Middle interhemispheric variant of holoprosencephaly associated with diffuse polymicrogyria.
نویسندگان
چکیده
An 11-month-old boy was discovered to have a cleft palate, club foot, hypospadias, and myoclonic seizures. No in utero exposure to teratogens was identified. Brain MR imaging revealed a middle interhemispheric fusion variant of holoprosencephaly, diffuse polymicrogyria, and a hypoplastic brain stem; this was a distinctly unusual association of findings. We hypothesize that an unknown genetic factor causes disturbances of cleavage of the prosencephalon as well as neuronal migration and organization.
منابع مشابه
Middle Interhemispheric Variant of Holoprosencephaly - Presenting as Non-Visualized Cavum Septum Pellucidum and An Interhemispheric Cyst in A 19-Weeks Fetus.
Middle Interhemispheric variant (MIH) is a rare subtype of holoprosencephaly (HPE), also known as syntelencephaly. We present a case of MIH, which was diagnosed as an interhemispheric cyst on antenatal sonography at 19 weeks, but later diagnosed as MIH variant of holoprosencephaly after a postabortal MRI and perinatal autopsy.
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متن کاملMiddle Interhemispheric Fusion: An Unusual Variant
PURPOSE: To describe the imaging features of a brain anomaly found on studies of three patients, and to speculate on the embryologic basis leading to the development of this abnormality. PATIENTS AND METHODS: Clinical records (three patients) , MR scans (two patients). and CT scans (two patients) of three patients with fusion of the middle portions of the cerebral hemispheres in the presence of...
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Recent advances in genetics and neuroimaging have greatly contributed to our understanding of the spectrum of midline brain and craniofacial malformations known as holoprosencephaly. Neuroradiologic studies have provided detailed characteristics of four major types of holoprosencephaly: alobar, semilobar, lobar, and middle interhemispheric variant. Clinical studies in children with these types ...
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Syntelencephaly or the middle interhemispheric variant of holoprosencephaly is a rare subtype of holoprosencephaly and consists of a nonseparation of the posterior frontal and parietal regions of both cerebral hemispheres. We report conventional MR imaging, diffusion tensor imaging, and fiber tractography findings of a girl with syntelencephaly and discuss the information that diffusion tensor ...
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ورودعنوان ژورنال:
- AJNR. American journal of neuroradiology
دوره 24 3 شماره
صفحات -
تاریخ انتشار 2003