Cephalalgia : an international journal of headache
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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene and is clinically characterized by recurrent stroke, cognitive decline, psychiatric disturbances and migraine. The prevalence of migraine in CADASIL is slightly higher than in the general population, and the proportion of migraine with aura is much higher. The pathophysiological mechanism that leads to increased aura prevalence in CADASIL is unknown. ⋯ It is also possible that the brainstem migraine area is involved in CADASIL. Last, it is possible that the NOTCH3 mutation acts as a migraine aura susceptibility gene by itself. In this narrative review we summarize the literature about migraine in CADASIL, with a special focus on what CADASIL might teach us about the pathophysiology of migraine.
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Pneumoceles of the paranasal sinuses are a very rare condition; characterized by a distended air-filled paranasal sinus extending beyond the margins of the paranasal bone, with bony defects and extension of air into the surrounding soft tissues. Also, spontaneous pneumo-cephalus is a rare condition which represented only 0.6% in the largest reported series of pneumocephalus. Although pneumocephalus caused by sinogenic origins, such as osteoma, has been reported, spontaneous pneumocephalus has not been reported as a complication associated with pneumocele of the frontal sinus. ⋯ Although pneumocele of the frontal sinus is a very rare condition, it can develop into spontaneous pneumocephalus, and thus it needs to be included in the differential diagnosis of acute severe headache.