Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Phenotype details: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial: initial diagnosis, before molecular testing
Diagnosis/Definite: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
- Unknown
- Familial
- Familial, autosomal dominant
- Familial, autosomal recessive
- Familial, X-linked
- Familial, X-linked dominant
- Familial, X-linked dominant, male sparing
- Familial, X-linked recessive
- Paternal, Y-linked
- Maternal, mitochondrial
- Isolated (sporadic)
- Di-genic
- Complex
- - = Not applicable
Age/Examination: age at which the individual was examined.
- 35y = 35 years
- 04y08m = 4 years and 8 months
- 00y00m01d12h = 1 day and 12 hours
- 18y? = around 18 years
- 30y-40y = between 30 and 40 years
- >54y = older than 54
- ? = unknown
Age/Diagnosis: age diagnosis was confirmed
- 35y = 35 years
- 04y08m = 4 years and 8 months
- 00y00m01d12h = 1 day and 12 hours
- 18y? = around 18 years
- 30y-40y = between 30 and 40 years
- >54y = older than 54
- ? = unknown
Age/Onset: Age first symptoms disease appeared in individual:
- 35y = 35 years
- 04y08m = 4 years and 8 months
- 00y00m01d12h = 1 day and 12 hours
- 18y? = around 18 years
- 30y-40y = between 30 and 40 years
- >54y = older than 54
- ? = unknown
Phenotype/Onset: individual's phenotype at Age/Onset described using HPO
Protein: result from protein staining

 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
0000034688 |
see paper; developmental delay, refractory epilepsy, prolonged survival, no evidence of mitochondrial or peroxisomal dysfunction (blood and urine); EEG nonspecific, background slowing with frequent epileptiform activity rontal and central head regions; EM skeletal muscle showed subtle, nonspecific abnormalities of cristal organization; confocal microscopy of patient fibroblasts showed striking hyperfusion mitochondrial network, ... |
- |
- |
Isolated (sporadic) |
- |
- |
00y06m |
not meeting expected motor milestones |
- |
Johan den Dunnen |
00047314 |
0000038254 |
epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00051661 |
0000038255 |
epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00051662 |
0000038256 |
epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00051663 |
0000038257 |
epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00051664 |
0000038258 |
epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00051665 |
0000080869 |
Macrocephaly (1y); refractory epilepsy with progressive cognitive regression (6y); bilateral strabismus; with normal strength and without muscle complaints (able to run, climb stairs and run) at 21y; brain MRI: white matter changes and occypital agyria. |
- |
- |
Isolated (sporadic) |
21y |
- |
- |
- |
IHC LAMA2 partially absent |
Jorge Oliveira |
00102728 |
0000094090 |
photosensitive genetic generalised epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christopher Reid |
00119071 |
0000094092 |
Idiopathic Photosensitive Occipital Epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christopher Reid |
00119072 |
0000094093 |
early onset absence epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christopher Reid |
00119073 |
0000094094 |
unclassified epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christopher Reid |
00119074 |
0000094095 |
generalized epilepsy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christopher Reid |
00119075 |
0000104251 |
Atypical absence seizures (HP:0007270), Generalized myoclonic seizures (HP:0002123) |
- |
- |
Isolated (sporadic) |
- |
- |
01y08m |
- |
- |
Qing Kenneth Wang |
00132052 |
0000104252 |
- |
- |
- |
Familial, autosomal dominant |
- |
00y08m |
- |
- |
- |
Qing Kenneth Wang |
00132053 |
0000104253 |
Absence seizures (HP:0002121), Atonic seizures (HP:0010819), Focal myoclonic seizures (HP:0011166), Focal tonic seizures (HP:0011167) |
- |
- |
Unknown |
- |
30y |
- |
- |
- |
Qing Kenneth Wang |
00132054 |
0000104254 |
epileptic encephalopathy (HP:0200134) |
- |
- |
Unknown |
- |
- |
01y02m |
- |
- |
Qing Kenneth Wang |
00132055 |
0000129519 |
Epileptic encephalopathy (HP:0200134); Abnormal facial shape (HP:0001999) |
epilepsy |
severe early onset epileptic encephalopathy |
Isolated (sporadic) |
06y |
07y |
- |
- |
- |
Rafał Płoski |
00164481 |
0000132917 |
see paper; ..., infantile-onset
symptomatic epilepsy syndrome, developmental stagnation, blindness |
infantile-onset symptomatic epilepsy syndrome |
SPDRS |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00168053 |
0000132931 |
