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Hemimegalencephaly: A Study of Abnormalities Occurring Outside the Involved Hemisphere

N. Satoa, A. Yagishitac, H. Obad, Y. Mikie, Y. Nakataa, F. Yamashitaf, K. Nemotog, K. Sugaib and M. Sasakib

a Department of Radiology, National Center Hospital for Mental, Nervous, and Muscular Disorders, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
b Department of Child Neurology, National Center Hospital for Mental, Nervous, and Muscular Disorders, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
c Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, Kokubunji, Tokyo, Japan
d Department of Radiology, Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan
e Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University School of Medicine, Kyoto, Kyoto, Japan
f Clinical Neuroscience, Medical Sciences for Control of Pathological Processes, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan
g Department of Psychiatry, Ibaraki Prefectural Tomobe Hospital, Ibaraki, Japan


Figure 1
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Fig 1. A 3-month-old boy with right hemimegalencephaly.

A and B, Coronal T2-weighted images demonstrate a diffusely enlarged right olfactory nerve from the exit to the distal portion (arrows). The left nerve is not enlarged.

B, Superficial cortical veins are asymmetrically dilated on the right (arrowheads).


Figure 2
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Fig 2. A 7-month-old boy with left hemimegalencephaly.

A, Axial FLAIR image shows an asymmetrically enlarged left optic nerve (arrow).

B, Coronal T1-weighted image reveals findings similar to those in A (arrow).

C, Coronal T2-weighted image reveals asymmetry of the olfactory nerves (arrow), as well, with the left one slightly larger than the right at the exit zone (arrowhead).


Figure 3
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Fig 3. An 8-month-old girl with right hemimegalencephaly. Axial T2-weighted image shows an asymmetrically dilated right internal cerebral vein (arrow).


Figure 4
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Fig 4. A 4-month-old boy with right hemimegalencephaly. Coronal T2-weighted image shows markedly dilated superficial cerebral cortical vessels (arrows) and subependymal veins (arrowhead) on the ipsilateral side.


Figure 5
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Fig 5. A 4-month-old girl with right hemimegalencephaly.

A, Axial T2-weighted image demonstrates asymmetry of the medulla oblongata and hemicerebellum with abnormal folia.

B, Axial T2-weighted image shows asymmetry of the pons and hemicerebellum.

C, Axial T2-weighted image also shows an asymmetric midbrain. The findings for this case are compatible with total hemimegalencephaly.


Figure 6
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Fig 6. A 4-month-old boy with left hemimegalencephaly.

A, Axial T2-weighted image shows an asymmetrically enlarged left hemicerebellum. No asymmetry of the brain stem is noted.

B, Coronal FLAIR image also shows asymmetry of both the left hemicerebrum and hemicerebellum.


Figure 7
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Fig 7. A 6-month-old boy with right hemimegalencephaly.

A and B, Coronal T2-weighted images demonstrate abnormal folia in both ipsilateral and contralateral cerebellar hemispheres.