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Neuroradiology

, Volume 34, Issue 6, pp 483–486 | Cite as

Tay-Sachs disease: progression of changes on neuroimaging in four cases

  • M. Fukumizu
  • H. Yoshikawa
  • S. Takashima
  • N. Sakuragawa
  • T. Kurokawa
Diagnostic Neuroradiology

Summary

The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter.

Key words

Tay-Sachs disease Basal ganglia Leukodystrophic change Magnetic resonance imaging Positron emission tomography 

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Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • M. Fukumizu
    • 1
  • H. Yoshikawa
    • 1
  • S. Takashima
    • 2
  • N. Sakuragawa
    • 3
  • T. Kurokawa
    • 1
  1. 1.Division of Child NeurologyNational Center Hospital for Mental, Nervous, and Muscular DisordersTokyoJapan
  2. 2.Division of Mental Retardation and Birth Defect ResearchNational Institute of Neuroscience, National Center of Neurology and PsychiatryTokyoJapan
  3. 3.Division of Inherited Metabolic DiseaseNational Institute of Neuroscience, National Center of Neurology and PsychiatryTokyoJapan

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