Lafora Disease [Laforova nemoc]

topical
1
Terms

nemoc Laforových tělísek s pozdním nástupem
progresivní myoklonická epilepsie Laforova typu

 

Epilepsy Progressive Myoclonic 2
Epilepsy, Progressive Myoclonic 2A
Epilepsy, Progressive Myoclonic, Lafora
Lafora Body Disease
Lafora Body Disease, Late Onset
Lafora Body Disorder
Lafora Progressive Myoclonic Epilepsy
Lafora Progressive Myoclonus Epilepsy
Lafora Type Progressive Myoclonic Epilepsy
Lafora-Body Disease, Late Onset
Late Onset Lafora Body Disease
Myoclonic Epilepsy of Lafora
Progressive Myoclonic Epilepsy Type 2
Progressive Myoclonic Epilepsy, Lafora
Progressive Myoclonic Epilepsy, Lafora Type
Progressive Myoclonus Epilepsy, Lafora Type

Persistent link   https://www.medvik.cz/link/D020192
Definition

A form of stimulus sensitive MYOCLONIC EPILEPSY inherited as an autosomal recessive condition. The most common presenting feature is a single seizure in the second decade of life. This is followed by progressive myoclonus, myoclonic seizures, tonic-clonic seizures, focal occipital seizures, intellectual decline, and severe motor and coordination impairments. Most affected individuals do not live past the age of 25 years. Concentric amyloid (Lafora) bodies are found in neurons, liver, skin, bone, and muscle (From Menkes, Textbook of Childhood Neurology, 5th ed, pp111-110).

DUI
D020192 MeSH Browser
CUI
M0328685
Previous indexing
Epilepsy (1966-1976); Epilepsies, Myoclonic (1977-1999)
History note
2000; use Epilepsy, Myoclonic 1977-1999
Public note
2000; see Epilepsy, Myoclonic 1977-1999

C Diseases
C10.228.140 Brain Diseases 1 177
C10.228.140.490 Epilepsy 3 247
C10.228.140.490.375 Epilepsy, Generalized 58
C10.228.140.490.375.130 Epilepsies, Myoclonic 45
C10.228.140.490.375.130.650 Myoclonic Epilepsies, Progressive 10
C10.228.140.490.375.130.650.500 Lafora Disease 1
C10.228.140.490.375.130.650.700 MERRF Syndrome 4
C10.228.140.490.375.130.650.900 Unverricht-Lundborg Syndrome 1
C10.228.140.490.493 Epileptic Syndromes 19
C10.228.140.490.493.063 Epilepsies, Myoclonic 45
C10.228.140.490.493.063.650 Myoclonic Epilepsies, Progressive 10
C10.228.140.490.493.063.650.500 Lafora Disease 1
C10.228.140.490.493.063.650.700 MERRF Syndrome 4
C10.228.140.490.493.063.650.900 Unverricht-Lundborg Syndrome 1
C10.574.500.024 Alexander Disease 6
C10.574.500.300 Canavan Disease 9
C10.574.500.362 Cockayne Syndrome 4
C10.574.500.497 Huntington Disease 322
C10.574.500.529 Lafora Disease 1
C10.574.500.545 Myotonia Congenita 17
C10.574.500.547 Myotonic Dystrophy 94
C10.574.500.549 Neurofibromatoses 28
C10.574.500.850 Tourette Syndrome 86
C10.574.500.865 Tuberous Sclerosis 141
C16.320.400.024 Alexander Disease 6
C16.320.400.150 Canavan Disease 9
C16.320.400.200 Cockayne Syndrome 4
C16.320.400.430 Huntington Disease 322
C16.320.400.480 Lafora Disease 1
C16.320.400.540 Myotonia Congenita 17
C16.320.400.542 Myotonic Dystrophy 94
C16.320.400.550 Neuroacanthocytosis 6
C16.320.400.560 Neurofibromatoses 28
C16.320.400.820 Tourette Syndrome 86
C16.320.400.880 Tuberous Sclerosis 141