Spinal Deformity and Parkinson Disease: A Treatment Algorithm

Cheerag D. Upadhyaya, M.D.; Philip A. Starr, M.D., Ph.D.; Praveen V. Mummaneni, M.D.


Neurosurg Focus. 2010;28(3):E5 

In This Article


Camptocormia, or "bent spine syndrome," is an extreme forward flexion of the thoracolumbar spine, which often worsens during standing or walking, but completely resolves when supine. The term itself is derived from the Greek "kamptos" (to bend) and "kormos" (trunk). While the condition was described as early as 1818, the term camptocormia was first proposed in 1914 to describe the forward flexion posture of some soldiers in World War I who had to move through the trenches in a bent posture to avoid injury.[8,18,28,31]

Camptocormia is used to describe the extreme forward flexion of the spine associated with a number of causes including dystonia, Tourette syndrome, amyotrophic lateral sclerosis, myopathy, myositis, multiple system atrophy, PD, and conversion disorder. While initially thought to be a rare manifestation of PD, recent estimates of the prevalence of camptocormia in patients with PD vary from 3–12.9%.[2,20,33] It is unclear if the prevalence of camptocormia varies with the severity of the PD.[3,6,11,20,33]

Medical management of camptocormia in PD remains suboptimal. Azher and Jankovic[3] have reviewed the cases of 16 patients with camptocormia associated with PD (11 patients), dystonia (4 patients), and Tourette syndrome (1 patient). Twelve patients received levodopa therapy with minimal or no improvement in camptocormia. In 9 patients botulinum toxin Type A was administered into the rectus abdominus muscle, with improvement in 4 patients. Ho et al.[17] have described a single patient in whom camptocormia improved after adjustments in dopaminergic therapy. Von Coelln and colleagues[37] reported on 4 patients with PD who received ultrasound-guided injections of botulinum toxin Type A into the iliopsoas muscle. While they found the technique to be safe, with only patients receiving the highest doses reporting mild weakness of hip flexion, they also found no significant postural improvement. Bloch et al.[6] have reported a case control study (8 patients in each arm) and found that patients with PD and camptocormia responded poorly to levodopa treatment and had levodopa-unresponsive axial symptoms.

Subthalamic nucleus DBS has been reported to improve camptocormia associated with PD. Sako et al.[27] have reported on 6 patients in whom they documented a mean improvement of 78 ± 9.1% in thoracolumbar angle after bilateral STN stimulation. Hellmann et al.,[16] Yamada et al.,[39] and Micheli et al.[23] each reported on a single case of PD and camptocormia in which the patient improved after bilateral subthalamic DBS or GPi stimulation. Reports on the responsiveness of camptocormia in PD patients have been inconsistent. Of the 16 patients reported on by Azher and Jankovic,[3] one underwent placement of bilateral STN electrodes with no improvement in camptocormia. Most recently, Umemura et al.[34] reported on a retrospective analysis of 18 patients (8 with camptocormia, 10 with Pisa syndrome) who underwent subthalamic DBS placement. In 13 patients with a moderate postural abnormality, 11 patients ultimately improved. In the 5 patients with severe postural abnormality, 2 patients improved slightly. Deep brain stimulation has also been reported to improve camptocormia associated with other movement disorders. Fukaya et al.[13] reported improvement of camptocormia in 3 patients with primary dystonia who underwent placement of bilateral GPi DBS. Nandi et al.[24] published a case report of a patient with tardive dyskinesia and camptocormia who responded to the placement of bilateral GPi electrodes for DBS. However, it is unclear if the pathophysiology of camptocormia in PD is similar to that of camptocormia associated with primary dystonia.

Babat et al.[4] reported on 14 patients with PD who underwent spinal surgery (mostly short-segment laminectomies/fusions; 1 patient underwent multiple-level cervical corpectomy, 1 underwent deformity correction, and 1 underwent L-1 transpedicular decompression for burst fracture). They noted that 11 patients underwent 22 additional operations at the same or adjacent levels for instability. Four of these patients had hardware failure or pullout, requiring 10 additional operations. Their conclusion was that the primary cause of failure was persistent kyphosis or segmental instability. Peek et al.[26] recently described the case of a patient with PD and camptocormia who underwent posterior T7–ilium fixation. The patient required several surgical revisions, prolonged hospitalization, and rehabilitation. Although they were ultimately successful in restoring spinal balance, their conclusion was to consider surgical intervention only after subthalamic nucleus DBS has been performed and then only in patients who were highly motivated to walk.