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Acute mountain sickness and high altitude cerebral edema are related neurological disorders that strike unacclimatized persons on fast ascent to high altitude. While the clinical aspects of diagnosis and treatment of a cerebral syndrome are well established, their pathophysiology remains elusive. Recent studies show that the exposure to hypobaric hypoxia during fast ascent to high altitude enhances development of cerebral vasogenic edema before the onset of symptoms and signs of acute mountain sickness. Reasons for increasesd blood-brain barrier permeability might include mechanical factors (loss of autoregulation and increased capillary pressure), ischaemia, neurogenic influences and biochemical mediators. In the advanced stages of the disease, vasogenic edema is probably accompanied by cytotoxic edema. Acute mountain sickness and clinical entity »cerebral edema« are considered two clinical ends of the cerebral syndrome in high altitude illness with the same pathophysiological background. Symptoms of cerebral syndrome in high altitude illness are related to increased intracranial pressure. It seems that the evolution of symptoms depends on the compliance of the craniospinal space.