ALKBH8

Clinical Characteristics

The clinical features of MRT71 include:

Neurologic abnormalities

  • Intellectual disability
  • Global developmental delay
  • Epilepsy (usually well-controlled on medication)
  • Behavioral abnormalities:
    • Hyperactivity
    • Attention deficit
    • Stereotypical behaviors (1 patient)
  • Hypotonia (1 patient)
  • Arachnoid granulations on MRI (1 patient)
  • EEG showing generalized slowing and episodes of generalized delta discharges, which may indicate underlying cortical irritability (1 patient)

Facial dysmorphism (variable, mild; found in some patients)

  • Long face
  • Large eyes
  • Large ears
  • Deep-set eyes
  • Overbite

Other (found in some patients)

  • Macrocephaly
  • Prune belly syndrome
  • Congenital heart disease in the form of VSD and thick aortic valve (1 patient)
  • Genitourinary malformations:
    • Small penis (1 patient)
    • Undescended testes (1 patient)
    • Unilateral renal agenesis (1 patient)