Journal of child neurology
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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.
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Children with neurological and neuromuscular diseases often present anesthetic problems in the perioperative period. The anesthetic technique can play a significant role in altering the state of the brain during neurosurgical procedures through effects on the cerebral circulation and metabolism. Pre-existing neuromuscular disease may also have specific anesthetic implications such as cardiorespiratory involvement (eg, myotonia dystrophica), the potential for drug interactions (eg, myasthenia gravis) or abnormal responses to commonly used drugs (eg, malignant hyperthermia). In this review, the perioperative anesthetic considerations in a number of common neurological and neuromuscular conditions in the pediatric patient are discussed.