Ramakrishna Karan N, Tambe Vikrant, Kattamanchi Adithya, Dhamoon Amit S
Department of Medicine, State University of New York (SUNY) Upstate Medical University, 750 East Adams Street, Room 5138, Syracuse, NY, 13210, USA.
J Med Case Rep. 2020 Feb 18;14(1):31. doi: 10.1186/s13256-020-2357-4.
Miller Fisher syndrome is a variant of acute inflammatory demyelinating polyneuropathy classically characterized by ataxia, ophthalmoplegia, and areflexia. Miller Fisher syndrome can present with uncommon symptoms such as bulbar, facial, and somatic muscle palsies and micturition disturbance.
We describe the case of a 76-year-old white man with new-onset ataxia, stridor, areflexia, and upper and lower extremity weakness who required intubation at presentation. An initial work-up including imaging studies and serum tests was inconclusive. Eventually, neurophysiological testing and cerebrospinal fluid analysis suggested a diagnosis of Miller Fisher syndrome. Our patient responded to treatment with intravenous immunoglobulin and supportive therapy.
The occurrence of acute or subacute descending paralysis with involvement of bulbar muscles and respiratory failure can often divert clinicians to a diagnosis of neuromuscular junction disorders (such as botulism or myasthenia gravis), vascular causes like stroke, or electrolyte and metabolic abnormalities. Early identification of Miller Fisher syndrome with appropriate testing is essential to prompt treatment and prevention of further, potentially fatal, deterioration.
米勒-费雪综合征是急性炎性脱髓鞘性多发性神经病的一种变异型,典型表现为共济失调、眼肌麻痹和腱反射消失。米勒-费雪综合征可出现如延髓、面部和躯体肌肉麻痹以及排尿障碍等不常见症状。
我们描述了一名76岁白人男性的病例,该患者新发共济失调、喘鸣、腱反射消失以及上下肢无力,就诊时需要插管。包括影像学检查和血清检测在内的初步检查结果不明确。最终,神经生理学检测和脑脊液分析提示诊断为米勒-费雪综合征。我们的患者对静脉注射免疫球蛋白治疗和支持性治疗有反应。
急性或亚急性下行性麻痹伴延髓肌肉受累和呼吸衰竭的情况常使临床医生转向诊断神经肌肉接头疾病(如肉毒中毒或重症肌无力)、血管性病因(如中风)或电解质和代谢异常。通过适当检测早期识别米勒-费雪综合征对于及时治疗和预防进一步的、可能致命的病情恶化至关重要。