نتایج جستجو برای: pachygyria

تعداد نتایج: 105  

Journal: :Russkij žurnal detskoj nevrologii 2022

Malformations of the cerebral cortex are often causes epilepsy. The latest changes in their classification summarized. description lissencephaly and Miller–Dicker syndrome, pachygyria, polymicrogyria, hemimegaloencephaly, holoprosencephaly, schizencephaly, gray matter heterotopia is given. features epilepsy these diseases described. Magnetic resonance imaging scans for focal cortical dysplasia,...

2014

Dandy-Walker malformation is characterised by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. Approximately 70-90% of patients have hydrocephalus. Lissencephaly is characterised by absence (agyria) or incomplete development (pachygyria) of the gyri of the cerebral cortex, causing the brain's surface to appear un...

2014
S Bhatnagar R Kuber D Shah VM Kulkarni

Schizencephaly is a rare malformation of the central nervous system. Both genetic and non-genetic etiologies like prenatal infections or ischemia have been postulated. Clinical manifestations most often include varying degrees of developmental delay, motor impairment and seizures. It can be associated with septo-optic dysplasia (SOD), optic nerve hypoplasia and absence of septum pellucidum, pac...

Journal: :Endokrynologia Polska 2010
Robert Smigiel Aleksander Basiak Blazej Misiak Karolina Pesz

We report an 8-year-old proband with severe motor and intellectual disability presenting a variety of dysmorphic features such as microcephaly, prominent glabella (ridged metopic suture) and congenital distal limb contractures. As well as panhypopituitary insufficiency, brain defects, e.g. agenesis of corpus callosum, colpocephaly, and pachygyria as well as strabismus and tracheo-laryngeal hypo...

Journal: :Brain : a journal of neurology 2002
Maria Daniela D'Agostino Andrea Bernasconi Soma Das Alexandre Bastos Rosa M Valerio André Palmini Jaderson Costa da Costa Ingrid E Scheffer Samuel Berkovic Renzo Guerrini Charlotte Dravet Jiro Ono GianLuigi Gigli Antonio Federico Fran Booth Bruno Bernardi Lilia Volpi Carlo Alberto Tassinari Mary Anne Guggenheim David H Ledbetter Joseph G Gleeson Iscia Lopes-Cendes David G Vossler Elisabetta Malaspina Emilio Franzoni Roberto J Sartori Michael H Mitchell Suha Mercho François Dubeau Frederick Andermann William B Dobyns Eva Andermann

Subcortical band heterotopia (SBH) or double cortex syndrome is a neuronal migration disorder, which occurs very rarely in males: to date, at least 110 females but only 11 in males have been reported. The syndrome is usually associated with mutations in the doublecortin (DCX) (Xq22.3-q23) gene, and much less frequently in the LIS1 (17p13.3) gene. To determine whether the phenotypic spectrum, th...

2011
Himanshu Aneja

A five month girl with extreme microcephaly and complex brain malformation is reported. Magnetic resonance imaging (MRI) revealed bilateral thickened smooth cortex with diffuse agyria-pachygyria spectrum, agenesis of corpus callosum, bilateral hypoplastic cerebellar hemispheres and ventriculomegaly with Dandy-Walker malformation. By adding our case in the list of similar case reports by László ...

Journal: :AJNR. American journal of neuroradiology 1996
C Y Chen R A Zimmerman S Faro B Parrish Z Wang L T Bilaniuk T Y Chou

PURPOSE To evaluate abnormalities of the cerebral operculum in infants and children and to propose the embryogenic basis of abnormal opercular formation as determined from MR imaging findings. METHODS Eighty-six infants and children who had abnormally wide interopercular distances and/or distorted opercular topography seen on MR images were studied retrospectively. Clinically, patients presen...

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