[Congenital pain-insensitivity syndrome; a rare indication of the benefit of pain].
A 34-year-old woman with a known congenital pain-insensitivity syndrome presented because of increasing weakness and sensory loss in her right leg. The cause was a rapidly progressive partial caudal compression syndrome in the absence ofknown prior trauma. Radiology revealed a lumbar Charcot spine, i.e. total destruction of the spine with compression of the dural sac. Emergency surgery included opening of the lumbar canal and spondylodesis. Postoperatively, there was almost full neurological recovery. In the pathogenesis the absence of protective pain sensation combined with trophic degeneration due to neurovascular dysregulation may play a role.
Kruyt
M C
MC
Afd. Orthopedie, Universitair Medisch Centrum Utrecht, Utrecht. mkruyt@umcutrecht.nl
Kruyt
N D
ND
Oner
F C
FC
Hanlo
P W
PW
Verbout
A J
AJ
D000328:Adult; D018450:Disease Progression; D005260:Female; D007839:Functional Laterality; D006801:Humans; D010146:Pain / Q000453:epidemiology*; D010291:Paresis / Q000175:diagnosis / Q000601:surgery; D013117:Spinal Cord Compression / Q000175:diagnosis* / Q000601:surgery*; D016896:Treatment Outcome