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Unverricht's disease

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A fatal form of progressive myoclonic epilepsy with onset between the ages of 6 and 13, marked by seizures and associated with myoclonus, precipitated by slight stimulus. Mental deterioration is milder and survival is longer than in Lafora's myoclonic epilepsy. Inheritance is autosomal recessive. Consanguinity of parents frequent. It is essentially the same as Lafora’s disease, but without the presence of Lafora’s bodies. Lundborg described the disease in a family in Listerlandet, Blekinge. He traced the family back to the 1700s,


  • H. Unverricht:
    Die Myoclonie. Wien, F. Deutiche, 1891.
  • H. B. Lundborg:
    Die progressive Myoklonus-Epilepsie (Unverricht's Myoklonie).
    Uppsala : Almqvist & Wiksell, 1903. 207 pages.
  • G. R. Lafora:
    Über das Vorkommen amyloider Körperchen im Inneren der Ganglienzellen; ein Beitrag zum Studium der amyloiden Substanz im Nervensystem.
    [Virchows] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, Berlin, 1911, 205: 295-303.
  • G. R. Lafora:
  • Beitrag zur Histopathologie der myoklonischen Epilepsie.
    Zeitschrift für die gesamte Neurologie und Psychiatrie, 1911, 6.
  • G. A. Schwartz, M. Yanoff:
    Lafora's disease. Distinct clinico-pathologic form of Unverricht's syndrome.
    Archives of Neurology, Chicago, February 1965, 12: 172-188.

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