Department of Internal Medicine, Providence-Providence Park Hospital, Southfield, Michigan, USA
Department of Internal Medicine, Providence-Providence Park Hospital, Southfield, Michigan, USA.
BMJ Case Rep. 2020 Dec 29;13(12):e237170. doi: 10.1136/bcr-2020-237170.
Altered mental status can have many causes ranging from emergent intracranial pathologies to more insidious, systemic toxic aetiologies. We report a rare case of dermato-neuro syndrome in a 71-year-old man with a known history of scleromyxoedema. The patient initially presented with encephalopathy which quickly progressed to generalised tonic-clonic seizures and coma. While his presentation fits with other, although rare, cases of dermato-neuro syndrome, it is imperative to rule out lethal, more common causes of altered mentation. Due to the rarity and difficulty in diagnosis of dermato-neuro syndrome, there is a significant debate regarding the optimal management as there are no standardised treatment protocols. In our case, the patient was successfully treated with plasmapheresis resulting in improved neurologic function.
精神状态改变可能有很多原因，从紧急颅内病变到更隐匿的全身性中毒性病因都有可能。我们报告了一例罕见的皮神经综合征病例，患者为 71 岁男性，既往有硬肿性黏液水肿病史。该患者最初表现为脑病，很快进展为全面强直阵挛发作和昏迷。虽然他的表现符合其他罕见的皮神经综合征病例，但必须排除更常见的致命性精神状态改变的原因。由于皮神经综合征的罕见性和诊断难度，关于最佳治疗方法存在很大争议，因为目前尚无标准化的治疗方案。在我们的病例中，患者成功接受了血浆置换治疗，神经功能得到改善。
