Neurodevelopmental Disorder with Neonatal Respiratory Insufficiency, Hypotonia, and Feeding Difficulties

Alternative Names

  • NEDRIHF
  • Mental Retardation, Autosomal Dominant 31
  • MRD31
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WHO-ICD-10 version:2010

Diseases of the nervous system

Episodic and paroxysmal disorders

OMIM Number

616158

Mode of Inheritance

Autosomal dominant

Gene Map Locus

5q31.3

Description

Neurodevelopmental disorder with neonatal respiratory insufficiency, hypotonia, and feeding difficulties (NEDRIHF) is characterized by severe hypotonia at birth associated with respiratory difficulties, including apnea and hypoventilation, and feeding difficulties. Many infants require ventilatory support or feeding tubes. Affected patients have global developmental delay, often never achieving walking or speech, although the severity can be variable. Additional common features may include seizures, exaggerated startle reflex, abnormal movements, and dysmorphic facial features. Brain imaging often shows hypomyelination and parenchymal atrophy. A subset of patients may have systemic features, such as cardiac defects, scoliosis, endocrine anomalies, constipation, or cryptorchidism. [From OMIM]

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
616158.1Saudi ArabiaFemale Motor delay; Hypotonia; Seizure; Failure...NM_005859.5:c.307_308delHeterozygousAutosomal, DominantMonies et al. 2017
616158.2Saudi ArabiaFemale Abnormal facial shapeNM_005859.5:c.307_308delHeterozygousAutosomal, DominantMonies et al. 2017
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