Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination

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Abstract

Fisher syndrome (FS), a variant of Guillain–Barré syndrome (GBS), is a rare disorder, and there are few reported studies of a large number of patients with FS. Cerebrospinal fluid (CSF) albuminocytological dissociation was found in 59% of 123 FS patients during the first 3 weeks of illness, while serum anti-GQ1b IgG antibody was positive in 85%. Whereas the incidence of CSF albuminocytological dissociation increased from the first to second weeks in FS, anti-GQ1b IgG antibody peaked in the first week, but there was no CSF albuminocytological dissociation. Statistically, anti-GQ1b antibody testing was superior to a CSF examination in supporting a diagnosis of FS during the first 3 weeks of illness, especially in the first week.

Introduction

The presence of cerebrospinal fluid (CSF) albuminocytological dissociation is evidence of Guillain–Barré syndrome (GBS), but “After the first week of symptoms, CSF protein concentration is elevated or has been shown to rise on serial lumbar punctures”, as stated in the criteria established for GBS (Asbury and Cornblath, 1990). Fisher syndrome (FS) is considered a variant of GBS, strong evidence being that both conditions share a common CSF feature, albuminocytological dissociation (Fisher, 1956). FS, however, is a rare disorder, the estimated annual incidence rate being 0.09 per 100,000 population (Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology, 1998). Few studies have been made of large numbers of patients with FS. Clinical features of 50 consecutive patients with FS recently were reported, but no CSF findings were given (Mori et al., 2001). We therefore investigated the frequency of CSF albuminocytological dissociation and its temporal profile in a large number of FS patients.

Chiba et al. (1992) first reported that all six patients with FS that they studied had anti-GQ1b IgG antibody during the acute phase, which strong association was confirmed by other studies Yuki et al., 1993, Willison et al., 1993. Most of the FS patients tested had this autoantibody. Equally significant was the absence of anti-GQ1b IgG antibody in the normal and other-disease control groups, indicative of the antibody's high specificity for FS. Our previous studies showed diagnostic specificities of anti-GQ1b IgG antibody in FS patients and also anti-GM1 and anti-GD1a IgG antibodies in GBS patients Yuki et al., 1993, Tagawa et al., 2002. Because antibody titers peak at the time of clinical presentation then decay rapidly during clinical recovery, our main aim was to clarify whether anti-GQ1b antibody testing is more useful than a CSF examination for supporting the diagnosis of FS during the early phase of illness. In contrast, the IgG class of autoantibody to GM1 or GD1a specifically is present during the acute phase in patients with axonal GBS Ho et al., 1999, Kornberg et al., 1994, Ogawara et al., 2000, Tagawa et al., 2002. Moreover, anti-GQ1b IgG antibody has been detected in GBS with ophthalmoplegia (Chiba et al., 1993). We therefore also investigated whether combined anti-GM1, anti-GD1a and anti-GQ1b antibody testing would be helpful for supporting a diagnosis of GBS.

Section snippets

Patients

Serum anti-ganglioside antibody testing was done between September 1995 and December 2000. Serum samples from 289 FS and 785 GBS patients were referred to us from university and district general hospitals throughout Japan. Because tentative diagnoses were made by the primary physicians, one of us (Y.N.) reviewed the medical records of each patient and made a definite diagnosis based on our proposed criteria for FS (Odaka et al., 2001) and the criteria for GBS (Asbury and Cornblath, 1990). The

Results

A statistical difference between FS and GBS was found for age (Mann–Whitney U-test, p=0.003) but not for sex or days when blood and CSF were taken (Table 1).

Discussion

In the first samples assayed at Massachusetts General Hospital (MGH), all taken during the first 3 weeks of GBS, 73% of 111 patients had raised CSF protein concentrations (Ropper et al., 1991). The incidence of raised protein found for the initial CSF sample increased from 66% in the first week to 82% in the second. The frequencies in our series showed a lower tendency, possibly because the former series was prospective and ours retrospective. Similar findings were obtained for our 123 patients

Acknowledgments

The authors thank Ms. Y. Tsuchiya for assistance in the serological assay. This research was supported by grant-in-aid for Scientific Research (B) (KAKENHI 14370210 to N.Y.) from the Ministry of Education, Culture, Sports, Science and Technology of Japan.

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