Serial brain MRI changes related to autoimmune pathophysiology in Hashimoto encephalopathy with anti-NAE antibodies: A case-series study.

PURPOSE: Hashimoto encephalopathy (HE) is an autoimmune-mediated encephalopathy associated with anti-thyroid antibodies. We previously discovered serum autoantibodies against the NH2-terminal of α-enolase (NAE), which serve as a specific diagnostic biomarker for HE and may be involved in the autoimmune pathophysiology of HE, including vasculitis. Although the common findings of brain magnetic resonance imaging (MRI) in HE have been recognized as normal or non-specific white matter lesions, serial MRI changes have been less well studied. The aim of this study was to clarify detailed and longitudinal MRI changes in HE associated with anti-NAE antibodies. METHODS: We investigated serial brain MR images in 12 Japanese patients with HE who had serum anti-NAE antibodies. RESULTS: Brain MRI showed diffuse white matter abnormalities and/or multiple small subcortical lesions in 10 patients. These lesions were apparently non-specific; however, in 7 of these patients we observed expanding and diminishing white matter lesions, emerging subcortical high-intensity spots on diffusion-weighted images, or reversible limbic lesions, which worsened at relapse and improved after recovery following immunotherapies. CONCLUSION: MRI lesions that fluctuate according to the disease condition were frequently observed in HE patients with anti-NAE antibodies, which suggests that these fluctuation may be associated with the autoimmune pathophysiology of HE.

Influenza A (H3N2)-induced rhabdomyolysis complicating anterior compartment syndrome: Serial changes in muscle MRI T2 fat suppression imaging.

BACKGROUND: Rhabdomyolysis with influenza infection is rarely reported in adults. We report here influenza A induced rhabdomyolysis and anterior compartment syndrome (ACS). CASE REPORT: This case report describes a 43-year-old woman exhibiting influenza A induced rhabdomyolysis. High levels of creatine kinase (97,000 IU/L) and high titer of anti-influenza A virus antibody (H3N2) (320 ×) with negative anti-influenza B virus antibody were observed. T2 fat suppression muscle MRI imaging showed high-intensity signals in rectus femoris, vastus lateralis, adductor magnus, and semimembranosus (SM) muscles. The existence of ACS was suspected out. Muscle biopsy showed that fiber size variations exist without infiltration of inflammatory cells. The symptoms and muscle MRI findings of T2 fat suppression imaging was markedly improved. CONCLUSIONS: Muscle MRI T2 fat suppression imaging is a useful method to monitor influenza A induced rhabdomyolysis. We should keep in mind the possibilities of rhabdomyolysis and ACS in patients with influenza A infection presenting serious muscle pain.

Diagnostic Performance and Patient Acceptance of Reduced-Laxative CT Colonography for the Detection of Polypoid and Non-Polypoid Neoplasms: A Multicenter Prospective Trial.

Purpose To evaluate the diagnostic accuracy and patient acceptance of reduced-laxative computed tomographic (CT) colonography without computer-aided detection (CAD) for the detection of colorectal polypoid and non-polypoid neoplasms in a population with a positive recent fecal immunochemical test (FIT). Materials and Methods Institutional review board approval and written informed consent were obtained. This multicenter prospective trial enrolled patients who had positive FIT results. Reduced-laxative CT colonography and colonoscopy were performed on the same day. Patients received 380 mL polyethylene glycol solution, 20 mL iodinated oral contrast agent, and two doses of 20 mg mosapride the day before CT colonography. The main outcome measures were the accuracy of CT colonography for the detection of neoplasms 6 mm or larger in per-patient and per-lesion analyses and a survey of patient perceptions regarding the preparation and examination. The Clopper-Pearson method was used for assessing the 95% confidence intervals of per-patient and per-lesion accuracy. Survey scores were analyzed by using the Wilcoxon and χ2 tests. Results Three hundred four patients underwent both CT colonography and colonoscopy. Per-patient sensitivity, specificity, positive predictive value, and negative predictive value of CT colonography for detecting neoplasms 10 mm or larger were 0.91 (40 of 44), 0.99 (255 of 258), 0.93 (40 of 43), and 0.98 (255 of 259), respectively; these values for neoplasms 6 mm or larger were 0.90 (71 of 79), 0.93 (207 of 223), 0.82 (71 of 87), and 0.96 (207 of 215), respectively. Per-lesion sensitivities for detection of polypoid and non-polypoid neoplasms 10 mm or larger were 0.95 (40 of 42) and 0.67 (six of nine), respectively; those for neoplasms 6 mm or larger were 0.90 (104 of 115) and 0.38 (eight of 21), respectively (P < .05 for both). Patient acceptance of preparation and examination with CT colonography was significantly higher than that with colonoscopy, and 62% (176 of 282) of patients would choose CT colonography as the first examination if they have a positive FIT result in the future. Conclusion Reduced-laxative CT colonography without CAD is accurate in the detection of polypoid neoplasms 6 mm or larger but is less accurate in the detection of non-polypoid neoplasms. Reduced-laxative CT colonography has high patient acceptance and is an efficient triage examination for patients with a positive FIT. © RSNA, 2016 Online supplemental material is available for this article.

