Affiliation:
1. Universidade Federal do Rio de Janeiro; Center for Myopathies Investigation and Treatment
2. Instituto de Neurologia Deolindo Couto; Center for Myopathies Investigation and Treatment
Abstract
This study presents the performance of 39 cases of myotonic dystrophy on Kohs' blocks test (21 females and 18 males, age range from 9 to 70 years). On this test, the patients have to reproduce figures from models previously showed to them. Some of the patients had some kind of professional activity, while others had never exerted a professional occupation. The patients denoted considerable difficulty to perform the test. Some cases constructed entirely different figures in comparison to the presented drawings, translating visuo-spatial and constructional disabilities. The performance was insufficient in 71.4 % of the cases. These cases solved less than 50% of the test. The levels of analysis and synthesis were severely impaired. A total of 18 cases got less than 10 points, not reaching 20% of the test. The results showed the sensitivity of this test in detecting visuo-spatial impairment in myotonic dystrophy.
Subject
Neurology,Clinical Neurology
Reference42 articles.
1. Detection on an unstable fragment of 1DNA specific to individuals with myotonic dystrophy;Buxton J;Nature,1992
2. Triplet repeats mutations in human disease;Caskey CT;Science,1992
3. Distrofia miotônica: atualização;Duro LAA;Rev Bras Neurol,1993
4. Structure and expression of the myotonic muscular dystrophy gene;Fu Y-H;Am J Hum Genet,1992
5. Expansion of an unstable DNA region and phenotypic variation in myotonic dystrophy;Harley HG;Nature,1992
Cited by
15 articles.
订阅此论文施引文献
订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献