engleski

ANTI-GQ1B ANTIBODY SYNDROME: A CASE REPORT

Introduction/Objectives: Studies have shown that serum samples from patients with Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS), Bickerstaff's brainstem encephalitis (BBE) and acute ophtalmoparesis often have positive anti-GQ1b antibodies. The aim of this case report was to present challenges in diagnosis and treatment of a young patient with overlapping GBS and MFS syndrome. Participants, Materials/Methods: A 31-year-old man presented in our emergency room due to the sudden onset of binocular diplopia. One week prior to the symptom onset he had diarrhea. Other past medical history didn't reveal any significant diseases. The physical examination was unremarkable, while the neurological examination revealed diplopia with right abducens nerve palsy and right ptosis with preserved pupil function. During first days after the hospitalization, the clinical state continued to worsen with the progression of neurological deficit affecting multiple cranial nerves, the absence of myotatic reflexes, paresthesias in the hands and feet and tetraparesis. Initial negative CT scan was followed by magnetic resonance (MR) of the brain and the magnetic resonance angiography (MRA) of the brain vessels. Neither showed any significant pathological ABSTRACT BOOK 2019 / Mind & Brain – 59th INPC / Posters Neurology 146 findings. The lumbar puncture revealed increased protein level (0, 61 g/L) with normal cell counts (2 cells) and normal CSF glucose levels. Electroneurography performed on day 7 of the illness revealed moderate loss of motoneurons in all examined muscle groups. Neurographic analysis showed low amplitude sensory nerve action potentials (SNAP) in all sensory nerves. Serum tests for antiganglioside antibodies were sent for analysis and came positive for anti- GD1b and anti-GQ1b. Considering the clinical presentation of overlapping Miller Fisher and Guillain-Barré syndrome, treatment with plasma exchange was initiated. Despite the treatment, the clinical state continued to worsen to the state of the respiratory arrest due to which the patient was urgently intubated and mechanically ventilated. In total, five plasma exchanges were given which gradually resulted in the separation of the patient from the mechanical ventilation. However, there was no other clinical improvement and since the slight progress of the motoric deficit in the arms was observed, therapy with intravenous immunoglobulin was indicated in dose 2g/kg over 5 days. Three months after the admission, the patient was discharged from the hospital and referred to rehabilitation. The first follow-up after the discharge from the hospital was two months later. The neurological exam revealed significant improvement with residual gait ataxia, dysarthria and slight weakness of the proximal musculature of both arms. On the second follow-up, four months after the discharge, the patient was independently mobile, without speech disturbances and with the residual mild motoric deficit of the left arm (motor strength 3/5). Conclusions: The optimal management for most severe cases of GBS remains uncertain. The current clinical practice requires further investigations to identify clinical predictors of early deterioration and to define the treatment modalities in patients with anti-GQ1b overlapping syndromes.

Anti-GQ1b

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