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PP-048 Pharyngeal-cervical-brachial variant of Guillain Barré syndrome with anti ganglioside antibodies
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  1. Merjen Orazova1,
  2. Vugar Abbasaliyev2,
  3. Elif Ceren Duru1,
  4. Hülya Maraş Genç2,
  5. Edibe Pembegül Yıldız2
  1. 1Istanbul University Istanbul Faculty Of Medicine Department Of Pediatrics
  2. 2Istanbul University Istanbul Faculty Of Medicine Department Of Pediatric Neurology

Abstract

Aim Guillain-Barré syndrome (GBS) is an acute-onset ascending sensorimotor neuropathy often provoked by a preceding infection. Atypical variants of GBS include; pure sensory variant, pure motor variant, bilateral facial palsy with paraesthesias, pharyngeal-cervical-brachial (PCB) variant, and the Miller Fisher syndrome (MFS). We describe a child with PCB predominant weakness overlapping with classical GBS syndrome.

Material and Method .

Results A 9-year-old boy was referred for acute onset dysphagia, speech, gait difficulty and weakness of limbs of 4 days. He had a history of acute gastroenteritis recently. He had bilateral peripheral facial palsy more prominent on the left side, dysphonia. Shoulder abduction was 2/5, elbow flexion and extension 4/5, distal upper extremity and lower extremity muscle powers were 5/5. Biceps and brachioradialis deep tendon reflexes were hypoactive, patella and Achille reflexes were absent. He had an ataxic gait. Other neurologic and systemic examination of the patient was normal. Brain MRI was normal, spinal MRI revealed enhancement of the root of the spinal nerve and the cauda equine (figure 1). Intravenous immunoglobulin was given at a dose of 2 gr/kg. During follow up, lower extremity weakness developed, and he could not walk without assistance. The EMG revealed demyelination, slow motor and sensory nerve conduction. He was diagnosed with GBS. Anti-Ganglioside antibody panel was tested from the serum and anti-GQ1b and anti-GD1b were found to be positive. His bulbar symptoms were resolved and he was discharged two weeks after the initial presentation. He can walk without support.

Abstract PP-048 Figure 1

Sagittal T1 Spinal MRI. Spinal MRI revealed enhancement of the root of the spinal nerve and the cauda equine (figure 1).

Conclusions Atypical variants of GBS can be seen with regional involvement. Anti-ganglioside antibodies are usually found in patients with cranial nerve involvement (2). Our case shares features of PCB variant of GBS and classical GBS with tetraparesis. Early recognition of atypical variants of GBS is important for early treatment.

  • Guillain Barré Syndrome
  • Pharyngeal cervical brachial variant
  • Neuropathy
  • Ataxia
  • Facial Palsy

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