Muscle MRI of the Upper Extremity in the Myotonic Dystrophy Type 1.

BACKGROUND: The purpose of this study was to explore the relation between muscle MRI findings and weakness of the upper extremity muscles in patients with myotonic dystrophy type 1 (DM1). METHODS: Nineteen DM1 patients from 15 families were enrolled in this study. Muscle weakness was evaluated using the modified Medical Research Council scale. Subjects also underwent a genetic study and muscle MRI of the upper extremities. RESULTS: In patients with DM1, the flexor digitorum profundus (FDP), flexor pollicis longus, flexor digitorum superficialis (FDS), extensor pollicis, abductor pollicis longus (APL), lateral head of triceps brachii and infraspinatus (INF) muscles were frequently and severely affected. Muscle strength was significantly correlated with the severity of muscle MRI findings in the FDP, short head of biceps brachii (SBB), and medial head of triceps brachii muscles. Disease duration was correlated significantly with MRI findings in the FDP, FDS, long head of biceps brachii, INF, APL, and SBB muscles. Unexpectedly, the degree of trinucleotide expansion of myotonin protein kinase was not correlated with muscle MRI findings. CONCLUSION: Muscle MRI of the upper extremity is useful to detect affected muscles in DM1 patients.

Familial Summer-type Hypersensitivity Pneumonitis: A Review of 25 Families and 50 Cases in Japan.

Summer-type hypersensitivity pneumonitis (SHP) is the most common form of pneumonitis in Japan; it accounts for 74% of all cases. It has been reported that 19.5-23.8% of SHP cases occur in families who live in the same house. We present our SHP cases and review 50 familial cases in 23 families that were reported in Japan (including our own) and 48 cases that were previously described in 22 articles published between January 1982 and October 2011. To the best of the authors' knowledge, this is the first review article in English to document the familial occurrence of SHP in Japan.

Clinical manifestations and prognostic factor of iliopsoas abscess.

INTRODUCTION: Iliopsoas abscess (IPA) is a collection of pus in the iliopsoas compartment, which is considered rare in Japan. However, the number of patients with IPA has recently increased among the elderly or compromised hosts. SUBJECTS AND METHODS: This retrospective study aims to examine the clinical pictures, pathological findings, and the prognostic factor of IPA. We analyzed all patients with IPA who were admitted to our hospital from April 2006 to July 2011. Patients' characteristics, treatment, clinical outcome, radiological findings, bacteria isolated, and comorbidities were evaluated. The comorbidities were evaluated by the Charlson comorbidity index (CCI). We compared the survival and non-survival groups to assess the prognostic factors of IPA. RESULTS: A total of 33 patients were enrolled in this study, which included 14 males and 19 females. The mean age of the patients was 71.5 years (range 32-92 years). The most common underlying disease was spinal disease (16 of 33, 48.5%). Twenty-nine patients (87.9%) were cured and four patients (12.1%) died. While 12 patients (36.4%) were initially treated conservatively with antibiotics alone, percutaneous drainage was performed initially in 19 patients (57.6%). Two patients (6%) directly underwent exploratory surgery and open drainage. In terms of patients' characteristics, there were no significant differences in either group. The score of CCI in non-survivors was higher than that in survivors (1.38 vs. 5.5, P < 0.001). CONCLUSIONS: The epidemiology of IPA is quite different from what it used to be. CCI appears to be useful in evaluating the patients' prognosis with IPA.

Is emphysema a risk factor for pneumothorax in CT-guided lung biopsy?

