Screening genetico per l’Epilessia dell’età evolutiva
GENE | OMIM | FENOTIPO | |
ADSL | 608222 | AD EMR | |
ALDH7A1 | 107323 | Pyridoxine-Dependent Epilepsy | |
ALG13 | 300776 | EE | |
ARHGEF9 | 300429 | EE | |
ARX | 300382 | West Syndrome | |
ATP1A2 | 182340 | FHM | |
ATP1A3 | 182350 | AHC | |
ATRX | 300032 | X-Linked EMR | |
CACNA1A | 601011 | EE, FHM with Ataxia | |
CACNA1D | 114206 | Febrile Seizures, ASD | |
CACNA1H | 607904 | Childhood Absence Seizures, GGE | |
CACNB4 | 601949 | Episodic Ataxia, Juvenile Myoclonic Epilepsy, GGE | |
CDKL5 | 300203 | EE | |
CHD2 | 602119 | EE | |
CHRNA2 | 118502 | ADNFLE | |
CHRNA4 | 118504 | ADNFLE | |
CHRNB2 | 118507 | ADNFLE | |
CLCN2 | 600570 | GGE | |
CNKSR2 | 300724 | LKS, ESES | |
CPA6 | 609562 | FFE | |
DEPDC5 | 614191 | Focal Epilepsy, FFEVF | |
DNM1 | 602377 | EE | |
EEF1A2 | 602959 | EIEE, AD MR | |
FASN | 600212 | ID, LGS, EE | |
FOXG1 | 164874 | West Syndrome | |
GABBR1 | 603540 | EE | |
GABBR2 | 607340 | EE | |
GABRA1 | 137160 | Dravet | |
GABRA5 | 137142 | EE | |
GABRB3 | 137192 | EE, Febrile Seizures, GEFS+ | |
GABRD | 137163 | Dravet, GEFS+ | |
GABRG2 | 137164 | Dravet, GEFS+ | |
GAMT | 601240 | Creatine Deficiency | |
GATM | 602360 | Creatine Deficiency | |
GNAO1 | 139311 | EE | |
GRIN1 | 138249 | EE | |
GRIN2A | 138253 | Focal Epilepsy, ESES | |
GRIN2B | 138252 | West Syndrome | |
GRIN2D | 602717 | ||
HCN1 | 602780 | EE | |
HDAC4 | 605314 | EE | |
HNRNPU | 602869 | EE | |
HUWE1 | 300697 | EMR (LKS like phenotype) | |
IQSEC2 | 300522 | EE | |
KCNA2 | 176262 | EE | |
KCNB1 | 600397 | EIEE | |
KCND2 | 605410 | Seizures, Autism | |
KCNH5 | 605716 | EE | |
KCNQ2 | 602235 | BFNC, EE | |
KCNQ3 | 602232 | BFNC | |
KCNT1 | 608167 | Focal Epilepsy | |
KCTD7 | 611725 | EE | |
KIAA2022 | 300524 | X-Linked MR | |
LGI1 | 604619 | Focal Epilepsy, ADLTE | |
MECP2 | 300005 | EE, Rett Syndrome | |
MEF2C | 600662 | West Syndrome | |
MTOR | 601231 | EE | |
NPRL2 | 607072 | Focal Epilepsy, FFEVF | |
NPRL3 | 600928 | Focal Epilepsy, FFEVF | |
NRXN1 | 600565 | Pitt-Hopkins-like Syndrome | |
PCDH19 | 300460 | EFMR | |
PIGA | 311770 | X-Linked EE | |
PIGO | 614730 | GPI Anchor Deficiency | |
PIGT | 610272 | GPI Anchor Deficiency | |
PIK3AP1 | 607942 | West Syndrome | |
PIK3R2 | 603157 | MPPHS | |
PLCB1 | 607120 | MMPSI | |
PNKP | 605610 | NEE/OS | |
PNPO | 603287 | Pyridoxine-Dependent Epilepsy | |
POLG | 174763 | OCCIP, Alper’s Syndrome, CSE, SV Tox | |
PRRT2 | 614386 | PKD, PKD+BFIS, BFIS | |
PURA | 600473 | EE | |
RELN | 600514 | ADLTE | |
SCN1A | 182389 | Dravet, FHM, GEFS+ MMPSI | |
SCN1B | 600235 | Dravet, GEFS+ | |
SCN2A | 182390 | BFNC, EE | |
SCN8A | 600702 | EE | |
SLC12A5 | 606726 | EIEE, GGE | |
SLC13A5 | 608305 | EIEE | |
SLC1A2 | 600300 | EE | |
SLC25A22 | 609302 | EE | |
SLC2A1 | 138140 | EOAE, GGE, PED | |
SLC35A2 | 314375 | EE | |
SLC35A3 | 605632 | EE | |
SLC6A1 | 137165 | EE, MAE/Doose Syndrome | |
SLC6A8 | 300036 | Creatine Deficiency | |
SLC9A6 | 300231 | ID, Epilepsy | |
SMARCA2 | 600014 | NBS | |
SPTAN1 | 182810 | West Syndrome | |
ST3GAL3 | 606494 | EIEE, AR MR | |
STX1B | 601485 | GEFS+ | |
STXBP1 | 602926 | EE | |
SYNGAP1 | 603384 | EE | |
SZT2 | 615463 | EIEE | |
TBC1D24 | 613577 | EE, MMPSI | |
TCF4 | 602272 | Pitt Hopkins Syndrome | |
UBE3A | 601623 | Angelman Syndrome | |
WWOX | 605131 | EIEE | |
ZDHHC9 | 300646 | X-Linked ID, Rolandic Epilepsy |
Lista delle abbreviazioni:
ADLTE: Epilessia del lobo temporale laterale autosomica dominante
ADNFLE: Epilessia del lobo frontale notturno autosomica dominante
AHC: Hemiplegia alternante dell’infanzia
AD: Autosomica dominante
BFIS: convulsioni infantili familiari benigne
BFNC: convulsioni neonatali familiari benigne
CSE: Convulsive Status Epilepticus
CSWS: picchi e onde continui durante il sonno lento
EE: Encefalopatia epilettica
EFMR: epilessia con ritardo mentale limitata alle femmine
EIEE: Encefalopatia epilettica infantile precoce
EMR: Epilessia con ritardo mentale
EOAE: Epilessia di assenza precoce
ESES: stato elettrico Epilettico durante il sonno
FFE: epilessia focale familiare
FFEVF: epilessia focale familiare con foche variabili
FHM: emicrania emiplegica familiare
GEFS +: Epilessia genetica generalizzata con convulsioni febbrili plus
GGE: Epilessia generalizzata genetica
GPI: glicosilfosfatidilinositolo
ICCA: PKD combinato con convulsioni infantili
ID: Disabilità intellettuale
LGS: sindrome di Lennox-Gastaut
LKS: Sindrome di Landau – Kleffner
MAE: epilessia mioclonica-astatica
MPPHS: Sindrome da megalencefalia-polimicrogiria-polidattilia-idrocefalo
MMPSI: Sequestri parziali migratori maligni dell’infanzia
NBS: Sindrome di Nicolaides-Baraitser
NEE: Encefalopatia epilettica neonatale
OCCIP: convulsioni occipitali
OS: Sindrome di Ohtahara
PED: discinesia da sforzo parossistico
PKD: discinesia kinesigenica parossistica
SV Tox: Tossicità epatica indotta da sodio valporato
West = Sindrome di West