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Título

OVERLAP OF MILLER FISHER SYNDROME (MFS) WITH BICKERSTAFF SYNDROME (BS)

RESUMO

CASE REPORT. A 15-year-old male patient presented with subacute weakness of the lower limbs associated with incoordination within 1 week, with a 48-hour progression to impaired gait, axial instability and diplopia. He denied fever and diarrhea but reported having a flu-like syndrome three weeks before the current condition. On examination: hypotonia and exalted osteotendinous reflexes with bilateral Babinski, and bilateral ophthalmoparesis was noted. On the day after admission, the patient developed ebrious dysarthria, dysphagia, ineffective cough and decreased level of consciousness, and orotracheal intubation was chosen. After 1 week of sedation withdrawal, he presented some improvement in the sensorium, proximal grade 0 and distal limbs grade IV strength, sustained bilateral ophthalmoparesis, facial diparesis, absent cough reflex, associated with dysautonomia with pressure variability and of heart rate.Tomography (CT) and Magnetic Resonance of the skull performed: no alterations; cerebrospinal fluid (CSF) with protein of 23mg/dL, glucose: 118mg/dL; leukocytes: 3, Oligoclonal bands absent. Electroencephalogram without changes. Laboratory tests/cultures/serologies without alterations. Chest CT: normal. Based on the above, the suspicion of SMF superimposed with SB was raised. He received treatment with plasmapheresis in 4 sessions on alternate days, being interrupted after motor worsening in the left side and evidence of intraparenchymal hematoma with hemoventricle and midline deviation on a new cranial CT, evolving with complications and death.
DISCUSSION: MFS is a variant of Guillain-Barre syndrome characterized by a subacute, monophasic onset and the classic triad of ophthalmoplegia, ataxia and areflexia. If there is progressive impairment of the level of consciousness and/or associated hyperreflexia, we recognize brainstem encephalitis, or BS. Sharing similarities in the autoimmune mechanism, both syndromes are associated with anti-GQ1b antibodies, which are highly specific for diagnosis. One of the main limitations of this case was the unavailability of dosing this antibody. Both intravenous immunoglobulin and plasmapheresis have been used in the treatment.
COMMMENTS: The overlap of SMF with SB is rare. It is important to remember the need for attention in clinical practice for possible diagnose because the prognosis is generally good and most patients recover well when the syndrome is quickly recognized and treated.

Palavras Chave

Anti-GQ1b antibody, Bickerstaff brainstem encephalitis, Fisher syndrome, Guillain–Barré syndrome