see paper; ..., early-onset refractory epilepsy, psychomotor delay, failure to thrive, blindness, deafness |
early-onset refractory epilepsy |
SPDRS |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00168065 |
0000143159 |
HP:0001249 |
- |
- |
Familial, autosomal dominant |
- |
- |
03y |
- |
- |
Anaïs Begemann |
00180896 |
0000154005 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205859 |
0000154007 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205862 |
0000154008 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205865 |
0000154009 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205866 |
0000154010 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205867 |
0000154011 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205868 |
0000154012 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00205869 |
0000155310 |
Infantile spasms, myoclonic seizures, cortical visual impairment, developmental delay, and minor dysmorphic features (Anteverted nares, tented upper lip), pectus excavatum, and mild flexion contractures of all fingers bilaterally. Alkaline phosphatase levels ranged from normal to mildly elevated. No evidence of metabolic bone disease. |
- |
- |
Familial, autosomal recessive |
00y08m |
- |
- |
- |
- |
Philippe Campeau |
00207532 |
0000157628 |
epilepsy |
epilepsy |
FESD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00209022 |
0000157630 |
Epilepsy and global developmental delay |
epilepsy |
EIEE-4 |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00209024 |
0000169968 |
- |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00224853 |
0000171268 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Jinliang Li |
00226156 |
0000171269 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226157 |
0000171270 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226158 |
0000171272 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226159 |
0000171273 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226160 |
0000171274 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226161 |
0000171276 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226162 |
0000171277 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Jinliang Li |
00226163 |
0000173071 |
Generalised epilepsy
Intellectual Disability
Gingival Abnormalities
Nail Abnormalities |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Marie Shaw |
00218061 |
0000179360 |
Infantile spasms (HP:0012469); Neurodevelopmental delay (HP:0012758); |
infantile spasms |
infantile spasms |
Unknown |
00y03m |
00y03m |
00y03m |
Infantile spasms (HP:0012469) |
- |
Mengna Zhang |
00239139 |
0000179361 |
infantile spasms(HP:0012469), Neurodevelopmental delay (HP:0012758), Muscular hypotonia (HP:0001252),Intrauterine growth retardation (HP:0001511) |
epilepsy |
infantile spasms |
Unknown |
00y10m |
00y10m |
00y06m |
0m |
- |
Mengna Zhang |
00239140 |
0000179408 |
5y-typical childhood absence epilepsy; 14-15y developed myoclonic seizures and later rare tonic-clonic seizures; EEG typical absences and subsequent persistent bilateral synchronous spike-wave discharges without accompanying clinical signs, last 20 years the EEG normal |
epilepsy |
- |
Unknown |
39y |
- |
05y |
- |
- |
Johan den Dunnen |
00239190 |
0000180000 |
persistent crisis of atipical febrile seizures |
- |
- |
Unknown |
00y10m |
- |
- |
- |
- |
GENBIOMOL - Carla Bidinost |
00239790 |
0000203658 |
Epileptic spasms |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Mitsuko Nakashima |
00265870 |
0000203659 |
Epileptic spasms |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Mitsuko Nakashima |
00265878 |
0000207372 |
generalized epilepsy with febrile seizures plus |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269544 |
0000207373 |
seizures, generalized tonic-clonic |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269545 |
0000207374 |
generalized epilepsy with febrile seizures plus |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269546 |
0000207375 |
myoclonic epilepsy of infancy |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269547 |
0000207376 |
generalized epilepsy with febrile seizures plus |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269548 |
0000207377 |
epilepsy, childhood absence with febrile seizures |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269549 |
0000207378 |
generalized epilepsy with febrile seizures plus |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269550 |
0000207379 |
