Isolated abducens nerve palsy as a regional variant of Guillain–Barré syndrome

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Abstract

The authors reviewed clinical profiles and laboratory findings for 100 cases of abducens nerve paresis without impairment of the other cranial nerves, limb weakness, and ataxia throughout the clinical course. Review of the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti-ganglioside antibody testing. Information was obtained from each primary physician on symptoms of preceding infection; initial symptoms; neurological signs during the illness; the clinical course; treatment provided; and outcome. Isolated abducens nerve paresis was present in 100 patients and bilateral paresis in 29. Tentative diagnoses made by the primary physicians on request of anti-ganglioside antibody testing were abducens nerve palsy (n = 68), Fisher syndrome (n = 14), acute ophthalmoparesis without ataxia (n = 14). Symptoms of infection anteceded in 63. Tendon reflexes were absent or decreased in 27. Distal paresthesias were experienced by seven. Serum anti-GQ1b antibody was positive in 25. These findings suggest that some cases of isolated abducens nerve palsy can be categorized as a regional variant of Guillain–Barré syndrome or mild form of Fisher syndrome.

Introduction

Most cases of isolated abducens nerve palsy are attributable to vascular disorders, trauma, or tumors, as well as to palsy after infection [1]. A case of “sixth nerve paresis with paresthesias” was described as a regional Guillain–Barré syndrome (GBS) variant, as were cases of pharyngeal–cervical–brachial weakness; paraparesis; bifacial weakness with paresthesias; and lumbar polyradiculopathy [2], [3]. The patient in the original case described by Ropper had sinusitis and showed unilateral sixth nerve paresis, minimal facial weakness, mild proximal limb weakness, areflexia in the legs, and paresthesias in the toes and feet, whereas the other patients showed bilateral weakness [2]. Cardinal clinical features of the former were an antecedent illness and areflexia as well as sixth nerve paresis and acral paresthesias. We therefore searched for patients who showed abducens nerve paresis without impairment of the other cranial nerves and had neither limb weakness nor ataxia throughout the clinical course. Whether some cases of isolated abducens nerve paresis should be categorized as a regional variant of GBS is discussed.

Section snippets

Patients and clinical data

One of the authors (M.T.) reviewed the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti–ganglioside antibody testing from hospitals throughout Japan between May 1994 and January 2004. The patients’ clinical features were reviewed by application form as well as medical records at both admission and discharge obtained from each primary physician. When they did not contain adequate information, questionnaires were sent to the physicians by fax.

Specificity of anti-GQ1b IgG antibody test

Of 1549 consecutive patients, anti-GQ1b IgG was positive in 205: Fisher syndrome (FS, n = 133), acute ophthalmoparesis without ataxia (n = 18), ataxic GBS (n = 12), Bickerstaff’s brainstem encephalitis (n = 11), GBS (n = 10), GBS with ophthalmoplegia (n = 8), pharyngeal–cervical–brachial weakness (n = 8), overlapping FS and GBS (n = 4), and isolated abducens nerve palsy (n = 2) [5], [6], [7]. Because autoantibody was negative in all patients with amyotrophic lateral sclerosis (n = 33), multiple sclerosis (n = 3),

Discussion

Anti-GQ1b IgG antibody identifies a cluster of closely related syndromes that have in common the presence of external ophthalmoplegia or cerebellar-like ataxia [7]. This was confirmed in our consecutive samples. The autoantibody is not a simple serological marker, rather it is considered an effector molecule that produces the syndromes subsequent to infection by microorganisms carrying a GQ1b epitope [7]. We found one case of “sixth nerve paresis with paresthesias” associated with an antecedent

Acknowledgments

We thank Ms. Y. Tamura for her technical assistance in the anti-ganglioside antibody tests. Supported in part by a grant-in-aid for Human Frontier Science Program (RGP0038/2003-C to N.Y.); a grant for Scientific Research (B) (KAKENHI 1724360 to N.Y.) from the Ministry of Education, Culture, Sports, Science and Technology of Japan; a Research Grant for Neuroimmunological Diseases to N.Y. from the Ministry of Health, Labour and Welfare of Japan; a Health Sciences Research Grant (Research on

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