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Objective To summarize the characteristics of fatty infiltration and edema of muscle MRI in Duchenne muscular dystrophy (DMD) patients. Methods A total of 70 DMD patients underwent Clinical Functional Scale and muscle MRI of the pelvic (gluteus maximus, gluteus medius, gluteus minimus, iliopsoas, piriformis, obturator internus, obturator externus, tensor fasciae latae, pectineus, erector spinae, psoas major muscle, iliacus), thigh (adductor magnus, gracilis, adductor longus, sartorius, rectus femoris, vastus intermedius, vastus medialis, vastus lateralis, biceps femoris, semitendinosus, semimembranosus) and leg (gastrocnemius, soleus, peroneus longus, tibialis posterior, tibialis anterior, flexor hallucis longus, extensor hallucis longus, extensor digitorum longus). T1WI fatty infiltration grade and fat suppression T2WI edema grade were performed to analyze the imaging features of fatty infiltration and edema. Results In 31 muscles, 30 muscles (96.77% ) presented fatty infiltration and 23 (74.19% ) presented edema. The occurrence rate of fatty infiltration was higher than that of edema. A total of 21 muscles including gluteus maximus, were found T1WI fatty infiltration grades greater than 2, and 10 muscles including tibialis posterior, were less than 1. Five muscles including soleus were found fat suppression T2WI edema grade 3. Spearman rank correlation analysis showed positive correlations between T1WI fatty infiltration grade and Clinical Functional Scale in 7 pelvic muscles [gluteus maximus (rs = 0.518, P = 0.016), gluteus medius (rs = 0.528, P = 0.014), gluteus minimus (rs = 0.528, P = 0.014), iliopsoas (rs = 0.695, P = 0.000), piriformis (rs = 0.451, P = 0.040), pectineus (rs = 0.567, P = 0.009), erector spinae (rs = 0.499, P = 0.025)], 7 thigh muscles [adductor magnus (rs = 0.607, P = 0.005), adductor longus (rs = 0.547, P = 0.013), rectus femoris (rs = 0.614, P = 0.004), vastus intermedius (rs = 0.566, P = 0.009), vastus medialis (rs = 0.522, P = 0.018), vastus lateralis (rs = 0.503, P = 0.024), biceps femoris (rs = 0.508, P = 0.022)] and 3 leg muscles [gastrocnemius (rs = 0.715, P = 0.001), peroneus longus (rs = 0.571, P = 0.017), tibialis posterior (rs = 0.514, P = 0.035)]. There was no correlation between T1WI fatty infiltration grade and Clinical Functional Scale in other muscles (P > 0.05, for all). There was no correlation between fat suppression T2WI edema grade and Clinical Functional Scale in all muscles (P > 0.05, for all). Conclusions Fatty infiltration grade can be well used to assess the severity of DMD. The role of muscle edema on assessing clinical severity should be analyzed according to individual muscle characteristics. DOI: 10.3969/j.issn.1672-6731.2018.07.006

Abstract Objective To evaluate different injury factors and pathological characteristics of the brain at different disease stages in toxic milk (TX) mice, an animal model of Wilson's disease (WD). Methods Thirty TX mice (10 each at 3, 6 and 12 months old) and 30 age‐matched C57 mice were used in this study. Corrected phase (CP) values were determined from susceptibility‐weighted images. Myelin content was determined by measuring inhibition optical density values of Luxol fast blue‐stained sections. Neurofilament protein 68 kDa (NF68), β‐amyloid precursor protein (β‐APP), and myelin basic protein (MBP) levels, as well as copper and iron content, in brain nuclei of the TX mouse were evaluated. Gene amplification ratios for catalase (CAT), GSH peroxidase (GSH‐PX), nitric oxide synthase (NOS), and superoxide dismutase (SOD) in mouse brain were also determined. Results Compared with C57 mice, neuronal cell counts were decreased in 12‐months‐old TX mice (p = .011). Myelin content was decreased in the lenticular nucleus (p = .029), thalamus (p = .030), and brainstem (p = .034) of 6‐months‐old TX mice; decreases in the corresponding nuclei (p = .044, .037, and .032, respectively) were also found in 12‐months‐old TX mice. MBP values were lower in the lenticular nucleus and thalamus (p = .027 and .016, respectively) of 6‐months‐old TX mice and in the corresponding nuclei (p = .24 and .040) of 12‐months‐old TX mice. NF‐68 values were lower in the lenticular nucleus and thalamus (p = .034 and .037, respectively) of 6‐months‐old TX mice and in the corresponding nuclei (p = .006 and .012) of 12‐months‐old TX mice. β‐APP values were higher in the thalamus of 6‐months‐old (p = .037) and 12‐months‐old (p = .012) TX mice. Iron content was higher in the lenticular nucleus, thalamus, and cerebellum (p = .044, .038, and .029, respectively) of 6‐months‐old TX mice and in the corresponding nuclei (p = .017, .024, and .029) of 12‐months‐old TX mice. The NOS gene amplification multiple was higher (p = .039), whereas the SOD1 gene amplification multiple was lower (p = .041) in 12‐months‐old TX mice. There was no correlation between metal content or oxidation index and pathological index. Conclusions The pathological characteristics of the brains of TX mice may differ at different ages. Different pathogenic factors, including copper and iron deposition and abnormal oxidative stress, are present at different stages.

Aims: The aim of this study was to evaluate the feasibility of characterizing the brain-mineral deposition in patients with Wilson disease (WD) using susceptibility-weighted imaging (SWI). Materials and Methods: The study enrolled 30 WD patients and 20 age-matched healthy controls. Neurological symptoms were scored using the modified Young Scale. The hepatic function indices, serum and urinary copper content, and serum iron content were determined. All study objects received the magnetic resonance imaging (MRI) and SWI test of the brain. The values of corrected phase (CP) were calculated on SWI. The relationship between CP values and the clinical status were evaluated. Results: The serum iron content of WD patients was higher than the normal. The CP values of substantia nigra, caudate nucleus, and globus pallidus of WD were lower than the normal values, while the CP value of substantia nigra was the lowest. No correlations were determined between the CP values and the iron and copper parameters. There was negative correlation between the scores of dysarthria and the CP values of the globus pallidus. There was negative correlation between the scores of tremor and the CP values of caudate nucleus. Some regions, which had high signals on T2-weighted image, had low signals on SWI. Conclusions: There might be abnormal iron metabolism in patients with WD. The decreased CP values might reflect a deposition of both copper and iron. SWI may be more sensitive than the ordinary MRI. The mineral deposition may contribute to the neural symptoms. [ABSTRACT FROM AUTHOR]