An unusual case of bulbar palsy plus/ASMAN overlap

ABSTRACT

Background and aims: Bulbar palsy plus(BPp) is a variant of Guillain Barrè 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.