Search

Background and aims: Bulbar palsy plus(BPp) is a variant of Guillain Barre syndrome (GBS), characterized by bulbar symptoms and others cranial nerves deficits or ataxia, without limbs or neck weakness, usually associated with presence of GQ1b and Gt1a antibodies. Methods: We describe a case of BPp overlapped by a sensory-motor axonal neuropathy (ASMAN). Results: A 62 years old man, without relevant medical history, was admitted to Emergency Room for dysphagia, voice reduction and paraesthesia to distal extremities started 4 days earlier, after a diarrhoeic episode. Neurological examination showed absent tendon reflexes, mild ophthalmoparesis and asymmetrical palpebral ptosis. Cerebrospinal fluid(CSF) analysis and neurophysiological study(NPS) were normal, showing only absent H reflex. Repetitive nerve stimulation at low and high frequencies was normal. Intravenous immunoglobulins 0,4 gr/kg die for 5 days were started. Subsequently he developed tetra-hyposthenia and worsening of bulbar function, complete gaze palsy and bilateral ptosis, requiring mechanical ventilation and nasogastric tube. 15 days after symptoms onset, CSF analysis showed albuminocytological dissociation and NPS showed diffuse sensory-motor axonal neuropathy. Initially no serum anti ganglioside were detected, but 15 days after GT1a, Gq1b, GD2 and GD3 IgG were positive. RT-PCR and serology for SARS-COV 2 were negative. Campylobacter search was negative. About 15 days after onset he started recovery and at 50 days he was discharged from rehabilitative ward. Conclusions: BPp is recently recognised as a rare GBS variant, such as Miller Fisher syndrome (MFS) or pharyngeal-cervical-brachial (PCB) variant. Overlap between AMSAN and the other variants was already described. This is an interesting case of BPp/ASMAN overlap syndrome.

Recently, during the pandemic infection of the novel SARS-CoV-2, some cases of Guillan-Barré Syndrome (GBS) have been reported. The aim of this work is to report the natural history of patients with GBS, both COVID and not-COVID related, hospitalized in Liguria region, during lock down period, in order to assess clinical features of both groups and possible managements pitfalls due to pandemic emergency. Fifteen GBS patients were admitted to the Hospitals of Liguria, from February 15th to May 3rd 2020, six with SARS-CoV-2 infection and nine without infection. In COVID-19 related GBS five patients presented with classical GBS and one with variant. Two patients presented neurologic symptoms during or shortly after the viral syndrome, suggesting the pattern of a para-infectious profile. Multi-organ involvement, delay in the diagnosis, incomplete work up and start of therapy, were registered in 50% of cases with a GBS-Disability scale ≥4 at follow-up evaluation. In not-COVID-19 related GBS, main problem was diagnostic delay. In three patients the first neurological observation took place after a mean of 33,6 days. Moreover, five patients went to emergency room after an average of 30 days since the onset of neurological symptoms because of fear of contagion. In conclusion, not only SARS-CoV-2 infection can cause GBS, but it can also, due to effects of pandemic on the health organization, affect the outcome of patients with not COVID-19 related GBS., Highlights • Covid-19 and GBS. • Diagnostic and therapeutic delay. • Management pitfalls.