Parkinson's disease with camptocormia

Abstract

Background: Camptocormia is defined as 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 neurone disorder. Camptocormia is also observed in a minority of patients with parkinsonism. Objective: To characterise the clinical and electrophysiological features of camptocormia and parkinsonian symptoms in patients with Parkinson's disease and camptocormia compared with patients with Parkinson's disease without camptocormia. Methods: 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. Results: The camptocormia developed after 8.5 (SD 5.3) years of parkinsonism, responded poorly to levodopa treatment (20%) and displayed features consistent with axial dystonia. Patients with camptocormia were characterised 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. Conclusion: 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, nondopaminergic neuronal dysfunction in the basal ganglia.

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 term camptocormia derives from the Greek words ‘kamptos’ (to bend) and ‘cormos’ (trunk) [3]. The abnormal posture leads to lumbar kyphosis and is increased or only visible when the patient stands up or walks [3,4]. Camptocormia is most frequently due to disorders of the striated muscles of the spinal column, due to central nervous system (CNS) disorders, or due to some rare conditions (table 1).…”

“…The term camptocormia derives from the Greek words ‘kamptos’ (to bend) and ‘cormos’ (trunk) [3]. The abnormal posture leads to lumbar kyphosis and is increased or only visible when the patient stands up or walks [3,4].…”

Spinal deformity and Parkinson disease: a treatment algorithm

“…While they found the technique to be safe, with only patients receiving the highest doses reporting mild weakness of hip flexion, they also found no significant postural improvement. Bloch et al 6 have reported a case control study (8 patients in each arm) and found that patients with PD and camptocormia responded poorly to levodopa treatment and had levodopa-unresponsive axial symptoms.…”

“…2,20,33 It is unclear if the prevalence of camptocormia varies with the severity of the PD. 3,6,11,20,33 Medical management of camptocormia in PD remains suboptimal. Azher and Jankovic 3 have reviewed the cases of 16 patients with camptocormia associated with PD (11 patients), dystonia (4 patients), and Tourette syndrome (1 patient).…”