
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
0000014935 |
diagnosis: epileptic encephalopathy and pervasive developmental disorder |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Lab Zuffardi |
00016322 |
0000014940 |
epileptic encephalopathy with severe cognitive impairment |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Lab Zuffardi |
00016327 |
0000014941 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Lab Zuffardi |
00016328 |
0000033427 |
Not Rett synd. - epileptic encephalopathy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
RettBASE |
00043144 |
0000054365 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Dong Li |
00074599 |
0000061147 |
see paper; ..., intellectual disability, epilepsy; two regression in adolescence, deceased (16y, 22y) presumed sudden unexpected death in epilepsy |
intellectual disability |
- |
Familial, X-linked dominant |
- |
- |
- |
- |
- |
Cheryl Shoubridge |
00081545 |
0000068588 |
early-onset epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089198 |
0000068589 |
early myoclonic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089199 |
0000068590 |
epilepsy with myoclonic-atonic seizures |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089200 |
0000068591 |
Dravet syndrome like, seggregates with Generalized epilepsy with febrile seizures plus |
- |
- |
Familial |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089201 |
0000068592 |
epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089202 |
0000068593 |
epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089203 |
0000068594 |
early-onset epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089204 |
0000068595 |
early-onset epileptic encephalopathy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089205 |
0000068596 |
Lennox-Gastaut syndrome |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089206 |
0000068597 |
epilepsy of infancy with migrating focal seizures |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089207 |
0000068598 |
early-onset epileptic encephalopathy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089208 |
0000068599 |
early-onset epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089209 |
0000068600 |
early-onset epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089210 |
0000068601 |
early-onset epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089211 |
0000068602 |
epileptic encephalopathy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089212 |
0000068603 |
movement disorder; developmental epileptic encephalopathy; 9d-onset epilepsy; severe developmental delay |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089213 |
0000068604 |
symptomatic generalized epilepsy |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089214 |
0000068605 |
early-onset epileptic encephalopathy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00089215 |
0000079003 |
see paper; partial status epilepticus characterized by right hemibody clonus and impaired consciousness 2w following Diptheria, Tetanus, and Pertussis (DTaP) booster, ... |
- |
- |
Isolated (sporadic) |
04y |
- |
04y |
partial status epilepticus |
- |
Johan den Dunnen |
00100789 |
0000079004 |
see paper; 5y-healthy typically developing, except mild expressive speech delay (dysarthria), presented suddenly with focal status epilepticus/encephalopathy after minor head trauma (collision with other child) without loss of consciousness and normal head CT, ... |
- |
- |
Isolated (sporadic) |
- |
- |
- |
mild expressive speech delay |
- |
Johan den Dunnen |
00100790 |
0000079005 |
see paper; born at term; normal development until 5m when developed seizures, developmental regression, MRI brain revealed progressive volume loss, demyelination; 14m-global developmental delay, hypotonia, status epilepticus; 5y-died from severe status epilepticus with respiratory failure; ... |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00100791 |
0000079006 |
see paper; born 37w3d, pregnancy
complicated by intrauterine growth restriction, hydrocephalus; 4d persistent lactic acidosis; died 10 m from pneumonia; .... |
- |
- |
Isolated (sporadic) |
- |
- |
00y00m04d |
- |
- |
Johan den Dunnen |
00100792 |
0000141818 |
HP:0008936
HP:0002273
HP:0000486
HP:0000617
HP:0010804 |
- |
EIEE-1 |
Familial, X-linked recessive |
- |
- |
00y06m |
- |
- |
Anaïs Begemann |
00177002 |
0000141820 |
Muscular hypotonia of the trunk (HP:0008936); Spastic paraparesis (HP:0002313); Horizontal pendular nystagmus (HP:0007811); Esotropia (HP:0000565); Abnormality of ocular smooth pursuit (HP:0000617) |
- |
- |
Familial, autosomal recessive |
- |
- |
00y07m |
- |
- |
Anaïs Begemann |
00176985 |
0000141821 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
HP:0003623 |
- |
Anaïs Begemann |
00177003 |
0000141822 |
HP:0000639
HP:0000817
HP:0000297
HP:0008936
HP:0000486
HP:0001285
HP:0003781
HP:0002015
HP:0002705 |
- |
- |
Familial, X-linked dominant |
- |
- |
00y01m |
- |
- |
Anaïs Begemann |
00177004 |
0000141823 |
