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首页> 外文期刊>The Canadian Journal of Neurological Sciences: le Journal Canadien des Sciences Neurologiques >P.029 Facial onset sensorimotor neuronopathy syndrome (FOSMN) associated with Non-Hodgkin Lymphoma (NHL)
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P.029 Facial onset sensorimotor neuronopathy syndrome (FOSMN) associated with Non-Hodgkin Lymphoma (NHL)

机译：P.029面部展开感觉传感器神经病综合征（FOSMN）与非霍奇金淋巴瘤（NHL）相关联

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摘要

Background: FOSMN is a recently describe neurological syndrome, characterizes by slow onset of facial sensory abnormalities and motor deficits. The initial description showed a very uniform clinical presentation. Since the initial description there are clinical cases describe in literature with subtle phenotype variations. Methods: We describe a clinical case associated with NHL. We will report clinical data, laboratory and neurophysiological findings. Results: Patient initiated with left perioral and mental sensory symptoms on her left side. It spread up to include left V2 area and spread to the right side. After 2 years she developed sensory symptoms on her right hand. Progressed to weakness and atrophy on the right upper limb. Also developed dysarthria, dysphonia, dysphagia, as well as photophobia, anisocoria and double vision. Had thorough work-up and everything unrevealing. Except for Spep that showed increased free kappa. Bone marrow biopsy showed evidence of a clonal cell expansion consistent with indolent lymphoma Conclusions: This case provides evidence of FOSMN associated with NHL. To our knowledge this is a first case describe with NHL. There had been reports with motor neuro diseases phenotype with lymphoma that may represent a paraneoplastic disorder. Our patient expands the clinical presentation. This finding should not lessen the diagnosis of FOSMN.
机译：背景：FOSMN是最近描述神经系统综合征，其特征是面部感官异常和电机缺陷的缓慢发作。初始描述显示了非常统一的临床介绍。由于初始描述，有临床病例在文献中描述具有微妙的表型变化。方法：我们描述了与NHL相关的临床病例。我们将报告临床数据，实验室和神经生理学发现。结果：患者在她的左侧患有左侧骨骼和精神感觉症状。它遍布包括左v2区域并蔓延到右侧。 2年后，她在右手上发育了感觉症状。进入右上肢的弱点和萎缩。还开发出扰动，呼吸困难，吞咽困难，以及噬菌体，人物和双重视野。有彻底的工作和一切肆无忌惮。除了SPEP外，显示出免费的kappa。骨髓活检显示克隆细胞扩增的证据表明，与惰性淋巴瘤结论一致：这种情况提供了与NHL相关的FOSMN的证据。据我们所知，这是第一个用NHL描述的案例。已经报道了Motor Neuro疾病表型，淋巴瘤可能代表骨盆疾病。我们的病人扩大了临床介绍。这一发现不应减少FOSMN的诊断。

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