Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum - Clinical analysis of 581 cases

被引:205
作者
Ito, Masafumi [1 ]
Kuwabara, Satoshi [2 ]
Odaka, Masaaki [1 ]
Misawa, Sonoko [2 ]
Koga, Michiaki [1 ]
Hirata, Koichi [1 ]
Yuki, Nobuhiro [1 ]
机构
[1] Dokkyo Med Univ, Dept Neurol, Shimotsuga, Tochigi 3210293, Japan
[2] Chiba Univ, Sch Med, Dept Neurol, Chuo Ku, Chiba 2608670, Japan
关键词
anti-GQ1b antibody; Bickerstaff's brainstem encephalitis; Campylobacter jejuni; Fisher syndrome; Guillain-Barre syndrome;
D O I
10.1007/s00415-008-0775-0
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Whether Bickerstaff's brainstem encephalitis (BBE) is a distinct disease or a subtype of Fisher syndrome (FS) is unclear as there have been no clinical studies with sufficiently large numbers of patients with FS or BBE. Our aim was to clarify the nosological relationship. Medical records of patients suffering acute ophthalmoplegia and ataxia within four weeks of onset were reviewed. BBE was the diagnosis for patients with impaired consciousness, FS for those with clear consciousness and areflexia. Clinical features, neuroimages, and laboratory findings were analyzed. Patients were grouped as having BBE (n = 53), FS (n = 466), or as unclassified (n = 62). The BBE and FS groups had similar features; positive serum anti-GQ1b IgG antibody (68% versus 83%), antecedent Campylobacter jejuni infection (23% versus 21%), CSF albuminocytological dissociation (46% versus 76%), brain MRI abnormality (11% versus 2%), and abnormal EEG findings (57% versus 25%). BBE (n = 4) and FS (n = 28) subgroups underwent detailed electrophysiological testing. Both groups frequently showed absent soleus H-reflexes, but normal sensory nerve conduction (75% versus 74%) and a 1-Hz power spectrum peak on postural body sway analysis (67% versus 72%). Common autoantibodies, antecedent infections, and MRI and neurophysiological results found in this large study offer conclusive evidence that Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum with variable CNS and PNS involvement.
引用
收藏
页码:674 / 682
页数:9
相关论文
共 39 条
[1]   BRAIN-STEM ENCEPHALITIS AND THE SYNDROME OF FISHER,MILLER - A CLINICAL-STUDY [J].
ALDIN, AN ;
ANDERSON, M ;
BICKERSTAFF, ER ;
HARVEY, I .
BRAIN, 1982, 105 (SEP) :481-495
[2]   ATAXIA AND AREFLEXIA IN SOAA [J].
ALDIN, ASN ;
TRONTELJ, JV ;
ANDERSON, M ;
EEGOLOFSSON, O .
ACTA NEUROLOGICA SCANDINAVICA, 1993, 88 (05) :363-369
[3]  
ALDIN ASN, 1994, ACTA NEUROL SCAND, V89, P87
[4]   ASSESSMENT OF CURRENT DIAGNOSTIC-CRITERIA FOR GUILLAIN-BARRE-SYNDROME [J].
ASBURY, AK ;
CORNBLATH, DR .
ANNALS OF NEUROLOGY, 1990, 27 :S21-S24
[5]   BODY SWAY AND VIBRATION PERCEPTION THRESHOLDS IN NORMAL AGING AND IN PATIENTS WITH POLYNEUROPATHY [J].
BERGIN, PS ;
BRONSTEIN, AM ;
MURRAY, NMF ;
SANCOVIC, S ;
ZEPPENFELD, K .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1995, 58 (03) :335-340
[6]   MESENCEPHALITIS AND RHOMBENCEPHALITIS [J].
BICKERSTAFF, ER ;
CLOAKE, PCP .
BMJ-BRITISH MEDICAL JOURNAL, 1951, 2 (4723) :77-81
[7]   BRAIN-STEM ENCEPHALITIS - FURTHER OBSERVATIONS ON A GRAVE SYNDROME WITH BENIGN PROGNOSIS [J].
BICKERSTAFF, ER .
BRITISH MEDICAL JOURNAL, 1957, 1 (JUN15) :1384-1387
[8]   SERUM ANTI-GQ(1B) IGG ANTIBODY IS ASSOCIATED WITH OPHTHALMOPLEGIA IN MILLER FISHER SYNDROME AND GUILLAIN-BARRE-SYNDROME - CLINICAL AND IMMUNOHISTOCHEMICAL STUDIES [J].
CHIBA, A ;
KUSUNOKI, S ;
OBATA, H ;
MACHINAMI, R ;
KANAZAWA, I .
NEUROLOGY, 1993, 43 (10) :1911-1917
[9]   SERUM IGG ANTIBODY TO GANGLIOSIDE GQ1B IS A POSSIBLE MARKER OF MILLER FISHER SYNDROME [J].
CHIBA, A ;
KUSUNOKI, S ;
SHIMIZU, T ;
KANAZAWA, I .
ANNALS OF NEUROLOGY, 1992, 31 (06) :677-679
[10]   The Miller Fisher syndrome: neurophysiological and MRI evidence of both peripheral and central origin in one case [J].
Echaniz-Laguna, A ;
Battaglia, F ;
Heymann, R ;
Tranchant, C ;
Warter, JM .
JOURNAL OF NEUROLOGY, 2000, 247 (12) :980-982