Literature DB >> 2073300

Cortical atrophy and cognitive performance in infantile nephropathic cystinosis.

S L Nichols1, G A Press, J A Schneider, D A Trauner.   

Abstract

A group of children and adolescents with infantile nephropathic cystinosis underwent cognitive testing and were examined for cortical atrophy using magnetic resonance imaging or computed tomography. Ten of 11 patients demonstrated cortical atrophy. A consistent pattern of lower cognitive performance was found in patients with greater atrophy; however, only the relationship between atrophy and short-term memory approached statistical significance. In addition, evidence for greater impairment of visual memory than of other cognitive functions was observed. This latter observation did not appear to be related to the degree of atrophy.

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Year:  1990        PMID： 2073300     DOI： 10.1016/0887-8994(90)90004-k

Source DB:  PubMed          Journal:  Pediatr Neurol        ISSN： 0887-8994            Impact factor:   3.372


  20 in total

1.  Visual and verbal learning in a genetic metabolic disorder.

Authors:  Amy M Spilkin; Angela O Ballantyne; Doris A Trauner
Journal:  Neuropsychologia       Date:  2009-03-09       Impact factor: 3.139

2.  Hierarchical processing of visual stimuli in nephropathic cystinosis.

Authors:  Aakash Sathappan; Doris Trauner
Journal:  J Inherit Metab Dis       Date:  2019-02-19       Impact factor: 4.982

3.  Intellectual and motor performance, quality of life and psychosocial adjustment in children with cystinosis.

Authors:  Francis F Ulmer; Markus A Landolt; Russia Ha Vinh; Thierry A G M Huisman; Thomas J Neuhaus; Bea Latal; Guido F Laube
Journal:  Pediatr Nephrol       Date:  2009-03-18       Impact factor: 3.714

4.  Executive function in nephropathic cystinosis.

Authors:  Angela O Ballantyne; Amy M Spilkin; Doris A Trauner
Journal:  Cogn Behav Neurol       Date:  2013-03       Impact factor: 1.600

5.  Neurological impairment in nephropathic cystinosis: motor coordination deficits.

Authors:  Doris A Trauner; Jennifer Williams; Angela O Ballantyne; Amy M Spilkin; Jennifer Crowhurst; John Hesselink
Journal:  Pediatr Nephrol       Date:  2010-07-22       Impact factor: 3.714

6.  Clinical polymorphism of cystinosis encephalopathy. Results of treatment with cysteamine.

Authors:  M Broyer; M J Tête; G Guest; J P Berthélémé; F Labrousse; M Poisson
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

Review 7.  Nephropathic cystinosis: late complications of a multisystemic disease.

Authors:  Galina Nesterova; William Gahl
Journal:  Pediatr Nephrol       Date:  2008-06       Impact factor: 3.714

8.  Effects of cysteamine on oxidative status in cerebral cortex of rats.

Authors:  Adriana Kessler; Micheli Biasibetti; Luciane Rosa Feksa; Virginia Cielo Rech; Denizar Alberto da Silva Melo; Moacir Wajner; Carlos Severo Dutra-Filho; Angela Terezinha de Souza Wyse; Clovis Milton Duval Wannmacher
Journal:  Metab Brain Dis       Date:  2007-11-21       Impact factor: 3.584

9.  Developmental changes in cerebral white matter microstructure in a disorder of lysosomal storage.

Authors:  Sunita Bava; Rebecca J Theilmann; Miriam Sach; Susanne J May; Lawrence R Frank; John R Hesselink; Duc Vu; Doris A Trauner
Journal:  Cortex       Date:  2009-04-05       Impact factor: 4.027

10.  Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex.

Authors:  Virginia Cielo Rech; Luciane Rosa Feksa; Rochele Marisa Müller Fleck; Genaro Azambuja Athaydes; Paula Karina Barcelos Dornelles; Valnes Rodrigues-Junior; Clovis Milton Duval Wannmacher
Journal:  Metab Brain Dis       Date:  2008-04-17       Impact factor: 3.584
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