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A 9-year-old girl was referred for evaluation of paroxysmal events of several types with the onset beginning in the first few days of life. She was the result of a normal pregnancy born at term with healthy nonconsanguineous parents. She had a history of global developmental delay and a few remote generalized tonic-clonic seizures. Her events evolved over time and increased in frequency and duration over the years. Between events, she returned to her baseline level of function. Her events were of various types, lasting seconds to minutes (video at Neurology.org/cp), including the following:
Arching of the back: These events were noted by her parents in the first few days of life.
Right body stiffening: The patient had her first event at age 17 months. From age 3, there was an associated head turn to the right, or left. Episodes lasted up to 10 minutes.
Right (or left)–sided weakness: These events began at age 8, and would start with right (or left) arm weakness, followed by facial weakness, and drooling. The patient often would also have speech arrest during her episodes, upon which she would type on a mobile device to communicate. Eventually her foot would become rigid. Arching of the back occurred with prolonged attacks. Ninety percent of events involved the right side. Left-sided events had similar phenomenology.
Vertical ocular oscillations: These began at 6 months of age. The patient's eyes would rhythmically oscillate vertically for 10–20 minutes.
Footnotes
Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.
Supplemental data at Neurology.org/cp
- Received October 17, 2016.
- Accepted January 11, 2017.
- © 2017 American Academy of Neurology
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