Barata Pedro Coelho, Morgado Joana, Sousa Ana Paula, de Oliveira Sónia Duarte, Custódio Maria Paula, da Costa Lígia Bruno, Pena José Esteves
Oncology Department, Centro Hospitalar Lisboa Central, Lisboa, Portugal.
Case Rep Oncol. 2012 Sep;5(3):616-21. doi: 10.1159/000345692. Epub 2012 Sep 20.
Paraneoplastic neurologic syndromes (PNS) pose quite an uncommon neurological complication, affecting less than 1% of patients with breast cancer. Nearly one third of these patients lack detectable onconeural antibodies (ONAs), and improvement in neurologic deficits with concomitant cancer treatments is achieved in less than 30% of cases.
A 42-year-old, premenopausal woman presented with facial paralysis on the central left side accompanied by a left tongue deviation, an upward vertical nystagmus, moderate spastic paraparesis, dystonic posturing of the left foot, lower limb hyperreflexia and bilateral extensor plantar reflex. After ruling out all other potential neurologic causes, PNS was suspected but no ONAs were found. A PET-CT scan detected increased metabolism in the right breast, as well as an ipsilateral thoracic interpectoral adenopathy. Core biopsy confirmed the presence of an infiltrating duct carcinoma. After breast surgery, the neurologic symptoms disappeared. One week later, the patient was readmitted to the hospital with a bilateral fatigable eyelid ptosis, and two weeks later, there was a noticeable improvement in eyelid ptosis, accompanied by a rapid and progressive development of lower spastic paraparesis. She started adjuvant treatment with chemotherapy with marked clinical and neurological improvement, and by the end of radiotherapy, there were no signs of neurologic impairment.
This case study highlights the importance of a high level of vigilance for the detection of PNS, even when ONAs are not detected, as the rapid identification and treatment of the underlying tumor offers the best chance for a full recovery.
副肿瘤性神经系统综合征(PNS)是一种相当罕见的神经系统并发症,在乳腺癌患者中的发生率不到1%。这些患者中近三分之一检测不到肿瘤相关神经抗体(ONA),并且在不到30%的病例中,伴随癌症治疗可使神经功能缺损得到改善。
一名42岁的绝经前女性出现左侧中枢性面瘫,伴有左侧舌偏斜、向上垂直性眼球震颤、中度痉挛性截瘫、左脚肌张力障碍姿势、下肢反射亢进和双侧巴宾斯基征阳性。排除所有其他潜在的神经病因后,怀疑为PNS,但未发现ONA。PET-CT扫描显示右乳代谢增加,同侧胸肌间淋巴结肿大。芯针活检证实存在浸润性导管癌。乳腺手术后,神经症状消失。一周后,患者因双侧可疲劳性眼睑下垂再次入院,两周后,眼睑下垂有明显改善,同时下半身痉挛性截瘫迅速进展。她开始接受辅助化疗,临床和神经症状均有明显改善,放疗结束时,无神经功能损害迹象。
本病例研究强调了即使未检测到ONA,对于PNS的检测仍需高度警惕的重要性,因为快速识别并治疗潜在肿瘤为完全康复提供了最佳机会。