The startle response is a universal and phylogenetically ancient reflex. Pathological exaggerations and modifications of startle underlie the symptomatology of a surprisingly diverse range of neuropsychiatric disorders, a fact that seems to have gone largely unappreciated. We review the available literature on the physiology of the normal human startle response and examine the neuropsychiatric conditions characterized by pathological startle. Startle epilepsy and primary hyperekplexia are considered as neurological disorders involving abnormal startle. Patients with hyperstartle and exotic culture-bound syndromes characterized by excessive startle are considered by the authors to represent extreme variants of the normal startle response. Post-traumatic stress disorder, drug and alcohol withdrawal states and schizophrenia all have abnormal startle as a clinical feature secondary to increased arousal and presumed disturbance of central neurotransmitter systems. The neurophysiological mechanisms by which abnormalities of the startle response may occur are discussed and a system of classification of neuropsychiatric hyperstartles is suggested.