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JAMA Pediatrics Clinical Challenge
July 2015

Recurrent Right-Sided Ptosis in a Child

Author Affiliations
  • 1Department of Neurology, Boston Children’s Hospital, Boston, Massachusetts
JAMA Pediatr. 2015;169(7):693-694. doi:10.1001/jamapediatrics.2015.0587

A young boy presented for evaluation of “droopiness” of the eyelid. He had 3 episodes of right-sided ptosis, each of which occurred at 18 months, 3 years, and 5 years of age. None of these episodes was preceded by fever. During the last 2 episodes, he experienced severe right-sided headache lasting for 6 to 7 days before development of ptosis; he also experienced double vision. There was no history of loss of consciousness, seizures, loss of vision or hearing, facial deviation, trouble swallowing, or weakness of the extremities in any of these episodes. There was history of migraine on the maternal side of the family. There was no family history of autoimmune disorders or cerebral aneurysms. At the time of the clinic visit, the patient was asymptomatic and results of the neurologic examination were normal. The family provided a photograph of the patient’s face at the time of the first episode (Figure, A). After the first episode, with a presumed diagnosis of acute disseminated encephalomyelitis, he received intravenous pulse methylprednisolone sodium succinate, 30 mg/kg, for 3 days and recovered completely in 3 weeks. The patient received intravenous immunoglobulin, 2 g/kg, for 2 days after the subsequent 2 episodes, with a presumed diagnosis of chronic inflammatory demyelinating neuropathy, and recovered fully. Results of brain magnetic resonance imaging performed at 18 months showed thickening of the cisternal part of the right third cranial nerve in the perimesencephalic cistern (Figure, B) with no parenchymal lesions. Results of tests on cerebrospinal fluid collected via lumbar puncture were normal.

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