2026, Number 2
Miller-Fisher syndrome in a patient with a history of Guillain-Barré after 40 years: a manifestation of the autoimmune neuropathy spectrum
Full text
10.35366/122616
Language: English/Spanish [Versión en español]
References: 4
Page: 133-134
PDF size: 173.25 Kb.
ABSTRACT
Introduction: Miller-Fisher syndrome is a variant of Guillain-Barré syndrome, distinguished by the clinical triad of ataxia, ophthalmoplegia, and areflexia. It can manifest several years after an initial episode of Guillain-Barré. Case report: a 54-year-old male with a history of Guillain-Barré syndrome at age 17 presented with ataxia, distal weakness, and ophthalmoplegia. Conclusion: Miller-Fisher syndrome should be included in the differential diagnosis of neurological emergencies to enable early diagnosis and optimize clinical outcomes.ABBREVIATIONS:
- EVC = stroke (cerebrovascular disease)
- IVIG = human intravenous immunoglobulin
- SGB = Guillain-Barré syndrome
- SMF = Miller-Fisher syndrome
INTRODUCTION
Guillain-Barré syndrome (GBS) encompasses a group of acute immune-mediated polyneuropathies.1 A rare variant, Miller-Fisher syndrome (MFS), is distinguished by the classic triad of ataxia, arreflexia, and ophthalmoparesis, primarily affecting the third, fourth, and sixth cranial nerves.2
CASE PRESENTATION
A 54-year-old male patient presented to the emergency department reporting a history of a previous diagnosis of GBS at age 17, an episode that required mechanical ventilatory support. Upon admission, he presented with symptoms of ataxia, distal weakness, left ophthalmoplegia, and bilateral ptosis. The physical examination revealed upper extremity weakness, arreflexia, and a bilateral positive Babinski sign. The patient reported having had three diarrheal evacuations in the four days prior to admission.
The Stroke protocol was activated, showing no evidence of acute ischemia or hemorrhage on the brain CT scan. Subsequently, neurophysiological evoked potential studies were performed, suggesting an impairment in internuclear conduction along the left v-vii pathway.
Treatment was initiated with human intravenous immunoglobulin (IVIG), administering 90 grams as initial management. Following this intervention, the patient showed a decrease in neurological symptoms. After monitoring, a favorable clinical course was observed, without additional complications. The patient was discharged from the hospital to continue outpatient follow-up.
DISCUSSION
Although GBS and MFS are variants of immune-mediated neuropathy, this case highlights how patients with a history of GBS can present other neurological disorders within the immunological spectrum, even decades after the initial episode.3 Facing any focal neurological deficit, the Stroke protocol must be activated.3,4
Initially, a stroke (CVD) was ruled out via CT scan, and the possibility of GBS recurrence was considered. Although it is uncommon for GBS to recur (2-3% of cases), it is possible, especially following gastroenteritis caused by Campylobacter jejuni. The patient had diarrhea four days before admission and a history of GBS requiring mechanical ventilation at age 17. Given the risk of rapid progression to respiratory failure, it is necessary to rule out this possibility.3
The probable diagnosis is MFS, due to the presence of the clinical triad that appears in 80% of patients, the history of GBS, and the gastrointestinal symptoms presented in the preceding days. The diagnosis is clinical; electrophysiological findings include reduced nerve action potentials and diminished Hoffmann reflexes. Unlike GBS, typical demyelinating alterations are not observed. The confirmatory diagnosis for this pathology is the detection of anti-GQ1b antibodies, which has a specificity of 100%. However, these studies were not performed in this case due to the high sensitivity of the clinical diagnosis. Furthermore, it was considered that performing them would not modify the established therapeutic approach.2
CONCLUSIONS
When facing focal neurological deficits, it is crucial to rule out emergency pathologies. A thorough neurological examination is essential to identify the origin of the symptoms. MFS, although rare, must be considered in the differential diagnosis, since early recognition can optimize management and improve patient prognosis.
REFERENCES
Powers WJ, Rabinstein AA, Ackerson T, Adeoye OM, Bambakidis NC, Becker K et al. Guidelines for the early management of patients with acute ischemic stroke: 2019 update to the 2018 guidelines for the early management of acute ischemic stroke: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2019; 50 (12): e344-e418. doi: 10.1161/STR.0000000000000211.
AFFILIATIONS
1 Médico interno de pregrado, Hospital Angeles México (HAM). Universidad Anáhuac México. Ciudad de México.
2 Especialista en Medicina Interna, Unidad de Terapia Intermedia, HAM. Laboratorio de Investigación Integral Cardiometabólica, Instituto Politécnico Nacional. Ciudad de México.
3 Médico interno de pregrado, Hospital Torre Médica Riobamba. Universidad Justo Sierra. Ciudad de México.
ORCID:
4 0009-0005-7875-3066
5 0000-0003-0294-7639
6 0000-0003-2863-8412
If you wish to consult the supplementary data for this article, please contact editorial.actamedica@saludangeles.mx
CORRESPONDENCE
Victoria Sosa Romo. Correo electrónico: victoria.sosaro7@gmail.comReceived: 2024-11-11. Accepted: 2025-02-10.