Parikh Roneil N, Muranjan Mamta, Karande Sunil, Sankhe Shilpa
Department of Pediatrics, Seth Gordhandas Sunderdas Medical College and King Edward VII Memorial Hospital, Parel, Mumbai, India.
Department of Pediatrics, Seth Gordhandas Sunderdas Medical College and King Edward VII Memorial Hospital, Parel, Mumbai, India.
Pediatr Neurol. 2014 Apr;50(4):380-3. doi: 10.1016/j.pediatrneurol.2013.11.014. Epub 2013 Nov 22.
Symptomatic atlantoaxial dislocation is common in predisposing genetic or acquired disorders. However, an isolated atlantoaxial dislocation frequently is congenital and silent unless discovered during course of evaluation for neurological symptoms of cervical spinal cord injury attributed to minor or chronic, repetitive trauma.
A 12-year-old girl working as a farm laborer developed calf pain provoked by walking, which increased in severity and progressed to involve the upper limbs. It was followed by progressive ascending quadriplegia. The illness resembled acute inflammatory demyelinating polyneuropathy with respiratory involvement. Presence of "claw" hands bilaterally and wasting of intrinsic muscles of the hands led to the suspicion of a cervical myelopathy.
Neuroimaging confirmed a congenital atlantoaxial dislocation with basilar invagination. The absence of abnormal signals in the cervical spinal cord was unusual. The symptomatic congenital atlantoaxial dislocation was postulated to be precipitated by chronic trauma suffered while habitually carrying heavy loads on the head and leading to spinal cord injury without radiographic abnormalities. Correction via surgery was successful.
Congenital atlantoaxial dislocation should be suspected in a clinical setting of neurological symptoms of cervical spinal cord injury without obvious trauma or predisposing primary diseases. Prompt cervical spine imaging reveals the correct diagnosis. Physicians in countries in which child labor is rampant should be aware of the potential complications of cervical cord injuries from child labor.
症状性寰枢椎脱位在遗传性或后天性易感疾病中很常见。然而,孤立性寰枢椎脱位通常是先天性的且无症状,除非在因轻微或慢性重复性创伤导致颈脊髓损伤的神经症状评估过程中被发现。
一名12岁从事农活的女孩出现行走诱发的小腿疼痛,疼痛程度加重并进展至上肢。随后出现进行性上升性四肢瘫。病情类似伴有呼吸受累的急性炎症性脱髓鞘性多发性神经病。双侧“爪形”手及手部内在肌萎缩导致怀疑存在颈髓病。
神经影像学检查证实为先天性寰枢椎脱位伴基底凹陷。颈脊髓无异常信号较为少见。推测有症状的先天性寰枢椎脱位是由于习惯性头部负重导致慢性创伤,进而引起脊髓损伤但无影像学异常。手术矫正成功。
在无明显创伤或易感原发性疾病的颈脊髓损伤神经症状临床背景下,应怀疑先天性寰枢椎脱位。及时进行颈椎成像可明确诊断。在童工泛滥的国家,医生应意识到童工导致颈髓损伤的潜在并发症。