congenital nervous system disorder nodular neuronal heterotopia periventricular nodular heterotopia https://www.malacards.org/card/periventricular_nodular_heterotopia icd11.foundation:20200096 periventricular nodular heterotopia MONDO:0020341 UMLS:C1868720 OMIMPS:300049 NANDO:1201079 GARD:0012724 DOID:0050454 Orphanet:98892 Periventricular nodular heterotopia (PNH) is a brain malformation, due to abnormal neuronal migration, in which a subset of neurons fails to migrate into the developing cerebral cortex and remains as nodules that line the ventricular surface. Classical PNH is a rare X-linked dominant disorder far more frequent in females who present normal intelligence to borderline intellectual deficit, epilepsy of variable severity and extra-central nervous system signs, especially cardiovascular defects or coagulopathy. The disorder is generally associated with prenatal lethality in males. MEDGEN:358387 MedDRA:10066854 MESH:D054091 hereditary neurological disease