HP:0008936
HP:0000490
HP:0011228
HP:0001763
HP:0001053 |
- |
- |
Familial, autosomal dominant |
- |
- |
02y04m |
- |
- |
Anaïs Begemann |
00177005 |
0000141824 |
HP:0008936
HP:0002078
HP:0002066
HP:0002522
HP:0001761
HP:0000154
HP:0004533 |
- |
- |
Familial, autosomal recessive |
- |
- |
01y06m |
- |
- |
Anaïs Begemann |
00177006 |
0000141825 |
HP:0001252
HP:0001285
HP:0000278
HP:0011369
HP:0010332 |
- |
- |
Familial, autosomal recessive |
- |
- |
00y06m |
- |
- |
Anaïs Begemann |
00177008 |
0000141826 |
HP:0001252
HP:0002066 |
- |
Dravet syndrome |
Familial, autosomal dominant |
- |
- |
00y04m |
- |
- |
Anaïs Begemann |
00177009 |
0000141827 |
HP:0002510
HP:0003781
HP:0002705
HP:0000431
HP:0011078 |
- |
Dravet syndrome |
Familial, autosomal dominant |
- |
- |
00y02m |
- |
- |
Anaïs Begemann |
00177010 |
0000141828 |
HP:0001285 |
- |
Dravet syndrome |
Familial, autosomal dominant |
- |
- |
00y03m |
- |
- |
Anaïs Begemann |
00177011 |
0000141829 |
no pre-/perinatal anomalies; birth 41w1d, weight 4120 g, length 51 cm; severe intellectual disability (HP:0001249); normal behavior; developmental delay; no sit, no walk; no speech; 3d-seizures, myoclonic seizures, tonic seizures, 1-7 seizures/day pharmacoresistant, no acquired microcephaly; spasticity, bilateral contractures knees; high narrow palate (HP:0002705); cortical visual impairment, gastro-jejunal tube; EEG-frontocentral spike-wave activity sides, burst suppression; 4d-MRI normal; 44y-MRI generalized supra- and infratentorial atrophy, bilateral hippocampal atrophy, left-sided hippocampal sclerosis, atrophy orpus callosum |
- |
DEE11 |
Isolated (sporadic) |
16y04m |
- |
- |
neonatal (HP:0003623) |
- |
Anaïs Begemann |
00177012 |
0000141830 |
HP:0001252
HP:0002066
HP:0100807
HP:0006184
HP:0000767 |
- |
Epileptic encephalopathy, early infantile, 13 |
Familial, autosomal dominant |
- |
- |
00y03m |
- |
- |
Anaïs Begemann |
00177013 |
0000141831 |
HP:0001252
HP:0002509
HP:0001266
HP:0000219
HP:0002705
HP:000319
HP:0000286
HP:0000494
HP:0000508
HP:0011359
HP:0100798 |
- |
Snyder-Robinson syndrome |
Familial, X-linked recessive |
- |
- |
01y00m |
- |
- |
Anaïs Begemann |
00177014 |
0000141832 |
HP:0000666
HP:0002451
HP:0001285 |
- |
Epilepsy, hearing loss, and mental retardation syndrome (MIM 616577) |
Familial, autosomal recessive |
- |
- |
00y08m |
- |
- |
Anaïs Begemann |
00177015 |
0000141833 |
HP:0000407
HP:0001285
HP:0012043
HP:0000565
HP:0002015
HP:0000276
HP:0000275
HP:0000286
HP:0000431
HP:0002705
HP:0012098
HP:0005257
HP:0000953 |
- |
- |
Familial, autosomal recessive |
- |
- |
00y05m |
- |
- |
Anaïs Begemann |
00177016 |
0000141835 |
HP:0008936
HP:0002313
HP:0005348
HP:0007028
HP:0007337
HP:0006811
HP:0002469
HP:0000340
HP:0002705
HP:0005257
HP:0000767
HP:0001212 |
- |
Epileptic encephalopathy, early infantile, 4 |
Familial, autosomal dominant |
- |
- |
- |
HP:0003623 |
- |
Anaïs Begemann |
00177023 |
0000141836 |
HP:0001252
HP:0000337
HP:0400005
HP:0000278
HP:0000331
HP:0002705
HP:0000772
HP:0000768
HP:0008743
HP:0002761
HP:000103 |
- |
Epileptic encephalopathy, early infantile, 18 |
Familial, autosomal recessive |
- |
- |
00y06m |
- |
- |
Anaïs Begemann |
00177024 |
0000141874 |
HP:0001252
HP:0001285
HP:0000549
HP:0007772 |
- |
- |
Unknown |
- |
- |
04y03m |
- |
- |
Anaïs Begemann |
00177064 |
0000141875 |
HP:0001252
HP:0002066
HP:0002007
HP:0400005
HP:0000457 |
- |
- |
Unknown |
- |
- |
01y11m |
- |
- |
Anaïs Begemann |
00177065 |
0000141876 |
HP:0007325
HP:0001285
HP:0005348
HP:0002705
HP:0010804
HP:0000293
HP:0011800
HP:0009748
HP:0009803 |
- |
- |
Unknown |
- |
- |
00y03m |
- |
- |
Anaïs Begemann |
00177066 |
0000141877 |
HP:0008936
HP:0002066
HP:0011220
HP:0008070
HP:0002761
HP:0001763 |
- |
- |
Unknown |
- |
- |
02y |
- |
- |
Anaïs Begemann |
00177082 |
0000141883 |
see paper; …, profound global developmental delay, failure to thrive, progressive microcephaly, refractive epilepsy, subtle facial dysmorphism, severe axial hypotonia, appendicular hypertonia; MRI brain brain dysmyelination, volume loss; EEG hypsarrhythmia; premature death one case |
early-onset encephalopathy |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179397 |
0000141884 |
see paper; …, profound global developmental delay, failure to thrive, progressive microcephaly, refractive epilepsy, subtle facial dysmorphism, severe axial hypotonia, appendicular hypertonia; MRI brain brain dysmyelination, volume loss; EEG hypsarrhythmia; premature death one case |
early-onset encephalopathy |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179398 |
0000141885 |
see paper; ..., severe early infantile encephalopathy, progressive microcephaly, axial hypotonia, appendicular hypertonia, refractory epilepsy |
early-onset encephalopathy |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179399 |
0000141886 |
see paper; ..., severe early infantile encephalopathy, progressive microcephaly, axial hypotonia, appendicular hypertonia, refractory epilepsy |
