies in regional distribution between neuromelanin accumulation and dopamine synthesis capacity, dopamine transporter binding or dopamine D2 receptor binding were observed in the substantia nigra.

Effects of dietary phosphate on glucose and lipid metabolism.

Recent epidemiological and animal studies have suggested that excess intake of phosphate (Pi) is a risk factor for the progression of chronic kidney disease and its cardiovascular complications. However, little is known about the impact of dietary high Pi intake on the development of metabolic disorders such as obesity and type 2 diabetes. In this study, we investigated the effects of dietary Pi on glucose and lipid metabolism in healthy rats. Male 8-wk-old Sprague-Dawley rats were divided into three groups and given experimental diets containing varying amounts of Pi, i.e., 0.2 [low Pi(LP)], 0.6 [control Pi(CP)], and 1.2% [high Pi(HP)]. After 4 wk, the HP group showed lower visceral fat accumulation compared with other groups, accompanied by a low respiratory exchange ratio (V̇CO2/V̇O2) without alteration of locomotive activity. The HP group had lower levels of plasma insulin and nonesterified fatty acids. In addition, the HP group also showed suppressed expression of hepatic lipogenic genes, including sterol regulatory element-binding protein-1c, fatty acid synthase, and acetyl-CoA carboxylase, whereas there was no difference in hepatic fat oxidation among the groups. On the other hand, uncoupling protein (UCP) 1 and peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) expression were significantly increased in the brown adipose tissue (BAT) of the HP group. Our data demonstrated that a high-Pi diet can negatively regulate lipid synthesis in the liver and increase mRNA expression related to lipid oxidation and UCP1 in BAT, thereby preventing visceral fat accumulation. Thus, dietary Pi is a novel metabolic regulator.

[Temporary use of centrifugal pump for pump thrombosis in patients with paracorporeal ventricular assist device].

Nipro paracorporeal ventricular assist device( VAD) is often associated with pump thrombosis which causes severe complications such as brain infarction, often requiring pump change. However, Nipro VAD pump is an expensive device and it is difficult to change pumps frequently at a short interval. We have temporarily used Rotaflow centrifugal pump for recurrent pump thrombosis in patients with Nipro VADs. From January 2012 through December 2013, 19 patients underwent Nipro VADs implantation at our institution, and 9 of them underwent pump change from Nipro pumps to Rotaflow centrifugal pumps. A total of 25 Rotaflow centrifugal pumps were used in these 9 patients, with the total circulatory support duration of 526 days. The median support period was 15 days (range;2-128 days). There were 2 cerebrovascular accidents and 1 Rotaflow pump circuit thrombosis during this period. Change from Rotaflow to Nipro VAD pump resulted in decrease in hematocrit by about 3 point. There was no difference in liver or renal function between before and after the pump change. Our results suggest that temporary use of Rotaflow centrifugal pump for recurrent pump thrombosis in patients with Nipro VADs may be a promising alternative.

Agmatine attenuates methamphetamine-induced hyperlocomotion and stereotyped behavior in mice.

We investigated whether pretreatment with the neurotransmitter/neuromodulator agmatine (decarboxylated L-arginine) affected methamphetamine (METH)-induced hyperlocomotion and stereotypy in male ICR mice. Agmatine pretreatment alone had no effects on locomotion or stereotypy, but it produced a dose-dependent attenuation of locomotion and the total incidence of stereotyped behavior induced by a low dose of METH (5 mg/kg). The stereotypy induced by this dose was predominantly characterized by stereotyped sniffing. By contrast, agmatine did not affect the total incidence of stereotypy induced by a higher dose of METH (10 mg/kg). However, the nature of stereotypy induced by this dose of METH was substantially altered; agmatine pretreatment significantly reduced stereotyped biting but significantly increased stereotyped sniffing and persistent locomotion. Agmatine pretreatment therefore appears to produce a rightward shift in the dose-response curve for METH. Pretreatment of mice with piperazine-1-carboxamidine (a putative agmatinase inhibitor) had no effect on locomotion or stereotypy induced by a low dose of METH, suggesting that endogenous agmatine may not regulate the METH action.

[Evaluation of optimal parameters for non-contrast-enhanced non-breath-holding pulmonary artery MRA using 3D-FSE imaging with variable flip angle echo trains].

