Camptocormia: the bent spine syndrome, an update
2010
Camptocormia, also referred to as bent spine syndrome (BSS) is defined as an abnormal flexion of the trunk, appearing in standing position, increasing during walking and abating in
supine position. BSS was initially considered, especially in wartime, as a psychogenic disorder. It is now recognized that in addition to psychiatric syndromes, many cases of reducible BSS have a somatic origin related to a number of musculo-skeletal or
neurological disorders. The majority of BSS of muscular origin is related to a primary idiopathic axial
myopathyof late onset, appearing progressively in elderly patients. Diagnosis of axial
myopathyfirst described by Laroche et al. is based upon CT/MRI examination demonstrating massive fatty infiltration of paravertebral muscles. The non-specific histological aspect includes an extensive endomysial fibrosis and fat tissue with irregular degenerated fibers. Weakness of the paravertebral muscles can be secondary to a wide variety of diseases generating diffuse pathologic changes in the muscular tissue. BSS can be the predominant and sometimes revealing symptom of a more generalized muscular disorder. Causes of secondary BSS are numerous. They must be carefully assessed and ruled out before considering the diagnosis of primary axial
myopathy. The principal etiologies include on the one hand
inflammatory myopathies, muscular dystrophies of late onset, myotonic
myopathies, endocrine and
metabolic myopathies, and on the other hand
neurological disorders, principally Parkinson’s disease.
Camptocormiain Parkinsonism is caused by axial dystonia, which is the hallmark of Parkinson’s disease. There is no specific pharmacologic treatment for primary axial
myopathy. General activity, walking with a cane, physiotherapy, and exercises should be encouraged. Treatment of secondary forms of BSS is dependent upon the variety of the disorder generating the muscular pathology. Pharmacologic and general management of
camptocormiain Parkinson’s disease merge with that of Parkinsonism.
Levodopatreatment, usually active on tumor rigidity and akinesia, has poor or negative effect on BSS.
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