A Case of Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome Responsive to IVIG

Objective: Raise awareness about pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. Background: The pharyngeal-cervical-brachial variant of Guillain-Barre syndrome belongs to the clinical spectrum of the anti-GQ1b syndrome which also includes Miller-Fisher syndrome, acute ophthalmoparesis, and Bickerstaff brainstem encephalitis. This condition may result in severe disability. Several therapeutic approaches have been tried with variable success. We present a case that responded very well to IVIG. Design/Methods: Case report Results: This is a 48-year-old woman who presented with acute vertigo, unsteadiness, diplopia, ascending paresthesias and numbness that started in her hands. She had nasal voice and dysphagia. She had upper respiratory infection one week before. Examination was remarkable for right eye esotropia on primary gaze, right lateral and left lateral gaze palsy. No nystagmus or ptosis was seen. She elevated the palate minimally symmetrically. Power of neck flexion and extension, shoulder adduction, forward flexion, external and internal rotation, elbow flexion and extension was 4/5. The remaining motor strength was 5/5. Reflexes were 0 at biceps, brachioradialis, triceps bilaterally, 1 at patella bilaterally, and 2 at Achilles tendon bilaterally. Sensory exam was intact in the legs but there was light touch and pin prick gradient at the wrist. Gait was cautious and wide-based but Romberg testing was negative. Brain MRI, lumbar puncture and blood tests were unremarkable except for positive IgG anti-GQ1b antibodies: 259 IV (normal 0–50 IV). Anti GM1 and anti-GD1b antibodies were negative. The nerve conduction studies demonstrated non-length dependent sensory neuronopathy. This presentation is suggestive of the pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. She received IVIG 2g/kg with recovery of weakness, voice and swallowing abnormalities within one week. Diplopia although improved, persists. Conclusions: This case of pharyngeal-cervical-brachial variant of Guillain-Barre following respiratory infection with positive anti-GQ1b antibodies responded well to treatment. Early recognition and treatment may improve the outcome of this rare but potentially disabling condition. Disclosure: Dr. Corn has nothing to disclose. Dr. Yeung has nothing to disclose. Dr. Marchidann has nothing to disclose. Dr. Treidler has nothing to disclose.