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- W2038331387 abstract "Two years ago, Wolsey and Warner (1) reported in this journal on two healthy infants with brief episodes of downgaze followed by abnormal body movements. They called this condition “paroxysmal tonic downgaze” and considered it idiopathic and transient. We extend their report by describing a 2-month-old healthy boy presenting with an episodic downward gaze disturbance (Fig. 1).FIG. 1: Downward eye deviation in a 2-month-old boy. The downward eye deviation occurred many times per day, lasted from 10 to 90 seconds, and resolved spontaneously within 3 months.Born at term after an uneventful delivery and normal development, he was transferred for possible “seizures” from another hospital. The spells had started 7 days before presentation, coincident with a diagnosis of a new upper respiratory tract infection. We observed many spells per day that lasted between 10 and 90 seconds without any impairment in consciousness and seemed to occur more frequently when he was supine or upon awakening but never in an upright position or during sleep. They did not appear to follow touching or shaking. Longer spells were associated with stiffening of the right upper extremities. Ophthalmologic examination disclosed that he fixed and followed equally with each eye. He had normal alignment and full extraocular movements without nystagmus. Neurologic examination showed slightly reduced muscle tone but normal muscle strength and deep tendon reflexes. There was no ataxia. The child underwent MRI and an electroencephalogram (EEG) because of a report of hydrocephalus and seizures in very similar downgaze episodes in children aged 2 to 8 months (2) who later showed severe developmental disabilities. Results of these studies were normal. Because we could not entirely exclude opsoclonus-myoclonus syndrome, we undertook evaluation for neuroblastoma with ultrasound of the abdomen, urine for vanillylmandelic acid levels, and iodine-131 metaiodobenzylguanidine (MIBG) scanning (3). Results of all studies were negative. The episodes began to decrease in frequency spontaneously 1.5 months after they began and resolved within 3 months. He did not develop any neurologic problems in the subsequent 18 months and has had normal development except for a slight delay in expressive language and a mild attention deficit. The pathogenesis of this transient gaze disturbance is not understood. Some observers have hypothesized that it may be the result of immature myelination of the corticomesencephalic vertical gaze pathways (4). Its transient nature, especially in older infants, may result from temporary failure of cortical compensation when a stressor such an illness is present (5). Our experience reinforces the idiopathic and transient nature of this condition. Even so, this manifestation is frightening. Because more serious conditions cannot be entirely excluded, imaging and an EEG cannot be avoided. Meropi Tzoufi, MD Polyxeni Sixlimiri, MD Alexandros Makis, MD Antigone Siamopoulou-Mavridou, MD Child Health Department Medical School of the University of Ioannina Ioannina, Greece E-mail: [email protected]" @default.
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- W2038331387 date "2009-03-01" @default.
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- W2038331387 title "Another Case of Paroxysmal Tonic Downgaze in Infancy" @default.
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- W2038331387 doi "https://doi.org/10.1097/wno.0b013e318198ccde" @default.
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