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Nyssen-van Bogaert syndrome

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Multisystemic CNS-degenerations with congenital degeneration of the optic, cochlear, dentate, and medial lemniscal structures, characterized by optic atrophy with blindness, deafness, defective speech, spasticity, hyperkinetic behaviour, contractures, mental deterioration, and death before the age of 10 years. Inheritance is probably autosomal recessive. First described by Nyssen and van Bogaert in 1934, expanded with further system degenerations by J. Meyer in 1949.


  • R. Nyssen, L. van Bogaert:
    La dégénérescence systématisée optico-cochléo-dentelée.
    Revue neurologique, Paris, 1934, 2: 321-345.
  • J. E. Meyer:
    Über eine kombinierte Systemerkrankung in Klein-, Mittel- und Endhirn.
    Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1949, 182: 731-758.

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