epilepsy, idiopathic generalised |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269551 |
0000207380 |
generalized epilepsy with febrile seizures plus |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269552 |
0000207381 |
febrile seizures |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269553 |
0000207382 |
generalized epilepsy with febrile seizures plus |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269554 |
0000207383 |
epilepsy, childhood absence with febrile seizures |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00269555 |
0000207402 |
Early severe epileptic encephalopathy, spastic tetraplegia, opisthotonos attacks |
epileptic encephalopathy |
genetic epileptic encephalopathy |
Isolated (sporadic) |
00y-08y |
- |
00y00m02d |
clusters of tonic spasms |
- |
Maurizio Elia |
00266658 |
0000207787 |
HPOs: Abnormality of the nervous system; Epileptic encephalopathy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Andreas Laner |
00270548 |
0000207792 |
HPO: Abnormality of the nervous system; Epileptic encephalopathy |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Andreas Laner |
00271176 |
0000209232 |
intractable atonic seizures |
- |
- |
Familial |
- |
- |
08y |
- |
- |
Marketa Wayhelova |
00274290 |
0000209233 |
intellectual disability, developmental delay, autistic features, cortical blindness |
- |
- |
Familial |
00y06m |
- |
- |
- |
- |
Marketa Wayhelova |
00274290 |
0000209234 |
biotinidase deficiency |
- |
- |
Familial, autosomal recessive |
- |
00y08m |
- |
- |
- |
Marketa Wayhelova |
00274290 |
0000209236 |
intractable seizures, developmental delay, central hypotonia
biotinidase deficiency not related to neurodevelopmental disorders |
- |
- |
Familial |
00y04m |
- |
00y14m |
- |
- |
Marketa Wayhelova |
00274291 |
0000209264 |
biotinidase deficiency not related to epilepsy and neurodevelopmental disorders |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Marketa Wayhelova |
00274291 |
0000213628 |
epilepsy and intellectual disability |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00279047 |
0000214097 |
- |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00279516 |
0000215676 |
seizures |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00281095 |
0000215879 |
seizures |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00281298 |
0000215880 |
seizures |
epilepsy |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Rosemary Ekong |
00281299 |
0000215881 |
seizures, developmental delay |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00281300 |
0000215938 |
- |
epilepsy |
- |
Unknown |
- |
- |
- |
- |
- |
Rosemary Ekong |
00281357 |
0000222961 |
see paper; ..., rapidly progressing cerebral atrophy, intractable seizures, and intellectual disability |
epilepsy |
EIEE61 |
Familial, autosomal recessive |
26y |
- |
- |
- |
- |
Johan den Dunnen |
00289331 |
0000223283 |
epileptic spasms
right frontal seizure
truncal hypotonia |
epileptic spasms |
- |
Familial, autosomal recessive |
00y12m |
00y12m |
00y07m |
epileptic spasms |
- |
Gabrielle Rudolf |
00295764 |
0000223746 |
rolling over-12m,; meaningful words 24-36m; seizure, developmental delay; walk-1y5m; severe intellectual disability (IQ=25 17y); speech few words; no microcephaly; 6y-generalized tonic-clonic seizures, 9y-absence seizure, 11y-atonic seizure; seizure therapy intractable; regression motor skill and dysarthria; ataxia; intention tremor; rigidity; myoclonus; spasticity; increased deep tendon reflex upper and lower limbs; pathogenic reflex Rossolimo sign positive, Mendel-Bechterew sign positive; no dysmorphic features; wheelchair-bound; MRI brain normal; EEG abnormal discharge in right hemisphere (6 years), burst of diffuse irregular spikes and slow waves (9 years), diffuse spike and slow waves in frontal, parietal and temporal regions (14 years); somatosensory evoked potential prolonged N20 latency and high amplitude of P24-N33 |
epilepsy |
- |
Isolated (sporadic) |
22y |
- |
6y |
- |
- |
Johan den Dunnen |
00296271 |
0000223747 |
walking without support-28m; seizure; walk-2y4m; severe intellectual disability (IQ=25 12y); speech few words; microcephaly (−2.0 SD); 11m-generalized tonic-clonic seizures, 5y-loss of consciousness with abnormal eye movement, 10y-complex partial seizure, 10y-atonic seizure,; seizure therapy intractable; regression motor skill; ataxia; intention tremor; rigidity; myoclonus; spasticity; increased deep tendon reflex upper and lower limbs; no pathogenic reflex; no dysmorphic features; wheelchair-bound; MRI brain mild cerebellar atrophy; EEG diffuse slow wave with 2–3 Hz and spike-and-wave in bilateral frontal region (3 years and 4 years), diffuse theta waves with 4–5 Hz and spike-and-wave burst with 2–3 Hz (9 years), multifocal spikes in left parietal region and bilateral frontal regions (12 years), multispikes in left occipital region (13 years), slow waves at baselines (23 years); giant somatosensory evoked potential; systemic lupus erythematosus |
