Lim Shen-Yang, Mason Warren P, Young Nathan P, Chen Robert, Bower James H, McKeon Andrew, Pittock Sean J, Lang Anthony E
University of Malaya, Kuala Lumpur, Malaysia.
Arch Neurol. 2009 Oct;66(10):1285-7. doi: 10.1001/archneurol.2009.203.
To describe and provide audiovisual documentation of a syndrome of polymyoclonus, laryngospasm, and cerebellar ataxia associated with adenocarcinoma and multiple neural cation channel autoantibodies.
Case report with video.
University hospitals. Patient A 69-year-old woman presented with subacute onset of whole-body tremulousness and laryngospasm attributed to gastroesophageal reflux.
Further evaluation revealed polymyoclonus, cerebellar ataxia, and laryngospasm suspicious of an underlying malignant neoplasm. Surface electromyography of multiple limb muscles confirmed the presence of polymyoclonus. The patient was seropositive for P/Q-type voltage-gated calcium channel antibody; subsequently, whole-body fluorine 18 fluorodeoxyglucose positron emission tomography and cervical lymph node biopsy revealed widespread metastatic adenocarcinoma. Follow-up serologic evaluation revealed calcium channel antibodies (P/Q type and N type) and potassium channel antibody.
We highlight the importance of recognizing polymyoclonus. To our knowledge, this is also the first description of a syndrome of polymyoclonus, laryngospasm, and ataxia associated with adenocarcinoma and these cation channel antibodies.
描述并提供与腺癌及多种神经阳离子通道自身抗体相关的多肌阵挛、喉痉挛和小脑共济失调综合征的视听记录。
带视频的病例报告。
大学医院。患者为一名69岁女性,因胃食管反流出现全身震颤和喉痉挛的亚急性发作。
进一步评估发现多肌阵挛、小脑共济失调和喉痉挛,怀疑存在潜在恶性肿瘤。多肢体肌肉的表面肌电图证实存在多肌阵挛。患者P/Q型电压门控钙通道抗体血清学呈阳性;随后,全身氟18氟脱氧葡萄糖正电子发射断层扫描和颈部淋巴结活检显示广泛转移性腺癌。随访血清学评估发现钙通道抗体(P/Q型和N型)及钾通道抗体。
我们强调认识多肌阵挛的重要性。据我们所知,这也是与腺癌及这些阳离子通道抗体相关的多肌阵挛、喉痉挛和共济失调综合征的首次描述。