AOCCN2017

Presentation information

Poster Presentation

[P1-1~141] Poster Presentation 1

Thu. May 11, 2017 9:30 AM - 4:00 PM Poster Room A (1F Navis A.B.C)

[P1-132] A Case of Miller Fisher Syndrome associated with consciousness alteration as seen in Bickerstaff brainstem encephalitis.

Tetsuharu KAMIOKA (Division of Neurology, National Center for Child Health and Development, Japan)

Miller Fisher Syndrome (MFS) shows various symptoms and occasionally overlaps with the symptoms of Bickerstaff brainstem encephalitis. We here report the detailed clinical course of a case with MFS presented with consciousness alteration and discuss the nosological position of the syndrome.
An 11-years-old boy was attacked by headache and nausea during swimming. Numbness of his face and diplopia developed two days later. He was admitted to our hospital because of suspicion of brain infarction. The severe headache and nausea persisted. Consciousness was alert. Dysesthesia in the trigeminal nerve regions was noticed. Deep tendon reflexes were normal but soon? became negative in lower limbs. Because abducens paralysis, trunckal ataxia developed on the 4th disease day, he was diagnosed as MFS. The oculomotor nerve paralysis appeared later. Consciousness alteration and disorientation developed from the 10th day. On 13th day, urinary retention, and hydronephrosis ware was observed. On 20th day, lucid, but a slowly responsive, anarthria, and the slow speech remained. Brain MRI revealed no pathological changes. The intravenous gamma-globulin (IVIG) therapy wasn’t not effective, but methyl prednisolone pulse therapy from the the 11th day successfully improved various symptoms.
Serologic test for glycolipid showed that anti-GQ1b antibody (IgG and IgM) was negative, and anti-GM1, GM2, GT1b and Gal-C (all IgG) was positive with phosphatidic acid?.
Kuwabara (2013) demonstrated Bickerstaff brainstem encephalitis can be regarded as a variant of MFSFMS with central nervous system involvement. Our patient’s clinical course was compatible with a variant of MFS. Because MFS symptoms are diverse, it is necessary to be careful about the changes of systemic nervous system findings.