How often and when Fisher syndrome is overlapped by Guillain-Barre syndrome or Bickerstaff brainstem encephalitis?

作者：Sekiguchi Y; Mori M^*; Misawa S; Sawai S; Yuki N; Beppu M; Kuwabara S

来源：European Journal of Neurology, 2016, 23(6): 1058-1063.

DOI：10.1111/ene.12983

• Bickerstaff brainstem encephalitis
• Fisher syndrome
• Guillain-Barre syndrome
• pharyngeal-cervical-brachial variant of Guillain-Barre syndrome

Background and purpose: Fisher syndrome (FS) may overlap with Guillain-Barre syndrome (GBS), in particular the pharyngeal-cervical-brachial variant form (PCB-GBS), or Bickerstaff brainstem encephalitis (BBE). Our aim was to elucidate the frequency of this overlap and the patterns of clinical progression in patients with FS.
Methods: Sixty consecutive patients with FS were studied. FS/PCB-GBS was diagnosed when the patients developed pharyngeal, cervical and/or brachial weakness. Patients with flaccid tetraparesis were diagnosed as having FS/conventional GBS. FS/BBE was defined as the development of consciousness disturbances.
Results: All 60 patients initially developed the FS clinical triad alone (pure FS). Of these, 30 (50%) patients had pure FS throughout their course, whereas the remaining 50% of patients showed an overlap: PCB-GBS in 14 (23%) patients, conventional GBS in nine (15%) patients and BBE in seven (12%) patients. The median (range) durations from FS onset to progression to FS/PCB-GBS, FS/GBS or FS/BBE were 5 (1-7), 3 (1-4) and 3 (1-5) days, respectively. Patients with overlap syndromes more frequently received immune-modulating treatment, and the outcomes were generally favourable. The frequencies of positivity for anti-GQ1b, GT1a, GD1a, GD1b, GalNAc-GD1a and GM1 antibodies were not significantly different amongst the four groups.
Conclusions: Of the patients with pure FS, 50% later developed an overlap with PCB-GBS, conventional GBS or BBE. The overlap occurred within 7 days of FS onset; thus, physicians should pay attention to the possible development of this overlap during the first week after FS onset.

• 出版日期2016-6