INTRODUCTION: Computed tomography (CT)-guided lung biopsy is commonly used to make a histological diagnosis for pulmonary lesions. Its most common complication is pneumothorax. While it is thought that CT-guided lung biopsy should be avoided in patients with emphysema, however, there is no scientific report documenting the relationship the occurrence of pneumothorax and the severity of emphysema. PURPOSE AND METHODS: To investigate the relationship between the severity of emphysema and the frequency of pneumothorax, we retrospectively reviewed all the patients who received CT-guided lung biopsy. Severity of emphysema is evaluated by Goddard classification, a visual scale by which areas of vascular disruption and low attenuation value were scored for each lung field of high resolution CT. Patients' characteristics, prognostic accuracy of this method, size and location of the lesion, length of intrapulmonary biopsy paths, and frequency of complications such as pneumothorax or intrapulmonary hemorrhage were evaluated. RESULTS: One hundred-two patients (69 males and 33 females) received 102 procedures. Diagnostic accuracy was 90.2%. Pneumothorax occurred in 41 of 102 biopsies (40.2%). Chest tube placement was required in 3 out of the 41 cases (7.3%) complicated by pneumothorax (2.9% of all the biopsies). The longer lesion depths from pleura were, the more frequently pneumothorax occurred (6.67 vs 3.66 mm, p=0.019). No correlation was found between location of lesions and frequency of pneumothorax. No significant differences of COPD staging or LAA score were seen between the patients with and without pneumothorax (5.73 vs 4.32 points, p=0.339). CONCLUSION: We suggest that severity of emphysema such as stage I or II COPD may not be related to the frequency of pneumothorax.

Therapeutic impact of CT-guided percutaneous catheter drainage in treatment of deep tissue abscesses.

Combination therapy of CT-guided percutaneous drainage and antibiotics is the first-line treatment for abscesses. Its effectiveness has been demonstrated. However, the therapeutic impact of this procedure for infection treatment has never been reported. We retrospectively analyzed all 47 patients who received CT-guided percutaneous drainage for infection treatment. Patients' characteristics, pathogens isolated, antibiotics administered, technical and clinical outcomes, complications related to this procedure and therapeutic impacts were investigated. Patients were 26 males and 21 females. The mean age was 63.5 years (±18.7). The diseases targeted were 19 retroperitoneal abscesses, 18 intra-abdominal abscesses, three pelvic abscesses, and seven others. As for technical outcomes, all of the 54 procedures (100%) were successful. As for clinical outcomes, 44 (93.6%) were cured and three patients (6.4%) died. No complications related to this procedure were found in this study. A total of 42 patients (88%) had a change in the management of their infection as a result of CT-guided percutaneous drainage, such as selection and discontinuation of antibiotics. In conclusion, CT-guided percutaneous drainage is a safe and favorable procedure in the treatment of deep tissue abscesses. Therapeutic impact of these procedures helped physicians make a rational decision for antibiotics selection.

A case of obstructive sleep apnea syndrome caused by malignant melanoma in the nasal cavity and paranasal sinus.

A 71 year-old obese woman complained of obstructive sleep apnea syndrome (OSAS) related symptoms. The apnea-hypopnea index (AHI) was 73.5/hour. She presented with nasal bleeding to an ENT doctor. A mass on the nasal septum was seen and biopsy was performed. Histological confirmation showed malignant melanoma. The tumor stage proved to be cT4aN2M1 (stage IV) due to multiple metastatic lesions. After palliative irradiation, the nasal tumor was reduced in size and her symptoms of OSAS were improved. The second AHI revealed 13.5/hour. This case was considered to be OSAS caused by a tumor obstructing the nasal cavity. This might suggest the necessity of routine work-up of the upper airway in cases of patients with sleep disorder. Otherwise, OSAS caused by such obstruction might be missed. We report a very rare case with secondary OSAS caused by malignant melanoma in the nasal cavity and paranasal sinus.

A successful diagnostic case of Pneumocystis pneumonia by the loop-mediated isothermal amplification method in a patient with dermatomyositis.

Pneumocystis pneumonia (PCP) can occur in patients with many causes of the immunocompromised state other than human immunodeficiency virus (HIV). It is quite difficult to diagnose PCP without HIV because there is no method for detecting Pneumocystis jirovecii. Thus, non-HIV PCP continues to have high mortality. Recently, loop-mediated isothermal amplification (LAMP) is becoming an established nucleic acid amplification method offering rapid, accurate, and cost-effective diagnosis of infectious diseases. We report a non-HIV PCP case successfully diagnosed by the LAMP method. It was previously reported that PCR in BALF specimens had been the most sensitive method in the diagnosis of PCP without HIV. The LAMP method would be more sensitive than conventional PCR and an effective tool in the early diagnosis of PCP.

A case of novel swine influenza A (H1N1) pneumonia complicated with virus-associated hemophagocytic syndrome.

