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March 1975

Congenital Insensitivity to Noxious Stimuli

Author Affiliations

From the Division of Electroencephalography and Clinical Neurophysiology (Dr. Chatrian and Mr. Lettich), and the Division of Neurology (Dr. Farrell), University of Washington School of Medicine, and the Department of Restorative Dentistry (Dr. Canfield), University of Washington School of Dentistry, Seattle.

Arch Neurol. 1975;32(3):141-145. doi:10.1001/archneur.1975.00490450021001

Cerebral-evoked potentials were used to study a 25-year-old man, the older of two siblings with congenital insensitivity to all noxious stimuli, gross impairment of temperature perception, and anhidrosis. Electrical stimulation of tooth pulp consistently eliciting pain and cerebral responses in normal subjects evoked neither cerebral potentials nor painful or other sensations in our patient. However, ordinarily painful electric shocks to the skin of his face evoked cerebral responses as well as sensations lacking disagreeable qualities. Those cerebral potentials elicited by electrical stimulation of the median nerve, clicks, and light flashes were within normal limits. These findings strongly suggest that a defect in transmission of noxious impulses presumably involving first order sensory neurons exists in our patient.

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