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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticlePediatric Neuroimaging

New MRI Findings in Fukuyama Congenital Muscular Dystrophy: Brain Stem and Venous System Anomalies

A. Hirasawa-Inoue, N. Sato, Y. Shigemoto, Y. Kimura, A. Ishiyama, E. Takeshita, M. Mori-Yoshimura, Y. Oya, Y. Takahashi, H. Komaki, H. Matsuda and M. Sasaki
American Journal of Neuroradiology June 2020, 41 (6) 1094-1098; DOI: https://doi.org/10.3174/ajnr.A6577
A. Hirasawa-Inoue
aFrom the Departments of Child Neurology (A.H.-I., A.I., E.T., H.K., M.S.)
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  • ORCID record for A. Hirasawa-Inoue
N. Sato
bRadiology (N.S., Y.S., Y.K.)
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Y. Shigemoto
bRadiology (N.S., Y.S., Y.K.)
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Y. Kimura
bRadiology (N.S., Y.S., Y.K.)
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A. Ishiyama
aFrom the Departments of Child Neurology (A.H.-I., A.I., E.T., H.K., M.S.)
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  • ORCID record for A. Ishiyama
E. Takeshita
aFrom the Departments of Child Neurology (A.H.-I., A.I., E.T., H.K., M.S.)
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  • ORCID record for E. Takeshita
M. Mori-Yoshimura
cNeurology (M.M.-Y., Y.O., Y.T.)
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  • ORCID record for M. Mori-Yoshimura
Y. Oya
cNeurology (M.M.-Y., Y.O., Y.T.)
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Y. Takahashi
cNeurology (M.M.-Y., Y.O., Y.T.)
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  • ORCID record for Y. Takahashi
H. Komaki
aFrom the Departments of Child Neurology (A.H.-I., A.I., E.T., H.K., M.S.)
dNational Center Hospital, Translational Medical Center (H.K.)
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H. Matsuda
eIntegrative Brain Imaging Center (H.M.), National Center of Neurology and Psychiatry, Tokyo, Japan.
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M. Sasaki
aFrom the Departments of Child Neurology (A.H.-I., A.I., E.T., H.K., M.S.)
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    Fig 1.

    Brain MR imaging of patient 15. Brain stem images on T2WI (A), FLAIR (B), and DIR (C). There is a fluffy structure on T2WI (A, black arrows) and high intensity of the hypoplastic pontine surface on the FLAIR image (B, white arrows). High signal intensity along the brain stem surface on the DIR image is recognized more clearly than on the T2WI or FLAIR image (C, white arrows). T2WIs of the left temporal lobe (D–F) show lissencephalic and the abnormally located cortical superficial vessels running through the superficial layer of the lissencephalic cortex (white arrows). Microhemorrhages were seen in the cerebellar cyst on T2WI (G) and SWI (H). More microhemorrhages are recognizable on the SWI than on T2WI (white arrowheads). Cerebral SWI (I) shows hypoplasia of a superficial cerebral vein in both frontal lobes (white arrows) and dilated and tortuous subependymal veins (white circle).

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    Fig 2.

    Brain stem (A and B) and left temporal (C–E) MRIs of patient 20. A brain stem T2WI (A) shows a fluffy structure and high intensity of the hypoplastic pontine surface (black arrows). The FLAIR image (B) detects high intensity of the pontine surface (white arrows). Left temporal T2WIs (C–E) show lissencephaly, which contains abnormally located superficial cortical vessels running through and beneath the cortex (white arrows).

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    Fig 3.

    Cerebral MRIs of patient 13. T2WIs (A and B) show polymicrogyria and diffuse high signal in the white matter. SWIs (C and D) reveal hypoplasia of superficial cerebral veins in the frontal lobe (white arrows) and meandering subependymal veins (white circles).

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    Fig 4.

    Cerebral MRIs of patient 25. There are hemorrhages in the deep and subcortical white matter of the atrophic left frontal lobe (white arrows) on T2WIs. The location of the hemorrhage in this patient is different from that of the subependymal hemorrhage in a premature neonate.

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American Journal of Neuroradiology: 41 (6)
American Journal of Neuroradiology
Vol. 41, Issue 6
1 Jun 2020
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A. Hirasawa-Inoue, N. Sato, Y. Shigemoto, Y. Kimura, A. Ishiyama, E. Takeshita, M. Mori-Yoshimura, Y. Oya, Y. Takahashi, H. Komaki, H. Matsuda, M. Sasaki
New MRI Findings in Fukuyama Congenital Muscular Dystrophy: Brain Stem and Venous System Anomalies
American Journal of Neuroradiology Jun 2020, 41 (6) 1094-1098; DOI: 10.3174/ajnr.A6577

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New MRI Findings in Fukuyama Congenital Muscular Dystrophy: Brain Stem and Venous System Anomalies
A. Hirasawa-Inoue, N. Sato, Y. Shigemoto, Y. Kimura, A. Ishiyama, E. Takeshita, M. Mori-Yoshimura, Y. Oya, Y. Takahashi, H. Komaki, H. Matsuda, M. Sasaki
American Journal of Neuroradiology Jun 2020, 41 (6) 1094-1098; DOI: 10.3174/ajnr.A6577
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