PP-042 Guillain-Barré syndrome with polyneuritis cranialis: a case report

Aim Guillain-Barré syndrome is a general term used to describe acute immune-mediated polyradiculoneuropathies. It's one of the most common causes of acute, acquired muscle weakness. Polyneuritis cranialis is believed to be an interface between Guillain-Barré and Miller-Fischer syndromes. Material and Method . Results Eight-year-old male patient with autism spectrum disorder presented with drooling, difficulty swallowing, slurred speech, inability to move facial muscles, drooping eyelids and unsteady gait. There was no history of fever, respiratory or gastrointestinal infection, travel, trauma, or toxic exposure. Except for consanguineous marriage between parents, the past medical and family history was unremarkable. Physical examination revealed normal vital signs, mid-dilated pupils bilaterally reactive to light, multiple cranial nerve involvement including cranial nerves II, IV, VI, VII, IX, X, XI, XII, mild upper and lower extremity weakness and ataxia. Atypical variant of Guillain-Barré syndrome was considered as a preliminary diagnosis, but differential diagnoses included brainstem lesions, myasthenic crisis, botulismus and toxic/metabolic diseases. Complete blood count, biochemistry, metabolic screenings were normal. Cranial MRI was normal, contrast weighted spinal MRI revealed enhancement of conus medullaris and cauda equina roots(figure 1). Test dose with pyridostigmine showed no improvement of symptoms excluding a myasthenic crisis. An electromyography(EMG) was performed subsequently, needle EMG revealed rare positive spikes and fibrillation in the left frontal muscle. Cerebrospinal fluid(CSF) was acellular and CSF protein was elevated (0.83 g/L, N: 0,15–0,45). All assessments indicated Guillain-Barré syndrome with multicranial neuropathy. Serum anti-ganglioside antibody test was positive for anti-GT1a, anti-GT1b, and anti-GQ1b. The patient was treated with 2 g/kg intravenous immunoglobulin. Although minor improvements were seen, because of severe bulbar involvement, 5 total plasma exchanges were done. Three months after the first presentation, the symptoms were resolved. Conclusions The symptoms and severity of Guillain-Barré syndrome and it's variants show diversity. It's crucial to consider all forms when clinical suspicion exists, to avoid underdiagnosis and delayed treatment.