Phenotypes for disease #02259 (PMD;HLD1 (Pelizaeus-Merzbacher disease (PMD, hypomyelinative leukodystrophy (HLD-1))), OMIM:312080)

77 entries on 1 page. Showing entries 1 - 77.
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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
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