Camptocormia is a disabling pathological, non-fixed, forward bending of the trunk. The clinical definition using only the bending angle is insufficient; it should include the subjectively perceived inability to stand upright, occurrence of
back pain, typical individual complaints, and need for walking
aids and compensatory signs (e.g. back-swept wing sign). Due to the heterogeneous etiologies of
camptocormia a broad diagnostic approach is necessary.
Camptocormia is most frequently encountered in
movement disorders (PD and
dystonia) and muscles diseases (
myositis and
myopathy, mainly facio-scapulo-humeral
muscular dystrophy (FSHD)). The main diagnostic aim is to discover the etiology by looking for signs of the underlying disease in the neurological examination, EMG, muscle MRI and possibly biopsy. PD and probably myositic
camptocormia can be divided into an acute and a chronic stage according to the duration of
camptocormia and the findings in the short time inversion recovery (STIR) and T1 sequences of paravertebral muscle MRI. There is no established treatment of
camptocormia resulting from any etiology. Case series suggest that
deep brain stimulation (DBS) of the subthalamic nucleus (STN-DBS) is effective in the acute but not the chronic stage of PD
camptocormia. In chronic stages with degenerated muscles, treatment options are limited to
orthoses, walking
aids, physiotherapy and
pain therapy. In acute myositic
camptocormia an escalation strategy with different immunosuppressive drugs is recommended. In dystonic
camptocormia, as in
dystonia in general, case reports have shown
botulinum toxin and DBS of the globus pallidus internus (GPi-DBS) to be effective.
Camptocormia in connection with primary
myopathies should be treated according to the underlying illness.