Phenotypes for disease #00777 (PVNH1 (heterotopia, nodular, periventricular, type 1), OMIM:300049)

54 entries on 1 page. Showing entries 1 - 54.
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Phenotype details     

Diagnosis/Initial     

Diagnosis/Definite     

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Age/Examination     

Age/Diagnosis     

Age/Onset     

Phenotype/Onset     

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Individual ID     
0000024727 death 3d, severe systemic bleeding and organ failure, severe arrest myeloid/erythroid differentiation in bone marrow, lymphoid depletion in thymus - - Familial - - - - - Yu Sun 00028669
0000024728 - - - Familial - - - - - Yu Sun 00028671
0000024729 - - - Familial - - - - - Yu Sun 00028672
0000024730 - - - Familial - - - - - Yu Sun 00028673
0000024731 - - - Familial - - - - - Yu Sun 00028674
0000024732 - - - Familial - - - - - Yu Sun 00028675
0000024733 - - - Familial - - - - - Yu Sun 00028677
0000024734 severe, non-lethal; shortened digits, syndactyly, clinodactyly; immune compromise, recurrent infections; small corpus callosum, hypoplastic cerebellum - - Familial - - - - - Yu Sun 00028681
0000024736 Developmental Regression, West Syndrome - - Familial - - - - - Yu Sun 00028684
0000024737 Developmental Regression, West Syndrome - - Familial - - - - - Yu Sun 00028685
0000024738 Developmental Regression, West Syndrome - - Familial - - - - - Yu Sun 00028686
0000024739 BPNH, scattered nodules along lateral ventricle wall, slightly vertically oriented posterior hippocampal region, mega cisterna magna, complex partial seizure onset 20y,rare, remission; EEG mild R sided slowing, verbal IQ74/performance IQ97/full-scale IQ81 - - Familial - - - - - Yu Sun 00028691
0000024740 BPNH, scattered nodules along lateral ventricle wall, slightly vertically oriented posterior hippocampal region, mega cisterna magna, complex partial seizure onset at 20yrs,rare,remission; EEG shows mild R sided slowing, verbal IQ/performance IQ/full-scale IQ=74/97/81 - - Familial - - - - - Yu Sun 00028692
0000024741 NH, scattered nodules along lateral ventricle wall, slightly vertically oriented posterior hippocampal region, mega cisterna magna, complex partial/secondarily generalized seizure onset at 14yrs,once in 4-5 mth,no remission; EEG shows L frontotemporal slow wave, use antiepileptic drugs:carbamazepine,topiramate;verbal IQ/performance IQ/full-scale IQ=70/81/74 - - Familial - - - - - Yu Sun 00028693
0000024755 BPNH, mega cisterna magna, complex partial/secondarily generalized seizure onset at 30yrs,rare,remission;use antiepileptic drugs:phenobarbital;verbal IQ/performance IQ/full-scale IQ=71/72/70 - - Familial - - - - - Yu Sun 00028725
0000024756 BPNH, mega cisterna magna, simple partial/partial secondarily generalized seizure onset at 14yrs,rare,remission;use antiepileptic drugs:phenobarbital;verbal IQ/performance IQ/full-scale IQ=100/69/83 - - Familial - - - - - Yu Sun 00028726
0000024776 - - - Familial - - - - - Yu Sun 00028754
0000024777 - - - Familial - - - - - Yu Sun 00028755
0000024778 - - - Familial - - - - - Yu Sun 00028756
0000024779 - - - Familial - - - - - Yu Sun 00028757
0000024851 - - - Familial - - - - - Yu Sun 00028835
0000024852 - - - Familial - - - - - Yu Sun 00028836
0000024853 - - - Familial - - - - - Yu Sun 00028839
0000024854 - - - Familial - - - - - Yu Sun 00028842
0000024855 - - - Familial - - - - - Yu Sun 00028843
0000024856 - - - Familial - - - - - Yu Sun 00028848
0000024857 - - - Familial - - - - - Yu Sun 00028849
0000024858 - - - Familial - - - - - Yu Sun 00028850
0000024859 - - - Familial - - - - - Yu Sun 00028851
0000024868 - - - Familial - - - - - Yu Sun 00028867
0000024869 - - - Familial - - - - - Yu Sun 00028868
0000024877 - - - Isolated (sporadic) - - - - - Yu Sun 00028679
0000024878 - - - Isolated (sporadic) - - - - - Yu Sun 00028680
0000024882 - - - Isolated (sporadic) - - - - - Yu Sun 00028722
0000024883 - - - Isolated (sporadic) - - - - - Yu Sun 00028723
0000024888 - - - Isolated (sporadic) - - - - - Yu Sun 00028837
0000024889 - - - Isolated (sporadic) - - - - - Yu Sun 00028838
0000024890 - - - Isolated (sporadic) - - - - - Yu Sun 00028840
0000024891 - - - Isolated (sporadic) - - - - - Yu Sun 00028841
0000024892 - - - Isolated (sporadic) - - - - - Yu Sun 00028844
0000024893 - - - Isolated (sporadic) - - - - - Yu Sun 00028845
0000024894 - - - Isolated (sporadic) - - - - - Yu Sun 00028846
0000024895 - - - Isolated (sporadic) - - - - - Yu Sun 00028847
0000024896 indistinguishable from females have FLN1 mutation. Symmetrical nodules, enlarged cisterna magna behind cerebellum, mild to moderate seizure, no MR - - Isolated (sporadic) - - - - - Yu Sun 00028854
0000024897 milder in MRI than females with FLN1 mutations, few heterotopic nodules only in right hemisphere. No MR. Not intractable seizure - - Isolated (sporadic) - - - - - Yu Sun 00028855
0000024898 - - - Isolated (sporadic) - - - - - Yu Sun 00028864
0000024906 periventricular heterotopia, type 1/frontometaphyseal dysplasia; delayed closure anterior fontanel, craniofacial anomalies, marked genua valga necessitating surgical corrections; 5y-MRI cranial bilateral PVNHs; no seizures, normal motor/mental development; 8y-radiological FMD - - Unknown - - - - - Yu Sun 00028666
0000024909 - - - Unknown - - - - - Yu Sun 00028670
0000024910 - - - Unknown - - - - - Yu Sun 00028676
0000024911 - - - Unknown - - - - - Yu Sun 00028678
0000024916 epilepsy - - Unknown - - - - - Yu Sun 00028690
0000024929 BPNH, mega cisterna magna, complex partial/secondarily generalized seizure onset at 9yrs,rare,no remission;use antiepileptic drugs:phenobarbital;verbal IQ/performance IQ/full-scale IQ=66/85/74 - - Unknown - - - - - Yu Sun 00028724
0000034212 see paper; classical X-linked periventricular nodular heterotopia , ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00046330
0000242926 (+) Abnormality of the nervous system,(+) Aortic regurgitation,(+) EEG abnormality,(+) Abnormality of the cerebral cortex,(+) Cortical tubers,(+) Cerebral hamartoma,(+) Hamartoma. Positive famliy history regarding sympotoms mentioned. - - Familial, X-linked dominant 28y - - - - Andreas Laner 00324385
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