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Unverricht's disease
Also known as:
Baltic myoclonus
Lafora’s syndrome
Lafora's disease
Lafora-Unverricht syndrome
Unverricht-Lundborg disease
Unverricht-Lundborg syndrome
Unverricht-Lafora disease
Unverricht-Lundborg-Lafora syndrome
Associated persons:
Gonzalo Rodriguez Lafora
Herman Bernhard Lundborg
Heinrich Unverricht
Description:
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,
Bibliography:
- 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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