Benign paroxysmal tonic upgaze of childhood

J Child Neurol. 1988 Jul;3(3):177-80. doi: 10.1177/088307388800300305.


Four cases of an apparently benign ocular motor syndrome of childhood are reported. The features of the disorder are: (1) onset in early life; (2) periods of constant or variably sustained tonic conjugate upward deviation of the eyes; (3) down-beating saccades in attempted downgaze, which are difficult to sustain below the neutral positions; (4) apparently normal horizontal eye movements; (5) frequent relief by sleep; (6) otherwise normal neurological findings apart from mild ataxia, chronic in one boy and at times of illness in one of the other patients; (7) absence of deterioration during observation spanning up to 15 years; (8) eventual improvement but with some residual ocular movement problems in two cases; (9) normal metabolic, electroencephalographic, and neuroradiologic investigations; (10) normal brain examination findings in one patient who died accidentally; and (11) an apparently good response to levodopa therapy in one patient. To the authors' knowledge, this condition has not been described previously. It may be a new levodopa-responsive condition, secondary to a localized neurotransmitter deficiency.

Publication types

  • Case Reports

MeSH terms

  • Brain / physiopathology
  • Child
  • Child Development
  • Child, Preschool
  • Eye Movements*
  • Female
  • Humans
  • Infant
  • Male
  • Nystagmus, Pathologic / physiopathology
  • Ophthalmoplegia / physiopathology
  • Saccades
  • Sleep Stages / physiology*