https://www.frontiersin.org/articles/10.3389/fnins.2023.1188839/full

https://metabolicas.sjdhospitalbarcelona.org/ecm/defectos-sintesis-coenzima-q10/info/como-diagnostica-deficiencia-coenzima-q10

https://www.aeped.es/comite-medicamentos/pediamecum/coenzima-q10-ubidecarenona

Indications for treatment in pediatric neurology:

  • Mitochondrial diseases:
    • Congenital coenzyme Q10 deficiency (primary). Symptoms in the form of cerebellar ataxia, encephalomyopathy, isolated myopathy.
    • Other mitochondrial diseases (secondary deficiency):
      • Friedrich's ataxia.
      • CPEO (Chronic Progressive External Ophthalmoplegia),
      • Mitochondrial complex I deficiency.
      • Mitochondrial complex II deficiency.
      • Mitochondrial complex II/IV deficiency.
      • Kearns-Sayre syndrome.
      • LHON (Leber's Hereditary Optic Neuropathy).
      • MELAS (Mitochondrial myopathy, Encephalopathy, Lactic Acidosis and Stroke-like).
      • NARP (Neurogenic muscle weakness, Ataxia, and Retinitis Pigmentosa).
      • Twinkle disorders (C10orf2)
      • Oculomotor apraxia.
      • Mitochondrial cardiomyopathy.
      • Alpers disease.
      • MERRF (Myoclonic Epilepsy associated with Ragged Red Fibers).
      • SANDO (Sensory Ataxia with Neuropathy, Dysarthria and Ophthalmoparesis)
      • Mitochondrial DNA depletion syndrome.
  • Other diseases (secondary deficiency):
    • Propionic acidemia.
    • Multiple system atrophy.
    • Corticobasal degeneration.
    • Muscular dystrophy.
    • Amyotrophic lateral sclerosis.
    • Multiple sclerosis.
    • Charcot-Marie-Tooth disease.
    • McArdle disease.
    • Leukodystrophy.
    • Migraine.
    • Myopathy due to myoadenylate deaminase (MADA) deficiency.
    • Progressive supranuclear palsy.
    • Cyclic vomiting syndrome.
    • Sanfilippo syndrome.
    • Wolf-Hirschhorn syndrome.
    • Bardet-Biedl syndrome.
    • Chronic fatigue syndrome.
    • Stroke.
    • Adrenoleukodystrophy.
    • Spinocerebellar ataxia.
    • Folic acid deficiency.
    • Oculopharyngeal dystrophy.
    • Steinert myotonic dystrophy.
    • Huntington's disease.
    • Niemann-Pick disease.
    • Pompe disease.
    • Spasticity.
    • Spastic paraparesis.
    • Prader-Willi syndrome.
    • Wolfram syndrome.