Parkinson's disease with camptocormia

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, non‐dopaminergic neuronal dysfunction in the basal ganglia.Camptocormia is defined as an abnormal flexion of the trunk that appears when standing or walking and disappears in the supine position (fig 1​1).). This rare symptom, usually found in patients >65 years of age, is often attributed to normal ageing and is not diagnosed. The term “camptocormia” is derived from the Greek words “kamptos” (to bend) and “kormos” (trunk), and was coined by the French neurologist Souques.¹ The authors reported a soldier, who had a forced posture with a bent‐forward trunk after a gunshot wound. The patient was reportedly cured after a psychotherapeutic interview and subsequent application, in narcosis, of a plaster jacket that was removed after 2 days. Souques even suggested that the disorder could be cured completely and permanently by means of a persuasive galvanisation or faradisation of the dorsolumbar region, and termed the condition “cyphose hystérique” (“hysterical kyphosis”). Although a few authors pointed out that patients with an organic condition such as spondylitis should be differentiated from those with hysterical kyphosis, a psychogenic explanation for the disorder prevailed, owing to the many cases similar to those of Souques that were reported during the First and Second World Wars.^2,3,4,5,6Open in a separate windowFigure 1 Drawing of a patient with Parkinson''s disease and camptocormia (Bibliothèque Charcot, Hôpital de la Salpêtrière; left) and photographs of one of the patients studied (camptocormia appears when the patient is standing or walking and disappears in the supine position; right). Patient consent was obtained for publication of this figure.In 1995, Laroche et al⁷ reported 37 patients with camptocormia who were compared with 15 age‐matched patients without camptocormia but with posterior interapophysial osteoarthritis and elderly patients surgically treated for narrowing of the lumbar canal. In patients with camptocormia, magnetic resonance imaging (MRI) showed features consistent with circumscribed myopathy in the paravertebral muscles, and it was stated that camptocormia might be ascribed to a primary paravertebral myopathy.⁸ The spectrum of these neuromuscular disorders was subsequently extended to myasthenia gravis, nemaline myopathy,^9,10 amyotrophic lateral sclerosis,¹¹ inclusion body myositis, polymyositis^9,12 and miscellaneous causes such as paraneoplastic disorder¹³ and valproate toxicity¹⁴ (see also review by Azher and Jankovic¹⁵). None of the above studies, however, mentioned the presence of parkinsonism. Indeed, in the few studies that have dealt with the issue of camptocormia and parkinsonism, the coexistence of these disorders was thought to result from the incidental occurrence of Parkinson''s disease and neuromuscular disorders,^9,15,16 features of multiple systemic atrophy (MSA),^9,15 or a rare type of dystonia peripherally induced¹⁵ or of unknown origin.^9,15,17 Although some studies were the first to draw attention to the disorder,¹⁷ the nosological position and the origin of this unusual symptom in association with parkinsonism remain unclear, as do the precise clinical features of this entity.The aim of the present prospective study was to characterise a selected sample of patients with parkinsonism and camptocormia and to compare them with age‐matched patients with Parkinson''s disease without camptocormia.