Brain : a journal of neurology
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Paroxysmal hemicrania is a rare syndrome characterized by repeated attacks of strictly unilateral, severe, short-lasting pain occurring with cranial autonomic features. The hallmarks of this syndrome are the relatively short attacks and the exquisite response to indometacin. We describe the phenotype of this condition in a series of 31 patients. ⋯ We suggest the International Headache Society criteria be revised to remove specification of attack site, and to include the full range of cranial autonomic features. Currently, the sine qua non for paroxysmal hemicrania is a response to indometacin. Since there is no reliable clinical marker of that response we recommend an indometacin test, either orally or by injection for any patient with lateralized discrete attacks of head pain with associated cranial autonomic symptoms.
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Aquaporin-4 (AQP4) is a water channel protein expressed in astrocytes throughout the CNS. In brain, AQP4 facilitates water balance and glial scar formation, which are important determinants of outcome after injury. Here, we provide evidence for AQP4-dependent spinal cord swelling following compression injury, resulting in remarkably improved outcome in AQP4-null mice. ⋯ AQP4 immunoreactivity at the injury site was increased in grey and white matter at 48 h. Taken together, our findings indicate that AQP4 provides a major route for excess water entry into the injured spinal cord, which in turn causes spinal cord swelling and elevated spinal cord pressure. Our data suggest AQP4 inhibition or downregulation as novel early neuroprotective manoeuvres in spinal cord injury.