CAMPTOCORMIA: THE BENT SPINE SYNDROME – A PARKINSONISM

For many years camptocormia, designating a forced posture with a forward-bent trunk, has been considered as related to psychiatric manifestations. Times have changed, and it is now well-known that somatic factors can produce this chronic abnormal curvature of the lumbar spine in standing position. Since the early wartime literature many developments have taken place, and it is now possible to establish a more precise etiologic diagnosis.

Camptocormia is an abnormal flexion of the trunk that appears when standing or walking and disappears in the supine position. The origin of the disorder is unknown, but it is usually attributed either to a primary or a secondary paravertebral muscle myopathy or a motor neuron disorder. Camptocormia can also be observed in a minority of patients with parkinsonism.

Patients with parkinsonism and camptocormia (excluding patients with multiple system atrophy) prospectively underwent a multidisciplinary clinical (neurological, neuropsychological, psychological, rheumatological) and neurophysiological (electromyogram, ocular movement recording) examination and were compared with age-matched patients with Parkinson’s disease without camptocormia.

The camptocormia developed after 8.5 years of parkinsonism, responded poorly to levodopa treatment (20%) and displayed features consistent with axial dystonia. Patients with camptocormia were characterized by prominent levodopa-unresponsive axial symptoms (ie, axial rigidity, gait disorder, and postural instability), along with a tendency for greater error in the antisaccade paradigm. (The anti-saccade (AS) task is a gross estimation of injury or dysfunction of the frontal lobe.

Neurologic disorders affecting both the frontal cortex or the basal ganglia have shown impaired performance on the anti-saccade task. These include schizophrenia, Huntington’s disease, Parkinson’s disease, and progressive supranuclear palsy. Additionally, disseminated brain disease such as Alzheimer’s disease or AIDS dementia also have an increased number of errors

We suggest that (1) the salient features of parkinsonism observed in patients with camptocormia are likely to represent a specific form of Parkinson’s disease and camptocormia is an axial dystonia and (2) both camptocormia and parkinsonism in these patients might result from additional, non-dopaminergic neuronal dysfunction in the basal ganglia.

Camptocormia is characterized by an abnormal flexion of the trunk appearing in standing position, increasing while walking and abating in the supine position. The term camptocormia is sometimes referred to as “bent spine syndrome” (BSS). The abnormal curvature must be a lumbar kyphosis. An anterior flexion of 45° or more is required to differentiate BSS from the kyphosis starting from the dorsal spine, frequently observed in elderly individuals.