Historical Background

Reports of individual who appeared insensitive to pain from birth onwards have a long history but it was not until the 1930’s that this condition attracted medical attention.

The 1^st case was reported by Dr. Dearborn in 1932 — he described his patient who acts as a human pincushion in a circus and crucifixion had to be called off when a woman in the audience fainted after a spike was driven through his one hand.

Initially, various terms were used to describe these individuals including ‘Congenital General Pure Analgesia’ (Dearborn, 1932), ‘Congenital Universal Insensitiveness to Pain’ (Ford & Wilkinsons, 1938), ‘Congenital Universal Indifference to Pain’ (Winkelmann et al., 1962). As these labels show, the phenomenon encompasses diverse abnormal responses to pain. Some patients have an absence of responses to injury, abnormal automatic responses to painful stimuli and difficulties in distinguishing various types of stimuli, whereas others exhibit lack of responsiveness to the stimuli but retain the ability to identify stimulus presence and mobility.

Over time 2 terms predominantly describe these individuals — ‘Congenital Insensitivity to Pain’ (McMurray 1950) and ‘Congenital Indifference to Pain’ (Jewesbury, 1970). Although the terms were often used interchangeably, only in recent years they have acquired distinct meanings and careful authors now to use them to distinguish two groups of individuals (Jewesbury, 1970; Landrieu et al., 1990).

Patients with congenital insensitivity pain seem not to perceive sensations of pain i.e. they have remarkably impaired ability to perceive the type, intensity and quality of painful stimuli. On the other hand, those with congenital indifference to pain, however, painful stimuli are perceived but there is an absence of the affective response to pain, rather than a lack of signal transmission. These individual reports experiencing sensations of pain but exhibit no aversion to or withdrawal from painful stimuli.