Occlusion of the basilar artery in a 6-year-old boy.
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Biomedical subjects
Publications and source records attributed to J M Dooley.
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We report a boy with rigidity and hyperekplexia or startle disease which began in adolescence. His symptoms completely resolved with valproic acid therapy. Two relatives have the minor form of this disorder, suggesting autosomal dominant inheritance with variable penetrance.
Once-daily ethosuximide was used to treat 10 consecutive children with typical absence seizures. Three patients had gastrointestinal side effects which resolved when the same total daily dose was divided into 2 doses. Two other patients continued to have seizures on ethosuximide, whether given once or twice daily. Five patients had complete seizure control without adverse effects on once-daily ethosuximide.
Two sisters with autosomal recessive cerebellar hypoplasia and severe nonprogressive retinal pigmentary disease are presented. This syndrome has been previously described in only 1 patient. The retinal changes may be difficult to discern and we suggest that all patients with congenital ataxia have a detailed ophthalmologic assessment, including electroretinography.
Chronic paroxysmal hemicrania and cluster headache are both characterized by recurrent, severe, unilateral headaches accompanied by symptoms and signs of autonomic dysfunction. They are differentiated by the frequency, duration, and medication responsiveness of the headaches. Both occur in childhood, although such reports are rare. A 6-year-old boy presented with chronic paroxysmal hemicrania-like headaches. Through the use of a headache diary, his headaches were found to follow stressful events and resolved shortly after the introduction of the diary. Precipitation of chronic paroxysmal hemicrania-like headaches by stress has not been previously reported. We recommend the use of a headache diary as both an aid to diagnosis and an initial nonpharmacological therapeutic intervention for children with such headaches.