Pediatric Neurology
Volume 26, Issue 1 , Pages 51-54 , January 2002

Juvenile dentatorubral-pallidoluysian atrophy: new clinical features

  • Daniel J Licht, MD

      Affiliations

    • Corresponding Author InformationCommunications should be addressed to: Dr. Licht; Division of Neurology, 6th Floor Wood Bldg; Children’s Hospital of Philadelphia; Philadelphia, PA 19104 USA
    • Division of Child Neurology, The Children’s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    • ,
  • David R Lynch, MD, PhD

      Affiliations

    • Division of Child Neurology, The Children’s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    • Department of Neurology, University of Pennsylvania, Philadelphia, PA 19104, USA

Received 27 March 2001 ,Accepted 9 July 2001.

References 

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  2. Tsuji S. Dentatorubral-pallidoluysian atrophy (Clinical features and molecular genetics). Adv Neurol. 1999;79:399–409
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  9. Basu P, Chattopadhyay B, Gangopadhaya PK, et al.  Analysis of CAG repeats in SCA1, SCA2, SCA3, SCA6, SCA7 and DRPLA loci in spinocerebellar ataxia patients and distribution of CAG repeats at the SCA1, SCA2 and SCA6 loci in nine ethnic populations of eastern India. Hum Genet. 2000;106:597–604
  10. Tang B, Chunyu L, Shen L, et al.  Frequency of SCA1, SCA2, SCA3/MJD, SCA6, SCA7 and DRPLA CAG trinucleotide repeat expansion in patients with hereditary spinocerebellar ataxia from Chinese kindreds. Arch Neurol. 2000;57:540–544
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  13. Shimohata T, Ishiguro H, Makino K, et al.  Sporadic cases of dentatorubral-pallidoluysian atrophy associated with maternal transmission. Neurology. 1998;50:282–283
  14. Wada Y, Matsuoka T, Imai K, et al.  A case of juvenile-type DRPLA with psychomotor retardation since infancy. No To Hattatsu. 1998;30:543–548
  15. Hattori M, Yuasa H, Takada K, et al.  Genetic analysis of a DRPLA family (Relevance to apparent sporadic cases). Intern Med. 1999;38:287–289
  16. Imamura A, Ito R, Tanaka S, et al.  High-intensity proton and T2-weighted MRI signals in the globus pallidus in juvenile-type of dentatorubral and pallidoluysian atrophy. Neuropediatrics. 1994;25:234–237
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PII: S0887-8994(01)00346-0

Pediatric Neurology
Volume 26, Issue 1 , Pages 51-54 , January 2002

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