CLINICAL CASE OF PARANEOPLASTIC NEUROLOGICAL SYNDROME IN A CHILD WITH NEUROBLASTOMA

T. Kadricheva, S. O. Falaleeva, I. Demyanova, M.V. Borisova, E. V. Shishkina, A.V. Krasitskiy, V.E. Kazakova, L.S. Karpova, E.O. Yashina, E.A. Lyamina
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Abstract

Paraneoplastic syndromes are of particular difficulties in establishing a timely diagnosis of a malignant tumor esp. in cases when the decease development is occurred chronologically much earlier than the appearance of its specific symptoms and the neoplasm growth’s first signs. And the clinical manifestation can be represented solely by an isolated clinic of paraneoplastic syndrome, which in its turn is much alike the symptoms of the paraneoplastic neurological syndrome (PNS). Authors represent a clinical case of a severe PNS coupled with the central nervous system (CNS) lesion, mainly the cerebellum, which was manifested clinically by ataxia, tremor, myoclonus, dysartria, cognitive and mental impairment and psychotic disorders, which in its turn had delayed the timely diagnosis of neuroblastoma (left-sided intermixed adrenal ganglioneuroblastoma with metastatic tumors in ipsilateral lymph nodes and paranephric fat, deletion 1q, deletion 1p36 and MYCN amplification - all negative and INSS stage 2B) in a 5 y/o male patient. No ‘classical’ opsoclonus-myoclonus was a matter of diagnosis due to the opsoclonus absence during the whole disease course. This clinical case observation publication is having a practical purpose of pinning attention to paraneoplastic syndromes among practitioners of wide spectrum: neurologists, infectionists and pediatricians.
神经母细胞瘤患儿副肿瘤性神经综合征临床病例
副肿瘤综合征在及时诊断恶性肿瘤方面具有特殊的困难,尤其是当疾病的发展在时间上早于其特殊症状的出现和肿瘤生长的最初征兆时。临床表现可能仅表现为副肿瘤综合征的孤立临床表现,而副肿瘤综合征又与副肿瘤性神经综合征(PNS)的症状十分相似。作者的一个临床病例显示,严重的副肿瘤性神经综合征伴有中枢神经系统(CNS)病变,主要是小脑病变,临床表现为共济失调、震颤、肌阵挛、构音障碍、认知和智力障碍以及精神障碍、该病例为一名5岁男性患者,其神经母细胞瘤(左侧混合性肾上腺神经节神经母细胞瘤,同侧淋巴结和肾旁脂肪有转移性肿瘤,1q缺失、1p36缺失和MYCN扩增--均为阴性,INSS 2B期)的及时诊断被延误。由于患者在整个病程中均未出现 "经典 "肌阵挛,因此诊断并不涉及肌阵挛。发表这篇临床病例观察报告的实际目的是引起神经科医生、感染科医生和儿科医生等各领域从业人员对副肿瘤综合征的关注。
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