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患者,女,22岁。左肩部麻木及左半身不适感2年,症状以夜间明显。无头痛、眩晕及饮水呛咳等。2年来病情未进展。无烫伤史。查体:脊柱轻度侧弯。发育及咽反射正常。四肢肌张力及肌力正常,无肌肉萎缩,腱反射两侧对称,病理征(一)。左 T_4以下痛温觉减退,触觉正常。共济运动正常。颈椎及枕大孔区 X 线平片未见异常。头颅 CT 未见异常。肌电图检查正常。MRI:矢状面及冠状面上均见小脑扁桃体下移入椎管内达10cm,约于第二颈椎下缘平行,延髓亦轻度下移,颅颈交界区脊髓轻度扭曲。C_2至T_2椎间脊髓可见有空洞形成,此节段脊髓增粗,椎管矢状径亦达16mm 以上。讨论 Arnold-Chrari 畸形(ACM),又称小脑扁桃体延髓联合畸形,为先天发育异常,由扁桃体延长经枕骨大孔疝入颈段椎管;延髓和第四脑室同时亦向下延
Patient, female, 22 years old. Left shoulder numbness and left half-discomfort 2 years, the symptoms at night obvious. No headache, dizziness and drinking cough and so on. 2 years the disease has not progressed. No history of burns. Examination: mild curvature of the spine. Development and normal pharyngeal reflex. Limb muscle tension and muscle strength is normal, no muscle atrophy, tendon reflex bilateral symmetry, pathological sign (a). Left T_4 below the pain temperature decreased, the sense of touch is normal. Masonic movement is normal. Cervical and occipital foramen X-ray showed no abnormalities. Head CT no abnormalities. EMG examination is normal. MRI: The sagittal and coronal plane are seen in the cerebellar tonsillar movement down into the spinal canal about 10cm, about the lower edge of the second cervical parallel, the medulla oblongata also mild down slightly cranial border spinal cord distorted. C_2 to T_2 intervertebral spinal cord visible cavity formation, this section of the spinal cord thickening, spinal canal sagittal diameter of up to 16mm above. Discussion Arnold-Chrari deformity (ACM), also known as cerebellar tonsils medulla oblongata deformity, congenital anomalies, prolonged by tonsil foramen magnum hernia into the cervical canal; medulla oblongata and the fourth ventricle also down