Miller-Fisher Syndrome After Oxford/AstraZeneca COVID-19 Vaccine

Abstract and Introduction

Abstract

Introduction: Miller-Fisher Syndrome (MFS) is a variant of Guillain–Barré syndrome (GBS), an acute immune-mediated neuropathy, which manifests as a rapidly evolving areflex motor paralysis. This syndrome presents as a classic triad: ophthalmoplegia, areflexia, and ataxia. MFS is usually benign and self-limited.

Case Report: A Caucasian patient was admitted to our hospital with the flu, loss of bilateral strength in the lower limbs and upper limbs and sudden-onset ataxia 7 days after receiving a first dose of the Oxford/AstraZeneca COVID-19 vaccine. On neurological examination, the patient had Glasgow Coma Scale score of 15, with absence of meningeal signs; negative Babinski sign; grade 2 strength in the lower limbs and grade 4 strength in the upper limbs; axial and appendicular cerebellar ataxia; and peripheral facial diparesis predominantly on the right, without conjugate gaze deviation. Cerebrospinal fluid (CSF) was collected on admission, and analysis revealed albuminocytological dissociation with CSF protein of 148.9 mg/dL; leukocytes, 1; chlorine, 122; glucose, 65 mg/mL; red cells, 2; and non-reactive venereal disease research laboratory test result. The COVID-19 IgG/IgM rapid immunological test was negative. Electroneuromyography revealed a recent moderate-grade and primarily sensory and motor demyelinating polyneuropathy with associated proximal motor block.

Discussion and Conclusion: Miller-Fisher Syndrome may be related to events other than infections prior to neuropathy, as in the case reported here. The patient presented strong correlations with findings for MFS reported in the literature, such as the clinical condition, the results of electroneuromyography, and results of the CSF analysis typical for MFS. When treatment was provided as proposed in the literature, the disease evolved with improvement. Ultimately, the diagnosis of incomplete MFS was made, including acute ataxic neuropathy (without ophthalmoplegia).

Introduction

Guillain–Barré syndrome (GBS) is currently recognized as a group of acute immune-mediated neuropathies and the most common and severe acute paralytic neuropathy worldwide, with about 100,000 people developing the disease each year.^[1,2]

The incidence rates of GBS in Europe and North America show a range of 0.8–1.9 cases per 100,000 people per year. This rate increases with age (0.6 per 100,000 per year in children and 2.7 per 100,000 per year in the elderly aged ≥ 80 years). It is more frequent in men than in women and, in Western countries, adults are affected more frequently than children.^[1,3] The incidence of GBS is higher in winter than in summer in some areas of the world, possibly associated to the prodromal periods of some infectious agents.^[4]

GBS manifests as a rapidly evolving areflexic motor paralysis, with or without sensory changes. The usual pattern is a rapidly evolving ascending paralysis. It may accompany dysesthesia, with tingling in the limbs and areflexia or hyporeflexia. In addition, pain in the neck, shoulders, back, or diffusely in the spine is common in the early stages of GBS, occurring in about 50% of patients. The lower limbs are usually more affected than the upper limbs and facial diparesis is present in 50% of the affected individuals. Inferior cranial nerves can be affected and bulbar weakness may also occur, affecting muscles involved in breathing, swallowing, speech, and tongue movement.^[3,5] Clinical worsening continues for 4 weeks, but thereafter further progression is unlikely. Up to 90% of patients with GBS have maximal weakness during this period.^[3,5]

There is a variety of GBS syndromes that are limited or regional and that have axonal or demyelinating characteristics. An example is Miller–Fisher syndrome (MFS), which presents with the classic triad: ophthalmoplegia, areflexia and ataxia. MFS accounts for about 5% of all cases of GBS, with an incidence of 1–2 cases per 1,000,000 people, and is strongly associated with antibodies against the ganglioside GQ1b. 3.4.^[3,4]

Due to MFS being a variant of GBS, it is of great importance to highlight the numerous case reports and studies that have linked GBS to coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), even though no definitive causal association of GBS with the use of vaccines has been proven.^[6–12]