Bloch F, Houeto J L, Tezenas du Montcel S, Bonneville F, Etchepare F, Welter M L, Rivaud-Pechoux S, Hahn-Barma V, Maisonobe T, Behar C, Lazennec J Y, Kurys E, Arnulf I, Bonnet A M, Agid Y
Centre d'Investigation Clinique-Fédération des Maladies du Système Nerveux, Groupe-Hospitalier Pitié-Salpêtrière, Paris, France.
J Neurol Neurosurg Psychiatry. 2006 Nov;77(11):1223-8. doi: 10.1136/jnnp.2006.087908. Epub 2006 Jun 5.
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.
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.
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.
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.
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（躯干前屈症）定义为站立或行走时出现的躯干异常前屈，仰卧位时消失。该病症的病因不明，但通常归因于原发性或继发性椎旁肌肌病或运动神经元疾病。在少数帕金森病患者中也观察到躯干前屈症。
与无躯干前屈症的帕金森病患者相比，描述帕金森病合并躯干前屈症患者的躯干前屈症及帕金森症状的临床和电生理特征。
对帕金森病合并躯干前屈症患者（排除多系统萎缩患者）进行前瞻性多学科临床（神经科、神经心理学、心理学、风湿病学）和神经生理学（肌电图、眼动记录）检查，并与年龄匹配的无躯干前屈症的帕金森病患者进行比较。
躯干前屈症在帕金森病发病8.5（标准差5.3）年后出现，对左旋多巴治疗反应不佳（20%），表现出与轴性肌张力障碍一致的特征。有躯干前屈症的患者的特点是左旋多巴无反应的轴性症状突出（即轴性强直、步态障碍和姿势不稳），以及在反扫视范式中出现更大误差的倾向。
我们认为，（1）有躯干前屈症的患者中观察到的帕金森病显著特征可能代表帕金森病的一种特殊形式，且躯干前屈症是一种轴性肌张力障碍；（2）这些患者的躯干前屈症和帕金森病可能都源于基底神经节中额外的非多巴胺能神经元功能障碍。