PURPOSE: The aim of this study was to find the optimal acquisition parameters of non-contrast-enhanced non-breath-holding pulmonary artery MRA using 3D-FSE imaging with variable flip angle echo trains. MATERIALS AND METHODS: The 3D-FSE imaging method with variable flip angle echo trains (CUBE) was employed in this study. Pulmonary artery MRA was performed in five healthy volunteers using a 1.5 tesla (T) and a 3 T clinical scanner with multi-channel torso coils. The institutional review boards approved the study, and informed consent was obtained from all subjects. Prior to the CUBE studies, ECG-gated single-shot FSE scans were performed to determine the timing of systole and diastole. After that, CUBE scans with systolic timing and three adjusted (early, middle and delayed) diastolic timings using both ECG and respiratory gating were performed and subtracted images between systolic and diastolic images were calculated. Subtracted intensities of both lung parenchyma and pulmonary arteries were evaluated using the region of interest (ROI) function. Maximum intensity projection (MIP) images with six different scan parameters (three timings and two static magnetic fields) were processed for evaluation by the ranking method with visual assessment. Three observers each scored all six images and a statistical analysis based on the variation of ratings was performed. RESULTS: The subtracted intensities of pulmonary arteries and lung parenchyma with middle diastolic timing were higher than that with both early and delayed systolic timing. The same tendency was shown in both 1.5 T and 3 T images. Though the subtracted intensity of 3 T was higher than that of 1.5 T, the contrast ratio between lung parenchyma and pulmonary artery of 1.5 T was higher than that of 3 T. The MIP image using the 1.5 T scanner with middle diastolic timing obtained the best score by the visual assessment using the ranking methods. The middle diastolic timing using the 1.5 T scanner provides the best non-contrast-enhanced non-breath-holding pulmonary artery MRA.

[A case of suspected IgG4-related hypophysitis presented with panhypopituitarism and central diabetes insipidus].

A 78-year-old man complained of subacute general fatigue and anorexia, following diplopia and gait disturbance. He demonstrated wide-based and small-stepped gait without objectively abnormal ocular movements. Brain |MRI showed enlargement of the pituitary stalk and gland with uniform contrast enhancement. PET-CT showed FDG |uptake in the pituitary gland, mediastinal lymph nodes, and left hilar lymph nodes. Blood investigations revealed panhypopituitarism and high serum IgG4 levels up to 265 |mg/dl. Histopathological examination revealed no IgG4-positive cell infiltration in the biopsied mediastinal lymph nodes. However, we suspected IgG4-associated hypophysitis based on the clinical symptoms and MRI findings, which were markedly resolved with steroid. Central masked diabetes insipidus was manifested, but was improved with oral desmopressin. We should pay close attention to the fact that IgG4-related hypophysitis may present with various symptoms regarded as indefinite complaints related to aging or underlying diseases, especially in elderly patients with multimorbidity.

Abnormal motor cortical plasticity as a useful neurophysiological biomarker for Alzheimer's disease pathology.

OBJECTIVE: Amyloid-beta (Aß) and tau accumulations impair long-term potentiation (LTP) induction in animal hippocampi. We investigated relationships between motor-cortical plasticity and biomarkers for Alzheimer's disease (AD) diagnosis in subjects with cognitive decline. METHODS: Twenty-six consecutive subjects who complained of memory problems participated in this study. We applied transcranial quadripuse stimulation with an interstimulus interval of 5 ms (QPS5) to induce LTP-like plasticity. Motor-evoked potentials were recorded from the right first-dorsal interosseous muscle before and after QPS5. Cognitive functions, Aß42 and tau levels in the cerebrospinal fluid (CSF) were measured. Amyloid positron-emission tomography (PET) with11C-Pittsburg compound-B was also conducted. We studied correlations of QPS5-induced plasticity with cognitive functions or AD-related biomarkers. RESULTS: QPS5-induced LTP-like plasticity positively correlated with cognitive scores. The degree of LTP-like plasticity negatively correlated with levels of CSF-tau, and the amount of amyloid-PET accumulation at the precuneus, and correlated with the CSF-Aß42 level positively. In the amyloid-PET positive subjects, non-responder rate of QPS5 was higher than the CSF-tau positive rate. CONCLUSIONS: Findings suggest that QPS5-induced LTP-like plasticity is a functional biomarker of AD. QPS5 could detect abnormality at earlier stages than CSF-tau in the amyloid-PET positive subjects. SIGNIFICANCE: Assessing motor-cortical plasticity could be a useful neurophysiological biomarker for AD pathology.

