Brain sagging syndrome presenting with chorea
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Patient 1 is a 42-year-old previously well man on no medications who presented to neurology clinic with 6 months of nonpositional chronic daily headache, hiccups, and behavior change. His wife reported impulsivity, disinhibition, and sexually inappropriate behavior. There was no family history of movement disorder or neurodegenerative disease. Six weeks prior to symptom onset, he had fallen 1 meter onto his back. Vital signs, bloodwork, and general examination were normal. He was disinhibited and inattentive and exhibited orofacial, tongue, and limb choreoathetosis, which worsened over 6 months of follow-up (video at Neurology.org/cp). MRI was consistent with brain sagging syndrome; basal ganglia were subtly deformed, but without signal disturbance (figure). CSF opening pressure was 18 cm H2O. A spinal CSF leak could not be located with conventional MRI, CT myelography, or radionuclide cisternography. Oral methylprednisolone, continuous epidural saline infusion, theophylline, and 2 lumbar epidural blood patches yielded no sustained improvement. Fat saturated MRI revealed a probable CSF leak at T4, but CT-guided T4 epidural blood patch was unsuccessful. He awaits surgical exploration.
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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 November 20, 2016.
- Accepted January 12, 2017.
- © 2017 American Academy of Neurology
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