By Lucy Piper, Senior medwireNews Reporter
Researchers have identified a new variant form of Guillain-Barré syndrome (GBS) that is characterised by prominent acute bulbar palsy without neck or limb weakness.
The fact that acute bulbar palsy was the most prominent clinical symptom across the disease course in the 11 patients studied differentiates this clinical syndrome from the Miller Fisher syndrome (MFS) and pharyngeal-cervical-brachial (PCB) variants of GBS, says the team.
Indeed, according to a recently proposed classification system, “none of the cases satisfied the criteria for the MFS or PCB variant”, they add.
The patients were identified from 184 enrolled in the Korean Inflammatory Neuropathy Consortium over a 2-year period. All of the patients showed the general features of GBS, including acute monophasic event and recovery, less than 4 weeks of clinical nadir and no accompanying upper motor neuron signs.
The most common initial symptom was slurred speech, followed by gait disturbance, diplopia, dizziness and tingling limbs.
All the patients had accompanying neurological symptoms. Ten patients had below normal or absent reflexes, while ophthalmoplegia and gait ataxia were each seen in nine patients, sensory abnormality in eight and unilateral facial palsy or diplegia in six patients.
Researcher Jong Seok Bae (Hallym University, Seoul, Korea) and colleagues propose that GBS presenting acute bulbar palsy without limb weakness could be classified into “acute bulbar palsy plus” if patients present with accompanying neurological symptoms – as in the case of the 11 study participants – and “isolated acute bulbar palsy” in the absence of such symptoms.
They add that all 11 patients had accompanying ophthalmoplegia, sensory ataxia or both, which are components of the MFS and PCB variants, and so acute bulbar palsy plus without limb weakness could be considered as a transitional spectrum between the two syndromes, appearing to be closer to PCB than MFS.
The study participants also shared positivity for immunoglobulin (Ig)G anti-GT1a antiganglioside antibodies, which have been associated with other kinds of GBS with bulbar palsy including the PCB variant, with all 11 patients testing positive, while just six tested positive for IgG anti-GQ1b antibodies.
“Therefore, IgG anti-GT1a antibodies appear to be involved with the pathogenesis of this syndrome and so can be used as a marker of this variant”, the team writes in Neurology.
Nine patients were treated with a 5-day course of intravenous Ig infusions and the response was good with symptoms resolving within 3 months. Only one patient had symptoms that persisted for more than 6 months.
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