[Mid-long term result of implantable ventricular assist device treatment for end-stage heart failure in the University of Tokyo Hospital].

Two implantable left ventricular assist devices(I-LVADs)were approved in Japan in 2011. I-LVADs were used for end-stage heart failure of 20 patients in our institute until March 2012. We examine the clinical course of these 20 patients who were treated with I-LVADs. End points are transplantation, death, or weaning from I-LVADs. Mean hospital days after I-LVAD implantation were 58.1 days, and all 20 patients could discharge from hospital alive. Until September 2012, mean follow up interval was 515 days. Five patients reached heart transplantation, 2 died, weaning from an I-LVAD could be done in 1 patient, and 12 continued to wait for heart transplantation. Survival rate was 100% at 1 month, 95% at 3 months, and 89% at 1 year, respectively. Mean follow up duration after discharge were 457 days, and 16 patients(80%)needed a total of 41 times of readmission in this period. Freedom from readmission was 75% at 1 month, 60% at 3 months, and 25% at 1 year, respectively. In conclusion, prognosis of I-LVAD treatment was good, but many patients needed readmission after I-LVAD implantation and follow up system for I-LVAD treatment should be improved immediately.

Oxygen extraction fraction is not uniform in human brain: a positron emission tomography study.

The regional differences in cerebral oxygen extraction fraction (OEF) in brain were investigated using positron emission tomography (PET) in detail with consideration of systemic errors in PET measurement estimated by simulation studies. The cerebral blood flow (CBF), cerebral blood volume (CBV), OEF, and cerebral metabolic rate of oxygen (CMRO2) were measured on healthy men by PET with 15O-labeled gases. The OEF values in the pons and the parahippocampal gyrus were significantly smaller than in the other brain regions. The OEF value in the lateral side of the occipital cortex was largest among the cerebral cortical regions. Simulation studies have revealed that errors in OEF caused by regional differences in the distribution volume of 15O-labeled water, as well as errors in OEF caused by a mixture of gray and white matter, must be negligible. The regional differences in OEF in brain must exist which might be related to physiological meanings.Article title: Kindly check and confirm the edit made in the article title.I have checked the article title and it is OK as is. Trial registration: The UMIN clinical trial number: UMIN000033382, https://www.umin.ac.jp/ctr/index.htm.

Performance Characteristics of a New-Generation Digital Bismuth Germanium Oxide PET/CT System, Omni Legend 32, According to NEMA NU 2-2018 Standards.

The Omni Legend 32 PET/CT system features silicon photomultiplier (SiPM)-based detectors with bismuth germanium oxide crystals and a 32-cm axial field of view (FOV). The present study aimed to determine the performance characteristics of the Omni Legend 32 PET/CT system according to National Electrical Manufacturers Association (NEMA) NU 2-2018 standards. Methods: The PET component of this system comprises 22 detector modules; each module contains 24 detector blocks with 72 bismuth germanium oxide crystals with a volume of 4.1 × 4.1 × 30 mm coupled to 18 SiPM devices with a 6 × 6 mm area, resulting in an axial FOV of 32 cm. The spatial resolution, sensitivity, count rate performance, and image quality delivered by PET were evaluated using the NEMA NU 2-2018 standard. PET images of 2 patients were evaluated to get a visual first impression of the Omni Legend 32 PET/CT system together with Precision DL. Results: The average spatial resolution at 1, 10, and 20 cm from the central axis was 4.3, 5.3, and 6.2 mm, respectively, for filtered backprojection and 3.7, 4.3, and 5.1 mm, respectively, for ordered-subset expectation maximization. The NEMA sensitivity was 47.30 and 47.05 cps/kBq at the axial center of the FOV and at a 10-cm radial offset, respectively. The scatter fraction, count rate accuracy, and peak noise-equivalent count rates were 35.4%, 1.7%, and 501.7 kcps, respectively, at 15.7 kBq/mL. Contrast recovery for the NEMA body phantom from the smallest to the largest sphere ranged from 61.3% to 93.0%, with a background variability of 5.4%-11.7% and a lung error of 5.1% for Q.Clear (ß-value, 50). Good patient image quality was obtained with the Omni Legend 32. Conclusion: The Omni Legend 32 has class-leading sensitivity and count rates within the category of whole-body PET systems while maintaining spatial resolution broadly comparable to that of other current SiPM-based PET/CT systems. This combination of properties results in a very good image quality.

