18º Congresso Brasileiro de Neurologia Infantil

Dados do Trabalho



Apresentação do caso

A 6-year-old female patient had fever, headache and odynophagia for 12 days, with intermittent periumbilical pain afterwards. She, then, presented pain on the right eye, associated with ocular discharge, edema and eyelid ptosis. She progressed over the days with dysarthria, labial rhyme deviation to the left, in addition to progressive limb paresis. Initially, he presented progression of paresis, with difficulty in overcoming gravity in the upper and lower limbs bilaterally, progressing with ataxic gait and dysphagia. Considering the clinical picture, Miller-Fisher Syndrome was suspected, which was corroborated with magnetic resonance imaging of the thoracic and lumbar spine, demonstrating thickening and post-contrast enhancement of the roots of the cauda equina, notably the dorsal roots, in addition to electroneuromyography with a demyelinating pattern and cerebrospinal fluid analysis with protein-cytological dissociation. In addition, a search for anti-GQ1b antibodies was performed, which confirmed the diagnostic hypothesis. Thus, targeted therapy with intravenous immunoglobulin was started with a significant response in the neurological condition, progressively recovering strength in the limbs, improvement in gait and dysphagia.


Miller Fisher syndrome is a rare disease, a benign variant of Guillain-Barré Syndrome and is characterized by the clinical triad of ataxia, cranial nerve involvement and areflexia. Associated with previous infections or immunizations. The most related etiological agents are Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus and Streptococcus pyogenes. Infection with Campylobacter jejuni, which has lipopolysaccharide similar to GQ1b, can trigger the production of anti-GQ1b IgG antibody. The cerebrospinal fluid findings and electrophysiological features are similar to those of acute inflammatory demyelinating polyneuropathy. Immunotherapy with intravenous immunoglobulin or plasmapheresis is indicated for patients four weeks after the onset of symptoms and still without clinical improvement.

Comentários finais

The description of this clinical case aims to help the recognition of the classic clinical manifestations of this neurological syndrome and guide the performance of laboratory and imaging tests, allowing the early initiation of treatment, in order to promote quality of life and avoid possible complications.

Referências (se houver)

Palavras Chave

Miller Fisher syndrome; Guillain-Barré Syndrome; Demyelinating;

Fonte de Fomento (se houver)

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Não apresentam conflitos de interesse


Neuroimunologia, esclerose múltipla e outras doenças desmielinizantes


Ariany Cibelle Costa Rezende, Stella Mandu Cicco , Hugo Matos Bandeira, Nathalia Dantas de Freitas Rêgo , Bianca Carolina Serafim do Nascimento