Influenza related to complications such as pneumonia and encephalitis have sporadically been reported. However, influenza A (H1N1)-virus-associated hemophagocytic syndrome (VAHS) has rarely been reported. A 39-year old woman complained of high fever and was referred to us. Chest infiltrations in both lungs and a positive polymerase chain reaction (PCR) for novel swine origin influenza A (H1N1) in bronchial alveolar lavage fluid (BALF) specimen was confirmed and she was diagnosed with influenza A (H1N1) pneumonia. Pancytopenia was found, and hemophagocytic syndrome (HPS) was diagnosed by bone marrow aspiration. Following intravenous administration of antiflu drug and combination therapy of steroid pulse and erythromycin IV, the patient's respiratory dysfunction and lab data gradually improved and she was discharged on day 21. Whereas secondary HPS related to viral infections such as Epstein­Barr virus, cytomegalovirus, and human herpesvirus type 6 are commonly seen, H1N1 pneumonia complicated with secondary VAHS is rare.

[Relationship between the prognosis of interstitial pneumonia and its comorbidities].

BACKGROUND: To investigate the relationship between the prognosis of chronic interstitial pneumonia (IP) and its comorbidities, we conducted a retrospective study for clinically and radiologically diagnosed IP. We assessed comorbidities by using the Charlson Comorbidity Index (CCI). METHODS: We classified 224 patients given clinical diagnoses of chronic IP (excluding the patients who had clear causes such as collagen disease, infection, drugs or radiation) in our institution between April 2000 and June 2010, into 2 groups; those with clinical diagnoses of idiopathic pulmonary fibrosis (IPF:108 cases) and those with other chronic IP but without honeycomb lung (116 cases); and analyzed their backgrounds and comorbidities. We also classified them into survival and non-survival groups to assess their prognostic factors. RESULTS: Although the smoking status of patients with clinically diagnosed IPF was higher, and SpO2 was lower than those with other chronic IP without honeycomb lung, the mean age, comorbidities and CCI did not differ between them. The 5-year overall survival of the clinically-diagnosed IPF group was lower than that of the other chronic IP without honeycomb lung group (50.8% vs. 76.3%, p<0.01). In cases of other chronic IP without honeycomb lung, the CCI of non-survival cases was higher than that of survival cases (4.05 vs. 2.47, p<0.01), although patient backgrounds did not differ between survival and non-survival cases in those with clinically diagnosed IPF (CCI : 2.32 vs. 2.98, p = 0.70). CONCLUSIONS: Our analysis revealed the possibility that comorbidities and CCI were prognostic factors in other chronic IP cases without honeycomb lung, although the prognosis of IPF was not affected by their comorbidity.

Muscle MRI in myotonic dystrophy type 1 with foot drop.

The purpose of this study was to investigate the relationship of muscle MRI findings and gait disturbance in myotonic dystrophy type 1 (DM1) patients. Thirteen patients with DM1 were evaluated by manual muscle strength test and muscle MRI of the lower limb. All DM1 patients presenting with foot drop showed high intensity signals in the tibialis anterior muscles on T1-weighted imaging (p < 0.001). The patients presenting with gait disturbance showed high intensity signals in the semimembranosus, vastus intermedius and gastrocnemius medialis muscles, too. Disturbance of the gastrocnemius lateralis muscles was mild in all DM1 patients. The patients without gait disturbance showed no abnormalities, especially in tibialis anterior muscles on muscle MRI. Muscle MRI is useful for the detection of pathological muscles in DM1 patients with gait disturbance.

Tissue characterization of glioma by proton magnetic resonance spectroscopy and perfusion-weighted magnetic resonance imaging: glioma grading and histological correlation.

BACKGROUND AND PURPOSE: Our intent was to clarify the usefulness of proton magnetic resonance spectroscopy (MRS) and perfusion-weighted magnetic resonance imaging (PW-MRI) in the grading of glioma. METHODS: Twenty-three consecutive patients with gliomas were investigated by both proton MRS and PW-MRI. For quantitative analysis, the metabolite data of the gliomas were estimated using the LCModel software. Receiver operating characteristic (ROC) curve analyses were also performed to assess which metabolite parameter was optimal for discrimination of glioma grade. From the PW-MRI data, the value of blood volume was measured on the parametric map corresponding to the location of MRS analysis. We then compared tumor blood volume with the amount of choline (Cho). RESULTS: The mean Cho/creatine (Cre) ratio was useful to discriminate between Grades II and III, and the mean lactate (Lac)/Cre ratio was found to be significantly different between Grades III and IV. ROC curve analysis showed that measurements involving Cho were superior indices for grading glioma compared with blood volume information. Furthermore, the correlation between tumor blood volume and the amount of choline was statistically significant. CONCLUSION: MRS may provide valuable information for glioma grading.

