Paraneoplastic neurological syndromes presenting with paraneoplastic ptosis in an infant with acute lymphoblastic leukemia: A case report

Eri Ohashi, Itaru Hayakawa, Yuichi Abe
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Abstract

Background

Paraneoplastic neurological syndromes (PNS) are rare in children. Unlike adults, children present with a variety of atypical neurological symptoms that are difficult to diagnose. Consequently, PNS remains an underrecognized disorder. Nevertheless, appropriate immunomodulatory therapy is crucial for neurological prognosis and should not be overlooked. We report a case in which intravenous immunoglobulin therapy effectively treated PNS while treating leukemia.

Case report

A 1.5-year-old girl with B-cell precursor acute lymphoblastic leukemia was referred to our neurology department with ptosis that developed 6 weeks after leukemia treatment and worsened over 8 days. Neurological evaluation revealed normal pupils, no ocular paralysis, no proximal muscle weakness, normal tendon reflexes, and no autonomic neuropathy. Cerebrospinal fluid and brain magnetic resonance imaging findings were normal. Antibodies against the acetylcholine receptor and P/Q-type voltage-gated calcium channels were negative. Low- and high-frequency repetitive median nerve stimulation tests revealed normal findings. We suspected PNS at the neuromuscular junction due to the persistent ptosis that occurred during leukemia treatment. Intravenous immunoglobulin therapy was effective, and ptosis disappeared after 2 weeks. The patient received standard chemotherapy for leukemia, and the ptosis did not relapse for 1 year.

Conclusion

Persistent ptosis in cancer patients requires appropriate evaluation and extensive differentiation for myasthenic syndrome. Timely treatment with immunomodulatory therapy improves neurological prognosis when PNS is suspected.

患有急性淋巴细胞白血病的婴儿出现副肿瘤性上睑下垂的副肿瘤性神经综合征:病例报告
背景副肿瘤性神经综合征(PNS)在儿童中非常罕见。与成人不同,儿童会出现各种非典型神经症状,难以诊断。因此,PNS 仍是一种未得到充分认识的疾病。然而,适当的免疫调节治疗对神经系统的预后至关重要,不容忽视。我们报告了一例在治疗白血病的同时通过静脉注射免疫球蛋白有效治疗 PNS 的病例。病例报告一名患有 B 细胞前体急性淋巴细胞白血病的 1.5 岁女孩因眼睑下垂转诊至我院神经内科,她在白血病治疗 6 周后出现眼睑下垂,并在 8 天后病情恶化。神经系统评估显示瞳孔正常,无眼球麻痹,无近端肌无力,腱反射正常,无自主神经病变。脑脊液和脑磁共振成像结果正常。乙酰胆碱受体和P/Q型电压门控钙通道抗体呈阴性。低频和高频重复性正中神经刺激测试结果显示正常。由于白血病治疗期间出现持续性上睑下垂,我们怀疑是神经肌肉接头处的 PNS。静脉注射免疫球蛋白治疗有效,眼睑下垂在两周后消失。患者接受了标准的白血病化疗,眼睑下垂在 1 年内没有复发。结论癌症患者出现持续性上睑下垂需要进行适当的评估,并与肌无力综合征进行广泛鉴别。如果怀疑存在肌无力综合征,及时进行免疫调节治疗可改善神经系统的预后。
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