Durán E, Chacón J R
Servicio de Neurología, Hospital Universitario Virgen Macarena, Sevilla, España.
Rev Neurol. 2001;32(1):60-2.
Spasmodic torticollis in young patients should give rise to a clinical suspicion that this is secondary to another primary disorder. Therefore a series of diagnostic tests should be carried out before it is labelled as idiopathic.
The patient was a thirty year old man who had had difficulty in writing with his right hand since childhood. At the age of 20 years he was diagnosed as having writer's cramp and idiopathic spasmodic torticollis. On general physical examination no abnormalities were found. On neurological examination he had: absence of reflexes of both arms, limited but painless rotation of the neck towards the left and hypertrophy of the left trapezius muscle. Laboratory, neurophysiological and neuroimaging investigations seeking a secondary cause for the torticollis were all normal. There were no Keyser-Fleischer rings. Chest X-ray showed, dorsal scoliosis with convexity to the left. CAT and MR of the spine showed a hemangioma in the body of T1. On arteriography of the supra-aortic and vertebral trunks a hemangioma was found at T1 which received contrast material via a branch of the right thyro-bi-cervico-scapular trunk. Various treatments were tried (diazepam, Botox, Dysport, tetrabenazine, baclofen, etc.) with no improvement. A definite diagnosis of secondary torticollis could not be made since the hemangioma was supplied by a very narrow vascular pedicle, so embolization was contraindicated.
Cervical spinal cord alterations may cause focal dystonia due to increased excitability of the spinal motor neurone, due to dysfunction of the disinhibitory descending reciprocal paths.
年轻患者出现痉挛性斜颈时,临床上应怀疑这是继发于另一种原发性疾病。因此,在将其标记为特发性之前,应进行一系列诊断测试。
患者为一名30岁男性,自幼右手书写困难。20岁时被诊断为书写痉挛和特发性痉挛性斜颈。全身体格检查未发现异常。神经系统检查发现:双臂反射消失,颈部向左旋转受限但无痛,左侧斜方肌肥大。为寻找斜颈的继发原因而进行的实验室、神经生理学和神经影像学检查均正常。无Kayser-Fleischer环。胸部X线显示,脊柱后凸向左。脊柱的计算机断层扫描(CAT)和磁共振成像(MR)显示T1椎体有一个血管瘤。在主动脉弓上和椎动脉干的血管造影中,发现T1处有一个血管瘤,它通过右甲状腺-双颈-肩胛干的一个分支接受造影剂。尝试了各种治疗方法(地西泮、肉毒杆菌毒素、Dysport、丁苯那嗪、巴氯芬等),但均无改善。由于血管瘤由非常狭窄的血管蒂供血,因此不能进行栓塞,所以无法明确诊断为继发性斜颈。
颈脊髓改变可能由于脊髓运动神经元兴奋性增加,以及去抑制性下行交互通路功能障碍而导致局灶性肌张力障碍。