Department of Neurology, University of Virginia Health System, Charlottesville, VA 22908-0394, USA.
Dev Med Child Neurol. 2012 Sep;54(9):863-6. doi: 10.1111/j.1469-8749.2012.04221.x. Epub 2012 Feb 9.
Paraneoplastic limbic encephalitis is a rare disorder characterized by personality changes, seizures, memory loss, and psychiatric symptoms often associated with antineuronal antibodies. It is well described in the adult literature but is still underreported in the pediatric literature. Symptoms are usually multifocal and subacute in presentation, occurring over days to weeks; however, in rare cases, symptom onset can be more gradual. We report the case of a 9-year-old male with anti-Hu-associated paraneoplastic limbic encephalitis that presented as episodic ataxia and behavioral changes evolving to intractable epilepsy and worsening behavioral changes over the course of a year. This case highlights the importance of considering a paraneoplastic disorder in the differential diagnosis for unexplained multifocal neurological symptoms of subacute or chronic onset as earlier detection and treatment may result in an improved outcome.
副肿瘤性边缘性脑炎是一种罕见的疾病，其特征是人格改变、癫痫发作、记忆力减退和精神症状，通常与神经元抗体有关。成人文献中对此有详细描述，但在儿科文献中报道较少。症状通常表现为多灶性和亚急性，持续数天至数周；然而，在少数情况下，症状发作可能更为缓慢。我们报告了一例 9 岁男性抗 Hu 相关副肿瘤性边缘性脑炎的病例，其表现为间歇性共济失调和行为改变，随后发展为难治性癫痫，并在一年的病程中出现行为恶化。这个病例强调了在鉴别诊断不明原因的多灶性亚急性或慢性起病的神经症状时，考虑副肿瘤性疾病的重要性，因为早期发现和治疗可能会改善预后。
