Amish, Mennonite, and Hutterite
Genetic Disorder Database

Epilepsy, generalized, with febrile seizures plus, type 1

Disorder
OMIM #: #604233  (Click to access OMIM database)
Disorder: Epilepsy, generalized, with febrile seizures plus, type 1 
Also known as: GEFS+, TYPE 1
GEFS+1 
Clinical
Phenotype: febrile seizures, later development of afebrile seizures, generalized seizures, absence seizures, myotonic seizures, atonic seizures 
Seen In: Amish
Old Order Mennonite
Old Colony Mennonite
Unknown/Other Mennonite
Hutterite
Remarks: Autosomal dominant 
Mutations
1   Old Order Mennonite  
Gene: SCN1B
Base Change: G>A, at nucleotide 350
Amino Acid Change: gly 117 --> asp
2   Old Order Mennonite  
Gene: SCN1B
Base Change: c.305_313delAGGATCTGT
Amino Acid Change: p.(Gln102_Ser105delinsPro)
Last updated: 2022-11-03 

References
Strauss KA, Gonzaga-Jauregui C, Brigatti KW, Williams KB, King AK, Van Hout C, Robinson DL, Young M, Praveen K, Heaps AD, Kuebler M, Baras A, Reid JG, Overton JD, Dewey FE, Jinks RN, Finnegan I, Mellis SJ, Shuldiner AR, Puffenberger EG. (2018) Genomic diagnostics within a medically underserved population: efficacy and implications. Genet Med Jan;20(1):31-41.
PubMed ID: 28726809 

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