Neurodevelopmental Disorder with Seizures and Brain Atrophy

Alternative Names

  • NEDSEBA
Back to search Result
WHO-ICD-10 version:2010

Mental and behavioural disorders

Disorders of psychological development

OMIM Number

619072

Mode of Inheritance

Autosomal recessive

Gene Map Locus

17q25.1

Description

Neurodevelopmental disorder with seizures and brain atrophy (NEDSEBA) is an autosomal recessive disorder with highly variable manifestations and severity of these core features. The most severely affected individuals develop symptoms in utero, which may lead to spontaneous abortion or planned termination. Those that survive may present with severe seizures at birth, have poor overall growth with small head circumference, achieve no developmental progress, and show significant brain atrophy and other brain abnormalities. Patients at the mildest end of the phenotypic spectrum have onset of seizures later in childhood and show developmental delay with mildly impaired intellectual development and minimal brain atrophy. [From OMIM]

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
619072.1.1United Arab EmiratesFemaleYesYes Death in infancy; Microcephaly; Arthrogr...NM_001013839.4:c.809-2A>GHomozygousAutosomal, RecessiveCoulter et al. 2020
619072.1.2United Arab EmiratesFemaleYesYes Death in infancy; Arthrogryposis multipl...NM_001013839.4:c.809-2A>GHomozygousAutosomal, RecessiveCoulter et al. 2020 Sister of 619072.1.1
© CAGS 2024. All rights reserved.