[P1-130] Anti GQ1b antibody syndrome in Korean children
Objectives: Anti-GQ1b antibodies have been found in patients with Miller-Fisher syndrome and related disorders. Our aim is to describe clinical features of anti GQ1b antibody syndrome in Korean children.
Method: We retrospectively identified 9 patients (5.4-18 years) with the elevated anti GQ1b antibody level from November 2008 to October 2016 in Pusan National University Hospital. Clinical features, laboratory findings neuroimaging, and outcomes were reviewed.
Results: Eight patient (72.7%) had preceding infectious illness, Diverse symptoms were reported; diplopia (n=6), dysesthesia (n=5), gait ataxia (n=5), weakness of extremities (n=3), blepharoptosis (n=2), dysphagia (n=1), and dysarthria (n=1). Neurologic signs are external ophthalmoplegia (n=6), areflexia (n=6), sensory ataxia (n=5), internal ophthalmoplegia (n=3), limb weakness (n=3), limb sensory change (n=3), blepharoptosis (n=1), and bulbar palsy (n=1). Diagnosis of patients were acute ophthalmoparesis (n=4), classical Miller-Fisher syndrome (n=2), Miller-Fisher syndrome/ Guillain-Barre syndrome (n=1), acute ataxic neuropathy (n=1), and pharyngeal-cervical-brachial weakness (n=1). In seven patients with cerebrospinal fluid analysis, seven patients (62.6%) had pleocytosis (>5 cells/mm3) and none of them showed a positive oligoclonal band result. Cross-reactivity with other antiganglioside antibodies were observed in 3 patients (27.3%) with the positivity of anti-GM1 antibody. Neuroimaging revealed demyelinating cerebral lesions (n=1) and increased enhancement of abducens nerves (n=1). Five patients (45.5%) revered fully and six patients (54.5%) has partially recovered and been following up.
Conclusion: The distribution of ganglioside and the presence of specific antiganglioside antibodies are associated with diverse neurological manifestations. Tests for antiganglioside antibodies, including anti-GQ1b antibodies are useful to confirm the diagnosis and pathogenesis.
Method: We retrospectively identified 9 patients (5.4-18 years) with the elevated anti GQ1b antibody level from November 2008 to October 2016 in Pusan National University Hospital. Clinical features, laboratory findings neuroimaging, and outcomes were reviewed.
Results: Eight patient (72.7%) had preceding infectious illness, Diverse symptoms were reported; diplopia (n=6), dysesthesia (n=5), gait ataxia (n=5), weakness of extremities (n=3), blepharoptosis (n=2), dysphagia (n=1), and dysarthria (n=1). Neurologic signs are external ophthalmoplegia (n=6), areflexia (n=6), sensory ataxia (n=5), internal ophthalmoplegia (n=3), limb weakness (n=3), limb sensory change (n=3), blepharoptosis (n=1), and bulbar palsy (n=1). Diagnosis of patients were acute ophthalmoparesis (n=4), classical Miller-Fisher syndrome (n=2), Miller-Fisher syndrome/ Guillain-Barre syndrome (n=1), acute ataxic neuropathy (n=1), and pharyngeal-cervical-brachial weakness (n=1). In seven patients with cerebrospinal fluid analysis, seven patients (62.6%) had pleocytosis (>5 cells/mm3) and none of them showed a positive oligoclonal band result. Cross-reactivity with other antiganglioside antibodies were observed in 3 patients (27.3%) with the positivity of anti-GM1 antibody. Neuroimaging revealed demyelinating cerebral lesions (n=1) and increased enhancement of abducens nerves (n=1). Five patients (45.5%) revered fully and six patients (54.5%) has partially recovered and been following up.
Conclusion: The distribution of ganglioside and the presence of specific antiganglioside antibodies are associated with diverse neurological manifestations. Tests for antiganglioside antibodies, including anti-GQ1b antibodies are useful to confirm the diagnosis and pathogenesis.