نتایج جستجو برای: lissencephaly

تعداد نتایج: 686  

Journal: :BMJ case reports 2015
Adeel Ejaz Syed

To cite: Syed AE. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014206522 DESCRIPTION A 5-month-old full-term male patient, born of a nonconsanguineous marriage presented to A&E department following parental concerns about abnormal movements and developmental regression. Development halted at 4 months of age. The patient increasingly showed episodes with flicke...

Journal: :Journal of medical genetics 1998
J E Allanson D H Ledbetter W B Dobyns

Both Miller-Dieker syndrome and isolated lissencephaly sequence are associated with classical lissencephaly. Both have been shown to be associated with deletions and mutations in LIS1 on 17p. Traditionally, the two disorders have been distinguished by the presence of a characteristic facial appearance in Miller-Dieker syndrome. The forehead is tall and prominent and may have vertical furrowing....

1999
Zhao Liu Ting Xie Ruth Steward

Lissencephaly is a congenital brain malformation manifested by a smooth cerebral surface due to incomplete neuronal migration (Dobyns, 1989). Type I lissencephaly occurs either as an isolated abnormality or in association with dysmorphic facial appearance in Miller-Dieker syndrome (MDS; Miller, 1963; Dieker, 1967). Lissencephaly patients usually die young with profound mental retardation, muscl...

1999
Zhao Liu Ting Xie Ruth Steward

Lissencephaly is a congenital brain malformation manifested by a smooth cerebral surface due to incomplete neuronal migration (Dobyns, 1989). Type I lissencephaly occurs either as an isolated abnormality or in association with dysmorphic facial appearance in Miller-Dieker syndrome (MDS; Miller, 1963; Dieker, 1967). Lissencephaly patients usually die young with profound mental retardation, muscl...

2013
Valentín Pérez Aroa Suárez-Vega Miguel Fuertes Julio Benavides Laetitia Delgado M Carmen Ferreras Juan José Arranz

BACKGROUND Lissencephaly is a rare developmental brain disorder in veterinary and human medicine associated with defects in neuronal migration leading to a characteristic marked reduction or absence of the convolutional pattern of the cerebral hemispheres. In many human cases the disease has a genetic basis. In sheep, brain malformations, mainly cerebellar hypoplasia and forms of hydrocephalus,...

2014
Catherine Fallet-Bianco Annie Laquerrière Karine Poirier Ferechte Razavi Fabien Guimiot Patricia Dias Laurence Loeuillet Karine Lascelles Cherif Beldjord Nathalie Carion Aurélie Toussaint Nicole Revencu Marie-Claude Addor Benoit Lhermitte Marie Gonzales Jelena Martinovich Bettina Bessieres Maryse Marcy-Bonnière Frédérique Jossic Pascale Marcorelles Philippe Loget Jamel Chelly Nadia Bahi-Buisson

Complex cortical malformations associated with mutations in tubulin genes are commonly referred to as "Tubulinopathies". To further characterize the mutation frequency and phenotypes associated with tubulin mutations, we studied a cohort of 60 foetal cases. Twenty-six tubulin mutations were identified, of which TUBA1A mutations were the most prevalent (19 cases), followed by TUBB2B (6 cases) an...

Journal: :American Journal of Medical Genetics Part A 2017

Journal: :Quantitative Imaging in Medicine and Surgery 2018

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