epilepsy |
- |
Isolated (sporadic) |
28y |
- |
11m |
- |
- |
Johan den Dunnen |
00296272 |
0000223748 |
walking without support-24m; seizure; walk-2y; severe intellectual disability; no speech; microcephaly (−2.5 SD); 2y-absence seizure ; seizure therapy responsive; regression motor and verbal skills; ataxia; intention tremor; no dysmorphic features; wheelchair-bound; MRI brain small vermis; EEG abnormal background activity (1 year), slow abnormal sleep features with paucity of sleep spindles (13 years) |
epilepsy |
- |
Isolated (sporadic) |
14y |
- |
2y |
- |
- |
Johan den Dunnen |
00296273 |
0000223749 |
eye pursuit-5m, walking without support-24m, meaningful words-30m; seizure and developmental delay; walk-2y; severe intellectual disability; speech few words; microcephaly (2nd percentile); 4y-atonic seizure; seizure therapy intractable, but improved by clobazam and sulthiame (responsive); regression; ataxia; intention tremor; myoclonus; no increased deep tendon reflex; no pathogenic reflex; no dysmorphic features; walking with support; MRI brain normal; EEG focal bifrontal epileptiform discharges accentuated during sleep (4 years), frequent frontocentral discharges during awake state (5 years), frequent intermittent slow spikes in right posterior region (11 years) |
epilepsy |
- |
Isolated (sporadic) |
11y |
- |
4y |
- |
- |
Johan den Dunnen |
00296274 |
0000231816 |
- |
developmental and epileptic encephalopathy |
- |
Isolated (sporadic) |
23y |
- |
- |
- |
- |
Johan den Dunnen |
00305970 |
0000231817 |
- |
developmental and epileptic encephalopathy |
- |
Unknown |
28y |
- |
- |
- |
- |
Johan den Dunnen |
00305971 |
0000231818 |
- |
Lennox Gastaut syndrome |
developmental and epileptic encephalopathy |
Unknown |
32y |
- |
- |
- |
- |
Johan den Dunnen |
00305972 |
0000231819 |
- |
developmental and epileptic encephalopathy |
- |
Isolated (sporadic) |
32y |
- |
- |
- |
- |
Johan den Dunnen |
00305973 |
0000231820 |
- |
developmental and epileptic encephalopathy |
- |
Unknown |
25y |
- |
- |
- |
- |
Johan den Dunnen |
00305974 |
0000231821 |
- |
Lennox Gastaut syndrome |
- |
Isolated (sporadic) |
27y |
- |
- |
- |
- |
Johan den Dunnen |
00305975 |
0000231822 |
- |
unspecified epilepsy |
- |
Isolated (sporadic) |
47y |
- |
- |
- |
- |
Johan den Dunnen |
00305976 |
0000231823 |
- |
Lennox Gastaut syndrome |
- |
Isolated (sporadic) |
29y |
- |
- |
- |
- |
Johan den Dunnen |
00305977 |
0000231824 |
- |
temporal lobe epilepsy |
- |
Unknown |
42y |
- |
- |
- |
- |
Johan den Dunnen |
00305978 |
0000231825 |
- |
developmental and epileptic encephalopathy |
- |
Unknown |
43y |
- |
- |
- |
- |
Johan den Dunnen |
00305979 |
0000231826 |
- |
multifocal epilepsy |
- |
Isolated (sporadic) |
32y |
- |
- |
- |
- |
Johan den Dunnen |
00305980 |
0000231827 |
- |
developmental and epileptic encephalopathy |
- |
Isolated (sporadic) |
45y |
- |
- |
- |
- |
Johan den Dunnen |
00305981 |
0000231828 |
- |
developmental and epileptic encephalopathy |
- |
Unknown |
41y |
- |
- |
- |
- |
Johan den Dunnen |
00305982 |
0000231829 |
- |
developmental and epileptic encephalopathy |
- |
Isolated (sporadic) |
23y |
- |
- |
- |
- |
Johan den Dunnen |
00305983 |
0000231830 |
- |
developmental and epileptic encephalopathy |
- |
Isolated (sporadic) |
27y |
- |
- |
- |
- |
Johan den Dunnen |
00305984 |
0000231831 |
- |
childhood onset epilepsy |
- |
Unknown |
21y |
- |
- |
- |
- |
Johan den Dunnen |
00305985 |
0000231832 |
- |
developmental and epileptic encephalopathy |
- |
Unknown |
22y |
- |
- |
- |
- |
Johan den Dunnen |
00305986 |
0000231833 |
- |
unspecified epilepsy |
- |
Unknown |
45y |
- |
- |
- |
- |
Johan den Dunnen |
00305987 |
0000231834 |
- |
focal epilepsy |
developmental and epileptic encephalopathy |
Unknown |
22y |
- |
- |
- |
- |
Johan den Dunnen |
00305988 |
0000231835 |
- |
ADNFLE |
focal epilepsy |
Unknown |
46y |
- |
- |
- |
- |
Johan den Dunnen |
00305989 |
0000231836 |
59y-deceased |
developmental and epileptic encephalopathy |
- |
Unknown |
59y |
- |
- |
- |
- |
Johan den Dunnen |
00305990 |