نتایج جستجو برای: cerebellar ataxias

تعداد نتایج: 26906  

Journal: :Development 2012
Marta Florio Ketty Leto Luca Muzio Andrea Tinterri Aurora Badaloni Laura Croci Paola Zordan Valeria Barili Ilaria Albieri François Guillemot Ferdinando Rossi G Giacomo Consalez

By serving as the sole output of the cerebellar cortex, integrating a myriad of afferent stimuli, Purkinje cells (PCs) constitute the principal neuron in cerebellar circuits. Several neurodegenerative cerebellar ataxias feature a selective cell-autonomous loss of PCs, warranting the development of regenerative strategies. To date, very little is known as to the regulatory cascades controlling P...

Journal: :Archives of neurology 2006
Jack W Tsao Jason Neal Kira Apse Mark J Stephan William B Dobyns Robert S Hill Christopher A Walsh Volney L Sheen

BACKGROUND Nonprogressive cerebellar ataxias are characterized by a persistent, nonprogressive ataxia associated with cognitive impairment. Cerebellar hypoplasia on imaging is variable but is not predictive of the degree of ataxia or cognitive impairment. OBJECTIVE To describe a family with a nonprogressive cerebellar ataxia associated with cognitive and motor impairments that improve with ag...

Journal: :Annals of the Academy of Medicine, Singapore 2017
Zheyu Xu Jinglin Zhang Samuel Ye Ng Josiah Yh Chai Louis Cs Tan

Dear Editor, The initial presentation of cerebellar ataxia remains a diagnostic challenge due to its multiple etiologies, one of which is paraneoplastic cerebellar degeneration (PCD), a rare neurological disease. Although paraneoplastic antibody testing may be helpful, seronegative PCD may account for up to 50% of PCD cases.1 The causes of ataxia in the Asian population differ from that of the ...

2010
Sanjeev Rajakulendran Stephanie Schorge Dimitri M Kullmann Michael G Hanna

Mutations in the CACNA1A gene are associated with episodic ataxia type 2 (EA2) and spinocerebellar ataxia type 6 (SCA6). CACNA1A encodes the α-subunit of the P/Q-type calcium channel or Ca(V)2.1, which is highly enriched in the cerebellum. It is one of the main channels linked to synaptic transmission throughout the human central nervous system. Here, we compare recent advances in the understan...

Journal: :Functional neurology 2010
L Rinaldo C Hansel

Adaptive processes within cerebellar circuits, such as long-term depression and long-term potentiation at parallel fiber-Purkinje cell synapses, have long been seen as important to cerebellar motor learning, and yet little attention has been given to any possible significance of these processes for cerebellar dysfunction and disease. Several forms of ataxia are caused by mutations in genes enco...

2016
Emma Perkins Daumante Suminaite Mandy Jackson

Spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of disorders all characterised by postural abnormalities, motor deficits and cerebellar degeneration. Animal and in vitro models have revealed β-III spectrin, a cytoskeletal protein present throughout the soma and dendritic tree of cerebellar Purkinje cells, to be required for the maintenance of dendritic architecture and for ...

2017
Nico Mauri Miriam Kleiter Elisabeth Dietschi Michael Leschnik Sandra Högler Michaela Wiedmer Joëlle Dietrich Diana Henke Frank Steffen Simone Schuller Corinne Gurtner Nadine Stokar-Regenscheit Donal O'Toole Thomas Bilzer Christiane Herden Anna Oevermann Vidhya Jagannathan Tosso Leeb

Spongy degeneration with cerebellar ataxia (SDCA) is a genetically heterogeneous neurodegenerative disorder with autosomal recessive inheritance in Malinois dogs, one of the four varieties of the Belgian Shepherd breed. Using a combined linkage and homozygosity mapping approach we identified an ∼10.6 Mb critical interval on chromosome 5 in a Malinois family with four puppies affected by cerebel...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید