Abstract Details

Title Clinical and Electrodiagnostic Presentation of Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome

Topic Peripheral Nerve

Presentation(s) P07 - (-)

Poster/Presentation
Number 060

Objective

Background BACKGROUND: Pharyngeal-cervical-brachial (PBC) variant of Guillain-Barre presents with acute oropharyngeal, neck, and upper extremity weakness with preserved lower extremity strength, reflexes and no sensory distrubances.

Design/Methods DESIGN/METHODS: 71 year old man presented 5 months after initial symptoms with history of sudden onset of mild dysphagia to solids and liquids which became severe over a few hour and dysarthria. 24 hour later developed proximal upper extremity weakness, and 36 hours later lower extremity weakness. Absence of sensory symptoms, autonomic dysfunction or pain. He had received a flu shot 3 weeks prior to the onset of symptoms. Patient required intubation, PEG tube. Was treated with 7 plasma exchange followed by IVIG, and intensive rehabilitation. Electrodiagnostic studies showed evidence of a diffuse polyradiculoneuropathy affecting predominantly the upper limbs, and cranial innervated muscles. A follow up visit at 8 month revealed improvement of arm strengh with no dysarthria, minimal facial weakness.

Results RESULTS: The pharyngo-cervical-brachial variant is a rare presentation characterized by oropharyngeal, neck and proximal upper extremity weakness, with sparing of the lower extremities. Sensory symptoms and autonomic dysfunction are absent. This patient findings on nerve conduction studies were not consistent with a demyelinating pattern, and showed axonal loss and polyradicular involvement. It is unclear of which is the primary mechanism of nerve damage in these patients, demyelination or axonal degeneration. In cases of this variant of Guillain-Barre there have been electrophysiological findings of motor axonal loss, similar to that found in AMAN.

Conclusions CONCLUSIONS: This variant of Guilan-Barre should be considered in patients with acute progressive bulbar and upper extremity weakness. Recognition of the atypical forms of Guillain-Barre syndrome, including the PCB variant should have a strong clinical impact and should raise awareness to help with early diagnosis and treatment.

Authors/Disclosures
Alexandra M. Soriano Caminero, MD PRESENTER	No disclosure on file
Aiesha Ahmed, MD, MBA, FAAN (Corewell Health)	Dr. Ahmed has stock in Doximity.
Daniel Pelletier, MD (Keck School of Medicine of USC)	Dr. Pelletier has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Pelletier has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi Genzyme. Dr. Pelletier has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. Pelletier has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Polpharma Biologics.

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