Evaluation of CH3-DTPA-Gd (NMS60) as a new MR contrast agent: early phase II study in brain tumors and dual dynamic contrast-enhanced imaging.

PURPOSE: A newly developed contrast material, CH3-DTPA-Gd (NMS60), a trimer containing 3 Gd(3+) atoms per molecule, has been shown to offer greater enhancement and longer vascular retention than gadopentetate dimeglumine (Gd-DTPA) in animals. We report on our early phase II study on NMS60 in brain tumor patients together with supplementary investigations. METHODS AND MATERIALS: The longitudinal relaxation rate (R(1)=1/T(1)) and the transverse relaxation rate (R(2)*=1/T(2)*) of NMS60 and Gd-DTPA were determined at 20 degrees C in water at 1.5 T. An NMS60 dose of 0.1 or 0.2 mmol (Gd)/kg was randomly assigned and administered to 10 patients (five women, five men; mean age: 49 years) with brain tumors. Safety and contrast-enhancing ability of NMS60 were evaluated. Dual dynamic contrast-enhanced T(1) and R(2)* studies (DUCE imaging) were also carried out in two patients. RESULTS: Regarding the relaxivity per Gd, R(1) and R(2)* of NMS60 were 9.5 and 11.0 (mmol/L x s)(-1), respectively, compared to 4.8 and 7.2 (mmol/L x s)(-1) for Gd-DTPA. Although a transient slight increase of alanine aminotransferase was observed in one case, no other adverse reactions were observed after administration of NMS60. Contrast enhancement by NMS60 was excellent at both concentrations, and when tumor detectability was assessed with a five-point scale, the diagnostic usefulness was 4 or higher in all cases. In DUCE imaging, NMS60 appeared to show high signal intensity, when compared with the data obtained separately for Gd-DTPA. CONCLUSION: NMS60 had a high contrasting effect and little toxicity, and is expected to be clinically useful.

Occult fractures of articular eminence and glenoid fossa presenting as temporomandibular disorder: a case report.

We report an unusual case of occult fractures of the articular eminence and glenoid fossa due to a previous traffic accident presenting as a temporomandibular disorder. A 24-year-old Japanese man was referred for trismus and pain in the right temporomandibular joint, and was suspected of having temporomandibular disorder. Although the magnetic resonance image did not show displacement of the articular disk, T2-weighted images revealed disruption of the cortical low-intensity line of the right articular eminence. On a computerized tomography (CT) scan, an isolated bone fragment of the right articular eminence was clearly seen, and fractures of the right glenoid fossa and articular eminence were noted. The patient was treated by instructing him not to open his mouth widely and to remain on a soft diet for 4 weeks. A CT examination performed 1 year after the treatment showed complete healing of the fracture in the right articular eminence and glenoid fossa.

Safety and efficacy of a novel hepatobiliary MR contrast agent, Gd-DTPA-DeA: results of phase I and phase II clinical trials.

PURPOSE: To assess the safety, effective dose, and efficacy of a novel hepatobiliary MR contrast agent Gd-DTPA-DeA for imaging liver tumors, from the clinical phase I and phase II trials in Japan. MATERIALS AND METHODS: In a phase I trial, 33 healthy volunteers were intravenously administered a single dose of 0.03-10 micromol/kg of Gd-DTPA-DeA. In a nationwide phase II trial, 80 patients suspected to have hepatic mass were divided into three dosing groups: 2.5, 5.0, or 7.5 micromol/kg. T1-weighted gradient echo images were obtained before and after Gd-DTPA-DeA administration at three time points. Liver signal-to-noise ratio (SNR) and lesion-liver contrast-to-noise ratio (CNR) were calculated at each time point. A reading committee evaluated the contrast, diagnostic, and overall efficacy using a five-point scale. RESULTS: In a phase I trial, dosages up to 10 micromol/kg were well tolerated by healthy volunteers. In a phase II trial, the contrast, diagnostic, and overall efficacy increased dose-dependently. The overall efficacy was 63.0%, 85.2%, and 88.0%, for 2.5, 5, and 7.5 micromol/kg, respectively. Liver SNR and CNR increase was greater at late phase than at early phase. No serious adverse events occurred. CONCLUSION: Gd-DTPA-DeA is a well-tolerated and promising contrast agent for liver MR imaging.