نتایج جستجو برای: ataxia oculomotor apraxia 1 aoa1

تعداد نتایج: 2770963  

Journal: :Sudanese journal of paediatrics 2016
Haifa A Bin Dahman Abdul-Hakeem M Bin Mubaireek Zain H Alhaddad

Joubert syndrome is a rare autosomal recessive disorder. It is characterized by congenital ataxia, hypotonia, developmental delay and at least one of the following features: neonatal respiratory disturbances and abnormal eye movements; including nystagmus and oculomotor apraxia. Molar tooth appearance is an essential finding for the diagnosis of Joubert syndrome. We report a five-days-old newbo...

2012
Amaya Alzu Rodrigo Bermejo Martina Begnis Chiara Lucca Daniele Piccini Walter Carotenuto Marco Saponaro Alessandra Brambati Andrea Cocito Marco Foiani Giordano Liberi

Transcription hinders replication fork progression and stability. The ATR checkpoint and specialized DNA helicases assist DNA synthesis across transcription units to protect genome integrity. Combining genomic and genetic approaches together with the analysis of replication intermediates, we searched for factors coordinating replication with transcription. We show that the Sen1/Senataxin DNA/RN...

Journal: :Arquivos de neuro-psiquiatria 2010
Pedro Braga-Neto Lívia Almeida Dutra José Luiz Pedroso Orlando Graziani Povoas Barsottini

MD, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo (UNIFESP), São Paulo SP, Brazil; MD, PhD, Department of Neurology and Neurosurgery, UNIFESP. Alpha-fetoprotein (AFP) is a fetal protein produced in the yolk sac and in the fetal liver. AFP has been implicated in both ontogenic and oncogenic growth disorders. Although the main biologic role of AFP during pregnancy re...

Journal: :Internal medicine 2014
Makoto Tokunaga Kimiko Fukunaga Ryoji Nakanishi Susumu Watanabe Hiroaki Yamanaga

We herein report the case of an 81-year-old woman with midbrain infarction causing pupil-sparing oculomotor nerve palsy with ipsilateral cerebellar ataxia. The lesion was located at the rostral end of the decussation of the superior cerebellar peduncle touching the dorsal side, further caudal and dorsal to causal lesions of Claude's syndrome, which presented as oculomotor palsy and contralatera...

Journal: :Archives of Neurology 2001

Journal: :Journal of medical genetics 1994
M A Hannan D Sigut M Waghray G G Gascon

Although ataxia-ocular motor apraxia (AOA) has been described as a disease entity mimicking ataxia telangiectasia (AT), no radiobiological studies have been carried out on cells from patients with AOA to find their possible relationship to AT. In the present study, cultured fibroblasts from three patients with AOA and their asymptomatic relatives (parents and sibs) were, therefore, compared wit...

Journal: :AJNR. American journal of neuroradiology 1995
E A Whitsel M Castillo O D'Cruz

The MR imaging findings in two patients with the clinical diagnosis of oculomotor apraxia are presented. Both cases showed dysgenesis of the cerebellar vermis, and the colliculi were fused in one patient. No supratentorial abnormalities were seen in either patient.

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