[Long-term course in congenital analgesia]

We describe the longterm history of eight patients with congenital insensitivity to pain, a rare autosomal recessive condition. Pain perception did not significantly improve with increasing age, but all affected individuals learned to live with their handicap. A marked tendency to dissimulate was obvious. The longterm outcome was dominated by orthopedic complications: there were impressive arthropathic changes in knee and ankle joints but - to our surprise - no hip joint involvement. These changes are the result of skeletal injuries in childhood (fractures, aseptic bone necrosis, osteomyelitis) and of apparently progressive neuropathic lesions due to pain insensitivity. The latter resulted in marked lumbar spine changes with progressive neurological deterioration, so far requiring spondylodesis in three patients. In three pregnant women total absence of labour pain was observed. All affected individuals had anosmia but no deficits of autonomic nervous system functions.