Charcot spinal arthropathy in patients with congenital insensitivity to pain: a report of two cases and review of the literature

Neurosurg Rev. 2018 Oct;41(4):899-908. doi: 10.1007/s10143-017-0814-3. Epub 2017 Jan 26.

Abstract

Charcot spine, or neuropathic spinal arthropathy, involves the progressive destruction of the spinal joint due to the lack of normal protective sensations and proprioception. A rare cause of Charcot spine is congenital insensitivity to pain, which is an absent or abnormal response to painful stimuli. There are few case reports describing this condition, and long-term follow-up data are limited. The presentation and treatment of two patients with Charcot spine secondary to congenital insensitivity to pain are described. Both cases were characterized by lumbar involvement and were treated with circumferential decompression and an extended lumbo-pelvic fusion construct. The cases described here demonstrate stable neurological status at 1.5 and 5 years follow-up. Patient characteristics, pre- and post-operative imaging, operative approach, and outcomes are described. The literature regarding this rare condition is also reviewed, with an emphasis on operative management and outcomes. Surgical management is traditionally complicated by a high rate of hardware failure and adjacent segment degeneration. The current review highlights the importance of prompt and aggressive management following diagnosis of Charcot spine, as well as extended follow-up.

Keywords: Charcot spine; Congenital insensitivity to pain; Deformity; Hereditary sensory and autonomic neuropathy; Neuroarthropathy; Neuropathic spinal arthropathy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Arthropathy, Neurogenic / complications
  • Arthropathy, Neurogenic / diagnostic imaging
  • Arthropathy, Neurogenic / surgery*
  • Disease Progression
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Mobility Limitation
  • Muscle Weakness / etiology
  • Neurosurgical Procedures / methods*
  • Pain Insensitivity, Congenital / complications
  • Pain Insensitivity, Congenital / diagnostic imaging
  • Pain Insensitivity, Congenital / surgery*
  • Spinal Fusion / methods
  • Spine / abnormalities
  • Spine / diagnostic imaging