Camptocormia: the bent spine syndrome, an update

Abstract

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 myopathy of late onset, appearing progressively in elderly patients. Diagnosis of axial myopathy first 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. Camptocormia in 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 camptocormia in Parkinson's disease merge with that of Parkinsonism. Levodopa treatment, usually active on tumor rigidity and akinesia, has poor or negative effect on BSS.

Camptocormia in Parkinson’s Disease: A Review of the Literature

“…For instance, Sławek et al [34] describe a 49-year-old woman with PD who showed a painful trunk flexion/CC which improved markedly after unilateral pallidotomy. The only case-control study performed by Bloch et al [21] suggests an axial or action dystonia of the trunk with excessive activation of the abdominal-wall muscles, supported by the observations that CC worsens during walking and standing, and that fatigue worsens CC [7,14] . Additional symptoms accompanied by axial dystonia could be striatal hand and foot deformities [7] .…”

“…The range between onsets of PD and CC varies widely (0-9 years) [22] . CC seems to worsen with increasing fatigue and during 'off' periods [7,14,23] .…”

Rigid Spine Syndrome in Children

“…The condition is common in old age. Various conditions related to muscles, bones, systemic diseases, drugs or neurological diseases result in rigid spine (1,2). Rigid spine is rare in children and the two most common causes are the primary muscle disease end stage and rigidity at onset of a rare muscle disease called primary rigid spine syndrome.…”

Rigid Spine Syndrome among Children in Oman

“…Differences have been noted between the underlying aetiopathology of a rigid spine in children and adults, although the usual causes are bone, neuromuscular and brain disorders. 3,4 Inheritance patterns also differ between adults and children as the condition is generally autosomal dominant in the former and recessive in the latter. 5 The disease presents between the 4-6 th decade of life for adults and during the first decade for children.…”

“…5 For adults, this condition is known as axial myopathy, camptocormia, rigid spine syndrome (RSS) and, more recently, paraspinal neuromuscular syndrome. 4,6,7 Rigidity of the spine is noted in the late stages of several childhood muscular dystrophies and myopathies. In these diseases, the rigid spine is due to the spread of the disease to the muscles and the late involvement of the paraspinal muscles.…”