The exact nosological status of ''congenital insensitivity to pain'' r
emains in doubt. Possible pathological correlates of this clinical syn
drome include sensory neuropathy, central lesions at the level of the
reticular formation or dorsal horn of the spinal cord, or a central in
difference to, or asymbolia for, pain. The reassessment of two members
of a kindred previously reported more than 20 years ago as having con
genital insensitivity to pain indicated that they in fact had an inher
ited sensory and autonomic neuropathy. Prolonged follow up and morphom
etric analysis of sequential nerve biopsies may be necessary to defini
tively establish this diagnosis.