
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
| 0000070604 |
severe IDD, progressive spasticity, nystagmus, ataxia, Perlizaeus- Merzbacher, severe hypomyelination of early myelinating structures (HEMS); leukodystrophy |
- |
- |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00092268 |
| 0000093336 |
mild classical PMD |
- |
- |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Grace Hobson |
00117958 |
| 0000093337 |
mild classical PMD phenotype |
- |
- |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Grace Hobson |
00117959 |
| 0000093338 |
mild classical PMD |
- |
- |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Grace Hobson |
00117960 |
| 0000142669 |
- |
hypotonia and horizontal nystagmus |
Pelizaeus-Merzbacher disease |
Familial, X-linked recessive |
16y |
09y |
00y06m |
- |
- |
Jesus Molano Mateos |
00180274 |
| 0000142694 |
brother (II2) and nephew (III1) died of PMD |
Pelizaeus-Merzbacher disease |
PMD |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180300 |
| 0000142695 |
- |
Pelizaeus-Merzbacher disease |
PMD |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180301 |
| 0000142696 |
3y-deceased; severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
3y |
- |
8m |
- |
- |
Jesus Molano Mateos |
00180302 |
| 0000142697 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
4y |
- |
3m |
- |
- |
Jesus Molano Mateos |
00180303 |
| 0000142698 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
7y |
- |
2m |
- |
- |
Jesus Molano Mateos |
00180304 |
| 0000142699 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Isolated (sporadic) |
5y |
- |
8m |
- |
- |
Jesus Molano Mateos |
00180305 |
| 0000142700 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
1y |
- |
2m |
- |
- |
Jesus Molano Mateos |
00180306 |
| 0000142701 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
9y |
- |
3m |
- |
- |
Jesus Molano Mateos |
00180307 |
| 0000142702 |
severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
5y |
- |
2m |
- |
- |
Jesus Molano Mateos |
00180308 |
| 0000142703 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
3y |
- |
11m |
- |
- |
Jesus Molano Mateos |
00180309 |
| 0000142704 |
severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
4y |
- |
0d |
- |
- |
Jesus Molano Mateos |
00180310 |
| 0000142705 |
complicated spastic paraplegia |
- |
- |
Isolated (sporadic) |
6y |
- |
8m |
- |
- |
Jesus Molano Mateos |
00180311 |
| 0000142706 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
- |
- |
4m |
- |
- |
Jesus Molano Mateos |
00180312 |
| 0000142707 |
12y-deceased; severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Unknown |
12y |
- |
2m |
- |
- |
Jesus Molano Mateos |
00180313 |
| 0000142708 |
PLP null syndrome |
- |
- |
Familial, X-linked recessive |
3y |
- |
1m |
- |
- |
Jesus Molano Mateos |
00180314 |
| 0000142709 |
severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
4y |
- |
8m |
- |
- |
Jesus Molano Mateos |
00180315 |
| 0000142710 |
PLP null syndrome |
- |
- |
Unknown |
8y |
- |
6m |
- |
- |
Jesus Molano Mateos |
00180316 |
| 0000142711 |
severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
11m |
- |
0d |
- |
- |
Jesus Molano Mateos |
00180317 |
| 0000142712 |
severe connatal Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
7m |
- |
0d |
- |
- |
Jesus Molano Mateos |
00180318 |
| 0000142713 |
13y-deceased; complicated spastic paraplegia |
- |
- |
Unknown |
13y |
- |
1y |
- |
- |
Jesus Molano Mateos |
00180319 |
| 0000142714 |
classic Pelizaeus-Merzbacher disease |
- |
- |
Familial, X-linked recessive |
28y |
- |
3m |
- |
- |
Jesus Molano Mateos |
00180320 |
| 0000142715 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180321 |
| 0000142716 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180322 |
| 0000142717 |
severe connatal Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180323 |
| 0000142718 |
severe connatal Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180324 |
| 0000142719 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180325 |
| 0000142720 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180326 |
| 0000142721 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180327 |
| 0000142722 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180328 |
| 0000142723 |
mild Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180329 |
| 0000142724 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180330 |
| 0000142725 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180331 |
| 0000142726 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180332 |
| 0000142727 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180333 |
| 0000142728 |
mild Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180334 |
| 0000142729 |
mild Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
01y-02y |
- |
- |
Johan den Dunnen |
00180335 |
| 0000142730 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180336 |
| 0000142731 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180337 |
| 0000142732 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180338 |
| 0000142733 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180339 |
| 0000142734 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180340 |
| 0000142735 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
00y-01y |
- |
- |
Johan den Dunnen |
00180341 |
| 0000142736 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180342 |
| 0000142737 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180343 |
| 0000142738 |
Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180344 |
