Case Report: Acute Bulbar Palsy Plus Syndrome: A Guillain-Barré Syndrome Variant More Prone to Be a Subtype Than Overlap of Distinct Subtypes

Abstract

Objective: Acute bulbar palsy plus (ABPp) syndrome is a rare regional variant of Guillain-Barré syndrome (GBS) characterized by acute bulbar palsy combined with other cranial symptoms or ataxia without limb and neck weakness. We aim to investigate characteristics of ABPp syndrome and analyze its nosological position within the GBS spectrum.Methods: A patient with ABPp syndrome was reported, and previous case reports of patients who met the criteria for ABPp syndrome from the literature were reviewed.Results: A total of 28 patients were included in our study. Median age was 32 years. Most of the patients (78.6%) were from Asia, and 75.0% had preceding infection. The main accompanying symptoms were ophthalmoplegia (85.7%), facial palsy (60.7%), and ataxia (50.0%). There existed asymmetric weakness in the form of unilateral facial palsy (32.1%) and ptosis (3.6%). Approximately half of the patients had albuminocytological dissociation. All the tested patients were seropositive for antiganglioside antibodies, of which the two most common were immunoglobulin G (IgG) anti-GT1a (77.3%) and anti-GQ1b (59.1%) antibodies. Over one-third of the patients who underwent electrophysiological assessment showed subclinical neuropathy beyond cranial nerves. The outcome was generally favorable as 89.3% of patients made full recovery within 5 months.Conclusion: The hitherto largest case series of ABPp syndrome advances our understanding of this disease. Serologically, the presence of IgG anti-GT1a and anti-GQ1b antibodies predicts and contributes to the disease. Phenotypically, ABPp syndrome is more prone to be a separate subtype of GBS than overlap of distinct subtypes and has the potential to complement current diagnostic framework of GBS.

Acute Bulbar Palsy-Plus Variant of Guillain-Barré Syndrome in a 3-Year-Old Girl

“…The patient's clinical presentation was similar to those reported in other cases of ABPp syndrome in the literature, which most commonly but not always presented with ophthalmoplegia, bilateral facial palsy, and ataxia. 6 , 10 – 12 Although our patient's extraocular muscles appeared intact on physical examination, her oculomotor nerves, along with multiple other cranial nerves, were T2 hyperintense on MRI brain. The ABPp variant has been most commonly associated with IgG antibodies to GT1a and sometimes GQ1b, though these antibodies can also be absent, as in our case.…”

“…The ABPp variant has been most commonly associated with IgG antibodies to GT1a and sometimes GQ1b, though these antibodies can also be absent, as in our case. 6 , 10 Other conditions such as acute myelopathy, myasthenia gravis, and botulism were less likely due to the presence of ataxia, areflexia and elevated CSF proteins in this patient. 13 …”

Guillain–Barré syndrome with unilateral peripheral facial paralysis in a Chinese child

“…However, neither the CSF examination nor the cranial, cervical spine and lumbar spine MRI results supported the first two diagnosis, and the symptoms outside the facial nerve such as limb weakness and numbness precluded the diagnosis of idiopathic facial palsy. Facial palsy was one of the main accompanying symptoms of acute bulbar palsy plus syndrome, a rare variant of GBS, with a presence rate of about 60% (Cao et al, 2020). Among them, asymmetric weakness could exist in the form of unilateral facial paralysis (32.1%) (Cao et al, 2020).…”

“…Facial palsy was one of the main accompanying symptoms of acute bulbar palsy plus syndrome, a rare variant of GBS, with a presence rate of about 60% (Cao et al, 2020). Among them, asymmetric weakness could exist in the form of unilateral facial paralysis (32.1%) (Cao et al, 2020). However, our case here did not fit the clinical profile of acute bulbar palsy plus syndrome obviously, which characterised by acute bulbar palsy combined with other cranial symptoms or ataxia without limb and neck weakness (Cao et al, 2020).…”

“…However, with the identification of several new phenotypes in recent decades such as pharyngeal‐cervical‐brachial variant, Miller Fisher syndrome and Bickerstaff brainstem encephalitis, the conceptual framework of GBS has become increasingly complex (Wakerley et al, 2014). Clinically evident cranial nerve palsies were commonly seen in GBS variants such as Miller Fisher syndrome (Zuccoli et al, 2011) and acute bulbar palsy plus syndrome (Cao et al, 2020). Among them, facial nerve was most commonly affected and was usually bilateral (Smith et al, 2010; Verma et al, 2012).…”