early-onset encephalopathy |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179400 |
0000141887 |
see paper; ..., severe early infantile encephalopathy, progressive microcephaly, axial hypotonia, appendicular hypertonia, refractory epilepsy |
early-onset encephalopathy |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179401 |
0000141888 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179402 |
0000141889 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179403 |
0000141890 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179404 |
0000141891 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179405 |
0000141892 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179406 |
0000141893 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179407 |
0000141894 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179408 |
0000141895 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179409 |
0000141896 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179410 |
0000141897 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179411 |
0000141898 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179412 |
0000141899 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179413 |
0000141900 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179414 |
0000141901 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179415 |
0000141902 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179416 |
0000141903 |
see paper; … |
hypomyelination with atrophy basal ganglia and cerebellum (HABC) |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179417 |
0000141910 |
see paper; … |
severe infantile-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179423 |
0000141911 |
see paper; … |
severe infantile-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179424 |
0000141912 |
see paper; … |
severe infantile-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179425 |
0000141913 |
see paper; … |
severe infantile-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179426 |
0000141914 |
see paper; … |
severe infantile-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179427 |
0000141915 |
see paper; … |
early-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179428 |
0000141916 |
see paper; … |
early-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179429 |
0000141917 |
see paper; … |
early-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179430 |
0000141918 |
see paper; … |
early-onset encephalopathy |
EIEE-44 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179431 |
0000141919 |
see paper; … |
early-onset epileptic encephalopathy |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00179432 |
0000143153 |
HP:0000252 |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Anaïs Begemann |
00180890 |
0000143154 |
HP:0000252 |
- |
- |
Familial, autosomal dominant |
- |
- |
00y02m |
- |
- |
Anaïs Begemann |
00180893 |
0000143157 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
00y07m |
- |
- |
Anaïs Begemann |
00180894 |
0000143158 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
01y02m |
- |
- |
Anaïs Begemann |
00180895 |
0000143160 |
HP:0000252 |
- |
- |
Familial, autosomal dominant |
- |
- |
00y09m |
- |
- |
Anaïs Begemann |
00180897 |
0000143162 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
00y06m |
- |
- |
Anaïs Begemann |
00180899 |
0000143164 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
00y08m |
- |
- |
Anaïs Begemann |
00180901 |
0000143349 |
HP:0000316
HP:0000331
HP:0000675
HP:0000699
HP:0000411
HP:0002808
HP:0001956
HP:0001763
HP:0001852 |
- |
EIEE-4 |
Isolated (sporadic) |
- |
- |
00y01m |
- |
- |
Anaïs Begemann |
00181099 |
0000143373 |
HP:0008936
HP:0001285
HP:0000639
HP:0000311
HP:0000160
HP:0000653
HP:0000286
HP:0003196
HP:0000463
HP:0000294
HP:0006610 |
- |
- |
Isolated (sporadic) |
- |
- |
00y03m |
- |
- |
Anaïs Begemann |
00181164 |
0000143994 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
05y05m |
- |
- |
Anaïs Begemann |
00181199 |
0000143995 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
04y03m |
- |
- |
Anaïs Begemann |
00181150 |
0000143996 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
01y00m |
- |
- |
Anaïs Begemann |
00181151 |
0000143998 |
- |
- |
- |
Unknown |
- |
- |
01y02m |
- |
- |
Anaïs Begemann |
00181152 |
0000143999 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
03y02m |
- |
- |
Anaïs Begemann |
00181155 |
0000144000 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
00y07m |
- |
- |
Anaïs Begemann |
00181194 |
0000144001 |
- |
- |
- |
Unknown |
- |
- |
00y05m |
- |
- |
Anaïs Begemann |
00181193 |
0000144002 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
00y11m |
- |
- |
Anaïs Begemann |
00181161 |
0000144005 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
01y06m |
- |
- |
Anaïs Begemann |
00181162 |
0000144006 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
04y03m |
- |
- |
Anaïs Begemann |
00181169 |
0000144007 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
00y02m |
- |
- |
Anaïs Begemann |
00181170 |