Left heart bypass support with the Rotaflow Centrifugal Pump® as a bridge to decision and recovery in an adult.

Since left heart bypass or biventricular circulatory assist with an extracorporeal centrifugal pump as a bridge to decision or recovery sometimes requires long-time support, the long-term durability of extracorporeal centrifugal pumps is crucial. The Rotaflow Centrifugal Pump(®) (MAQUET Cardiopulmonary AG, Hirrlingen, Germany) is one of the centrifugal pumps available for long-term use in Japan. However, there have been few reports of left heart bypass or biventricular circulatory support over the mid-term. This is a case report of left heart bypass support with the Rotaflow Centrifugal Pump(®) as a bridge to decision and recovery for an adult patient who could not be weaned from cardiopulmonary bypass and percutaneous cardiopulmonary support after cardiac surgery. We could confirm that the patient's consciousness level was normal; however, the patient could not be weaned from the left heart bypass support lasting 1 month. Therefore, the circulatory assist device was switched to the extracorporeal Nipro ventricular assist device (VAD). This time, left heart bypass support could be maintained for 30 days using a single Rotaflow Centrifugal Pump(®). There were no signs of hemolysis during left heart bypass support. The Rotaflow Centrifugal Pump(®) itself may be used as a device for a bridge to decision or recovery before using a VAD in cardiogenic shock patients.

Survey of blood pump diaphragm damage in the NIPRO-ventricular assist device.

We surveyed the incidence of blood pump diaphragm damage (rupture or crack) in the NIPRO-ventricular assist device (VAD). In the cases in which rupture or suspected blood pump crack was detected, we disassembled the pumps to visually check the condition of the diaphragm after replacement or use. Of 366 blood pumps surveyed, diaphragm damage was observed in 2.7 %. The duration of use of the blood pumps with diaphragm damage was significantly longer than that of pumps without damage. The incidence of diaphragm damage increased with longer duration of use. On the basis of these findings, blood pump diaphragm damage in the NIPRO-VAD may be associated with duration of use. However, some blood pumps were used for prolonged periods without diaphragm damage. All blood pumps with damage had a crack in the diaphragm on the air chamber side near the diaphragm-housing (D-H) junction. Cracks were not found in any specific part of the diaphragm. In blood pumps with diaphragm rupture, the crack had a through-hole reaching the blood-contacting surface. Although we were unable to identify the causes of the cracks, it is suggested that when a crack appears in the diaphragm it will gradually expand and eventually lead to rupture. If a crack is detected in a blood pump, we advocate replacing the pump before it grows. When the NIPRO-VAD is used, it is necessary to keep in mind that blood pump diaphragm damage may occur.

Subclinical involvement of the trunk muscles in idiopathic inflammatory myopathies.

BACKGROUND: Whole-body magnetic resonance imaging (WB-MRI) is a useful tool for revealing the disease-specific distribution of affected muscles and clinically asymptomatic muscle involvements in idiopathic inflammatory myopathies (IIMs). PURPOSE: To examine inflammatory changes in the systemic skeletal muscles, including the thoracoabdominal trunk, in IIMs using WB-MRI. MATERIAL AND METHODS: We prospectively obtained WB-MRI axial images from 10 patients with IIMs, including antisynthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), sporadic inclusion body myositis, and myopathy associated with antimitochondrial antibody. We evaluated 108 systemic skeletal muscles in short-tau inversion recovery (STIR) images and rated changes in signal intensity using a semiquantitative scale. Correlations between STIR sum score, peak creatine kinase (CK) and muscle strength were examined. We also investigated the correlation between STIR sum score within the thoracoabdominal trunk and forced vital capacity. RESULTS: High STIR signal changes were frequently identified in asymptomatic and routinely unexamined muscles. Thoracoabdominal trunk muscles were frequently involved in ASS and IMNM. Peak CK was positively correlated with the STIR sum score (R 2 = 0.62, p < .01). There was no significant correlation between the STIR sum score within the thoracoabdominal trunk and forced vital capacity. CONCLUSION: WB-MRI can detect subclinical muscle inflammation in the systemic muscles including the trunk muscles. STIR sum score is positively correlated with serum peak CK level; therefore, it could be a biomarker of overall muscle inflammation.