نتایج جستجو برای: spastic paraplegia

تعداد نتایج: 11676  

2017
Jennifer Gass Patrick R Blackburn Jessica Jackson Sarah Macklin Jay van Gerpen Paldeep S Atwal

Hereditary spastic paraplegia is a group of clinically and genetically heterogeneous neurodegenerative disorders, often characterized by weakness and spasticity in the lower limbs. In our study, we describe a spastic paraplegia type 7 patient with an expanded phenotype who was diagnosed after the discovery of pathogenic variants in SPG7.

Journal: :The Turkish journal of pediatrics 2017
Gonca Bektaş Gözde Yeşil Edibe Pembegül Yıldız Nur Aydınlı Mine Çalışkan Meral Özmen

Bektaş G, Yeşil G, Yıldız EP, Aydınlı N, Çalışkan M, Özmen M. Hereditary spastic paraplegia type 35 caused by a novel FA2H mutation. Turk J Pediatr 2017; 59: 329-334. Hereditary spastic paraplegia type 35 (SPG35) is a rare disorder characterized by progressive spasticity. Mutations in the fatty acid 2-hydroxylase (FA2H) gene in different loci are responsible for phenotypic variability. We aimed...

Journal: :Archives of Neurology 2004

Journal: :Proceedings of the Royal Society of Medicine 1911

Journal: :The Dublin Journal of Medical Science 1907

Journal: :Archives of neurology 2006
Shirley Rainier Carron Sher Orit Reish Donald Thomas John K Fink

BACKGROUND Mutations in the SPG3A gene (atlastin protein) cause approximately 10% of autosomal-dominant hereditary spastic paraplegia. For many subjects with an SPG3A mutation, spastic gait begins in early childhood and does not significantly worsen even over many years. Such subjects resemble those with spastic diplegic cerebral palsy. To date, only 9 SPG3A mutations have been reported. OBJE...

Journal: :Brain & development 2010
Carlo Fusco Daniele Frattini Enrico Farnetti Davide Nicoli Bruno Casali Francesco Fiorentino Andrea Nuccitelli Elvio Della Giustina

Mutations in the SPG3A gene (atlastin protein) cause approximately 10% of autosomal-dominant hereditary spastic paraplegia. Most patients with an SPG3A mutation present with a pure phenotype and early-onset disease, although complicated forms with peripheral neuropathy are also reported. We report a new heterozygous S398F mutation in exon 12 of the SPG3A gene causing a very early-onset spastic ...

2016
Eleanna Kara Arianna Tucci Claudia Manzoni David S. Lynch Marilena Elpidorou Conceicao Bettencourt Viorica Chelban Andreea Manole Sherifa A. Hamed Nourelhoda A. Haridy Monica Federoff Elisavet Preza Deborah Hughes Alan Pittman Zane Jaunmuktane Sebastian Brandner Georgia Xiromerisiou Sarah Wiethoff Lucia Schottlaender Christos Proukakis Huw Morris Tom Warner Kailash P. Bhatia L.V. Prasad Korlipara Andrew B. Singleton John Hardy Nicholas W. Wood Patrick A. Lewis Henry Houlden

The hereditary spastic paraplegias are a heterogeneous group of degenerative disorders that are clinically classified as either pure with predominant lower limb spasticity, or complex where spastic paraplegia is complicated with additional neurological features, and are inherited in autosomal dominant, autosomal recessive or X-linked patterns. Genetic defects have been identified in over 40 dif...

2011
Sanjeev Rajakulendran Coro Paisán-Ruiz Henry Houlden

BACKGROUND Mutations in the spatacsin gene are associated with spastic paraplegia type 11 (SPG11), which is the most-common cause of autosomal recessive hereditary spastic paraplegia. Although SPG11 has diverse phenotypes, thinning of the corpus callosum is an important feature. CASE REPORT Clinical, genetic, and radiological evaluations were undertaken in a large family from Gujarat in North...

2013
Ji Seon Kim Jong Min Kim Yu Kyeong Kim Sang Eun Kim Ji Young Yun Beom S. Jeon

Sporadic spastic paraplegia (SSP) and hereditary spastic paraplegia (HSP) belong to a clinical and genetically heterogeneous group of disorders characterized by progressive spasticity and weakness in the lower extremities. The symptoms are associated with pyramidal tract dysfunction and degeneration of the corticospinal tracts. Parkinsonism is uncommon in SSP/HSP patients. However, both disorde...

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