| 0000142739 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180345 |
| 0000142740 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180346 |
| 0000142741 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180347 |
| 0000142742 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180348 |
| 0000142743 |
severe Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180349 |
| 0000142744 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180350 |
| 0000142745 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180351 |
| 0000142746 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180352 |
| 0000142747 |
mild Pelizaeus-Merzbacher disease, no nystagmus, ambulant |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180353 |
| 0000142748 |
mild Pelizaeus-Merzbacher disease, no nystagmus, ambulant |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180354 |
| 0000142749 |
mild Pelizaeus-Merzbacher disease, no nystagmus, ambulant |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180355 |
| 0000142750 |
mild Pelizaeus-Merzbacher disease, no nystagmus, ambulant |
Pelizaeus-Merzbacher disease |
PMD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180356 |
| 0000142751 |
classic Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180357 |
| 0000142752 |
severely handicapped, disease onset shortly after birth |
Pelizaeus-Merzbacher disease |
PMD |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180358 |
| 0000142753 |
nystagmus from birth; 1m-respiratory difficulty, tracheomalacia, brain stem auditory evoked response showed extremely slow conduction velocity with only waves 1 and 2 present bilaterally; 13m-MRI scan was extremely abnormal with poor myelination white matter, EMG showed no involvement peripheral nervous system; 4y-not able to sit, speak, feed himself. |
Pelizaeus-Merzbacher disease |
PMD |
Familial, X-linked recessive |
04y |
- |
- |
- |
- |
Johan den Dunnen |
00180359 |
| 0000142754 |
inspiratory stridor, failure to thrive, nystagmus, spasticity, joint contractures, scoliosis; 21y-head CT scan showed severe atrophy with
demyelination; died at 23y autopsy showed marked cerebellar atrophy, microscopic brain sections showed absence of myelin with preservation of axons diffusely within the white matter: |
Pelizaeus-Merzbacher disease |
PMD |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00180360 |
| 0000142755 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; no pyramidal signs; tremor; auditory brain response only I-III waves; MRI delayed myelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Familial, X-linked recessive |
1y |
- |
1m |
nystagmus |
- |
Johan den Dunnen |
00180362 |
| 0000142756 |
severity score 2; 7m-head control; 4y-sitting; 4y-walking; nystagmus; no muscular hypotonia; pyramidal signs; ataxia; dysarthria; disease deteriorating, now only sitting; auditory brain response only I-III waves; MRI incomplete myelination |
classic Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
14y |
- |
1d |
nystagmus |
- |
Johan den Dunnen |
00180363 |
| 0000142757 |
severity score 0; no head control; not sitting; not walking; no nystagmus; muscular hypotonia; pyramidal signs |
connatal Pelizaeus-Merzbacher disease |
PMD |
Unknown |
20y |
- |
- |
- |
- |
Johan den Dunnen |
00180364 |
| 0000142758 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; seizures; choreoathetosis; auditory brain response only I wave; MRI hypomyelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
4m |
- |
1d |
nystagmus |
- |
Johan den Dunnen |
00180365 |
| 0000142759 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; pyramidal signs; auditory brain response only I wave; MRI hypomyelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
2y |
- |
1m |
nystagmus, hypotonia |
- |
Johan den Dunnen |
00180366 |
| 0000142760 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia (upper limbs); pyramidal signs (lower limbs); auditory brain response only I wave; MRI delayed myelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
2y |
- |
2m |
nystagmus |
- |
Johan den Dunnen |
00180367 |
| 0000142761 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; no pyramidal signs; strider; auditory brain response only I-III waves; MRI delayed myelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
1y |
- |
1d |
asphyxia |
- |
Johan den Dunnen |
00180368 |
| 0000142762 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; no pyramidal signs; seizures; auditory brain response only I wave; MRI delayed myelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
1y |
- |
7d |
abnormal auditory brain response |
- |
Johan den Dunnen |
00180369 |
| 0000142763 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; no pyramidal signs; seizures; microcephaly; auditory brain response only I wave; MRI delayed myelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
4m |
- |
1m |
microcephaly (-3.2SD) |
- |
Johan den Dunnen |
00180370 |
| 0000142764 |
severity score 0; no head control; not sitting; not walking; nystagmus; muscular hypotonia; sensori-neural seizures; auditory brain response only I-II waves; MRI hypomyelination |
connatal Pelizaeus-Merzbacher disease |
PMD |
Isolated (sporadic) |
1y5m |
- |
1m |
nystagmus |
- |
Johan den Dunnen |
00180371 |
| 0000350436 |
Neurodevelopmental delay, Delayed gross motor development, Expressive language delay, Intention tremor, Hyperreflexia, Babinski sign, Brachycephaly, Plagiocephaly |
- |
- |
Unknown |
02y |
- |
- |
- |
- |
Andreas Laner |
00464416 |