Camptocormia in idiopathic Parkinson's disease: A focal myopathy of the paravertebral muscles

Abstract

The objective of our study was to describe the clinical features of camptocormia, an involuntary, marked flexion of the thoracolumbar spine in idiopathic Parkinson's disease (PD) and to understand its etiology. In a prospective, cross-sectional study, we examined 15 patients with PD and camptocormia using laboratory parameters, EMG, muscle magnetic resonance imaging, and biopsy of the paravertebral muscles. The clinical data were compared with a matched control group of PD patients without camptocormia, and the biopsies were compared with muscles from age-matched autopsies. Almost all the patients (median age, 68.0 years; 7 women) with camptocormia suffered from advanced PD. Camptocormia occurred at a median of 9.0 years after the PD diagnosis. Compared with our clinical control group, back pain was more frequent and less dopa-sensitive in the patients with camptocormia who suffered more often from additional diseases of the back. On EMG, we found mainly a myopathic pattern. The MRI of the paravertebral muscles showed localized changes ranging from edema with contrast enhancement, which are considered to be early signs, to atrophy and/or fatty degeneration, interpreted as late degenerative changes. Early signs were seen mainly during the first year and degenerative changes after 1.5 years. Biopsies revealed consistently myopathic changes and in some cases fatty degeneration. Clinical or electromyographic features favoring dystonia were absent. Camptocormia is a major disabling, non-fluctuating and levodopa-resistant complication of advanced PD. The cause of camptocormia in idiopathic PD is a focal myopathy. Our findings suggest that the myopathy has a progressive course, which finally leads to degeneration of the paravertebral muscles.

Mood and behavioural effects of subthalamic stimulation in Parkinson's disease

“…68,70,71 Paraspinal muscle biopsies showed unspecific myopathic changes with type 1 fiber hypertrophy, type 2 fiber loss, loss of oxidative enzyme activity, and myofibrillar disorganization. 68 Similar myopathic changes have been found in patients with tenotomy, suggesting that they could be secondary to altered proprioception in PD and result from, rather than induce, chronic postural deviations.…”

Presentation, Etiology, Diagnosis, and Management of Camptocormia

“…Camptocormia is enhanced during standing and walking [17] and relieved in recumbent or supine position [18,19,20]. In quite a number of cases, camptocormia is associated with lower back pain [1,3,7,21,22,23,24] but in others it is painless [19]. Camptocormia may be associated with concomitant weakness of the gluteus maximus and hip and genuflexion [3].…”

“…The CNS disorder most frequently reported in association with camptocormia is Parkinson’s disease [1,12]. Clinical features of camptocormia in Parkinson’s disease include old age, predominantly male sex, long disease duration, and early axial involvement [3].…”

“…Only in single cases camptocormia may be the initial manifestation of Parkinson’s disease [30]. Camptocormia in Parkinson’s disease starts to develop between the age of 60 and 70 years [3] and the latency with which camptocormia occurs after onset of Parkinson’s disease is 5–10 years [1,31]. Camptocormia is most frequently found in patients with more severe Parkinson’s disease.…”

“…Camptocormia, also known as bent spine syndrome, or cyphose hystérique, is a major disabling, non-fluctuating, acquired postural abnormality due to various different causes, characterized by an involuntary forward-flexed posture of the thoracolumbar spine [1,2]. The term camptocormia derives from the Greek words ‘kamptos’ (to bend) and ‘cormos’ (trunk) [3].…”