Saluja Alvee, Verma Jyoti, Anees Shahbaz, Nangia Anita, Dhamija Rajinder K
Department of Neurology, Lady Hardinge Medical College, New Delhi, India.
Department of Pathology, Lady Hardinge Medical College, New Delhi, India.
J Neurosci Rural Pract. 2022 Oct-Dec;13(4):768-770. doi: 10.25259/JNRP-2022-3-49. Epub 2022 Nov 18.
Morvan's syndrome is a rare anti-contactin-associated protein-like 2 (CASPR2) antibody-mediated autoimmune disorder. The clinical features of this syndrome include muscular twitching, insomnia, dysautonomia, peripheral nerve hyperexcitability, and fluctuating delirium. An underlying tumor is commonly found among Morvan's syndrome cases, with thymoma being the most frequent association. We describe an unusual case of a 39-year-old female with excruciating bilateral leg pain, insomnia, hyperhidrosis, peripheral nerve hyperexcitability, serum anti-CASPR2 antibody positivity, and a solid pseudopapillary tumor of the pancreas on histopathology. Furthermore, the patient's symptoms improved after receiving intravenous immunoglobulin (0.4 g/kg per day for 5 days). To the best of our knowledge, this is the first case of Morvan syndrome associated with a solid pseudopapillary pancreatic tumor to be reported in the literature to date. Our case adds to the spectrum of malignancies that are associated with Morvan's syndrome. The recognition of this rare syndrome and its various associations are important for the neurologist, as it is a potentially treatable condition.
莫旺综合征是一种罕见的抗接触蛋白相关蛋白样2（CASPR2）抗体介导的自身免疫性疾病。该综合征的临床特征包括肌肉抽搐、失眠、自主神经功能障碍、周围神经兴奋性增高和波动性谵妄。莫旺综合征病例中常发现潜在肿瘤，其中胸腺瘤最为常见。我们描述了一例不寻常的病例，一名39岁女性，患有双侧腿部剧痛、失眠、多汗、周围神经兴奋性增高、血清抗CASPR2抗体阳性，组织病理学检查发现胰腺实性假乳头状肿瘤。此外，患者在接受静脉注射免疫球蛋白（每天0.4 g/kg，共5天）后症状有所改善。据我们所知，这是迄今为止文献中报道的首例与胰腺实性假乳头状肿瘤相关的莫旺综合征病例。我们的病例增加了与莫旺综合征相关的恶性肿瘤谱。认识这种罕见综合征及其各种关联对神经科医生很重要，因为它是一种潜在可治疗的疾病。
