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Impending Respiratory Failure in Miller Fisher Syndrome: A Report of a Unique Case
Sondos Badran1, Johnny S Randhawa1, Renard Jerome1
1Internal Medicine, Arrowhead Regional Medical Center, Colton, USA.
Abstract:
Miller Fisher syndrome (MFS) is a variant of Guillain-Barré syndrome (GBS), where the body's immune system erroneously attacks its own nerves. It typically presents with a triad of symptoms: ataxia, ophthalmoplegia, and areflexia. These symptoms often develop rapidly, usually within a few days after a viral or bacterial infection, most commonly following respiratory or gastrointestinal illnesses. The diagnosis of MFS involves clinical examination, electromyography (EMG), nerve conduction studies, and sometimes lumbar puncture to analyze cerebrospinal fluid (CSF). Treatment primarily focuses on supportive care and symptomatic management, with more severe cases requiring intravenous immunoglobulin (IVIG) or plasma exchange (PLEX). Most patients experience spontaneous recovery over several weeks to months, although some may require rehabilitative therapy to regain full function. We present a case of a 47-year-old female with no known past medical history who presented to the emergency department with complaints of left-sided facial droop, dysphagia, pressure-like chest pain, and progressively worsening bilateral upper and lower extremity weakness. Computed tomography (CT) of the head without intravenous contrast was unremarkable for any intracranial abnormalities. Lumbar puncture was performed and was grossly unremarkable for albuminocytologic dissociation; however, CSF serology was significant for positive anti-GQ1 antibody. On the first day of hospitalization, the patient was noted to have increased work of breathing and was subsequently intubated for acute hypercapnic respiratory failure. The patient received therapy with IVIG and PLEX and eventually had a tracheostomy and a percutaneous endoscopic gastrostomy (PEG) tube was placed. The patient was then discharged to a long-term acute care facility (LTAC) with outpatient neurology follow-up. This case aims to emphasize the importance of physical exams and clinical intuition when guiding diagnostics and interventions for complex medical conditions with atypical presentation, such as our case of MFS.
Insights
Miller Fisher syndrome (MFS), a Guillain-Barré syndrome variant, presents with ataxia, ophthalmoplegia, and areflexia. Early diagnosis and treatment with IVIG or PLEX are crucial for recovery.
Area of Science:
- Neurology
- Immunology
Background:
- Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS).
- MFS involves autoimmune attack on peripheral nerves, typically presenting with ataxia, ophthalmoplegia, and areflexia.
Observation:
- A 47-year-old female presented with atypical MFS symptoms including facial droop, dysphagia, chest pain, and progressive weakness.
- Diagnostic workup revealed positive anti-GQ1 antibodies in CSF, despite unremarkable initial lumbar puncture findings.
- The patient experienced acute respiratory failure requiring intubation and mechanical ventilation.
Findings:
- The patient received intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) for MFS treatment.
- Tracheostomy and percutaneous endoscopic gastrostomy (PEG) tube placement were necessary due to disease severity.
- The case highlights the importance of clinical examination and diagnostic intuition in managing atypical MFS presentations.
Implications:
- This case underscores the need for prompt recognition and management of MFS, even with non-classical symptoms.
- Effective treatment strategies, including IVIG and PLEX, can improve outcomes for patients with MFS.
- Further research into MFS pathophysiology and treatment optimization is warranted.
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