An immunologic abnormality common to Bickerstaff's brain stem encephalitis and Fisher's syndrome

doi:10.1016/0022-510X(93)90250-3

Journal of the Neurological Sciences

Volume 118, Issue 1, August 1993, Pages 83-87

https://doi.org/10.1016/0022-510X(93)90250-3 Get rights and content

Abstract

The nosological position of Bickerstaff's brain stem encephalitis (BBE) has yet to be established, and its etiology is not clear. Because anti-G_Q1b antibody frequently occurs in patients with Fisher's syndrome (FS) and there are clinical similarities between FS and BBE, we investigated anti-ganglioside antibodies in sera from 3 BBE patients who had transient long tract signs in addition to acute ophthalmoplegia and cerebellar-like ataxia in order to clarify the etiology and nosological position of BBE. High IgG anti-G_Q1b antibody titers were present in all 3 sera samples but decreased with the clinical course of the illness. In contrast, no anti-G_Q1b antibody was found in sera from patients with other neurologic diseases which were able to produce transient brain stem disturbance: multiple sclerosis, neuro-Behçet's disease, brain stem infarction, herpes simplex virus encephalitis, and Wernicke's encephalopathy. The finding that BBE and FS shared common autoantibody suggests that autoimmune mechanism common to FS is likely in BBE, and that both conditions represent a distinct disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia.

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Although rare, patients presenting with optic neuropathy associated with these diseases have been reported in the literature [4–7]. Reports of BBE patients presenting with optic neuropathy are summarized in Table 1 [5,6]. Visual acuity can be as severely affected as hand motion as in our case.
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The anti-GQ1b autoantibody, which is also related to Fisher syndrome, may be detected in the serum, but the pathogenesis remains unclear [73]. The standard treatment for BBE includes IVMP, IVIG, and apheresis [74]. The ASFA guidelines did not indicate a category for apheresis in the treatment of patients with BBE.
In recent years, immunoadsorption has been increasingly recognized as an alternative to therapeutic plasma exchange and used for the treatment of neurological disorders such as Guillain–Barré syndrome, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, neuromyelitis optica spectrum disorders, and multiple sclerosis, as well as autoimmune encephalitis. Unlike therapeutic plasma exchange, which requires fluid replacement with a blood solution such as fresh frozen plasma or albumin, immunoadsorption is a blood purification technique that enables the selective removal of humoral factors from separated plasma through a high-affinity adsorbent with tryptophan or phenylalanine. Although the mechanisms underlying the therapeutic effects of immunoadsorption treatment remain to be fully elucidated, they are based on the removal of pathogenic humoral factors from circulating blood, such as disease-specific autoantibodies, complement, and inflammatory cytokines. The American Society for Apheresis has published evidence-based guidelines on the use of therapeutic apheresis in clinical practice, with specific instructions on 16 neurological disorders. However, the modality recommended in the guidelines for most of these disorders is therapeutic plasma exchange. This part of our review focuses on the clinical aspects of immunoadsorption. We also describe the efficacy of immunoadsorption and the evidence obtained by previous studies of the treatment of neurological disorders. Immunoadsorption could greatly improve the treatment of patients with autoimmune neurological disorders but further evidence is needed to confirm the efficacy of immunoadsorption in clinical practice.
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Research paperAn immunologic abnormality common to Bickerstaff's brain stem encephalitis and Fisher's syndrome

Abstract

J. Neurol. Sci.

Assessment of current diagnostic criteria for Guillain-Barré syndrome

Ann. Neurol.

Brainstem encephalitis and the syndrome of Miller Fisher: a clinical study

Brain

The nosological position of the ophthalmoplegia, ataxia and areflexia syndrome: “the spectrum hypothesis”

Acta. Neurol. Scand.

Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis

Br. Med. J.

Brain stem encephalitis (Bickerstaff's encephalitis)

Mesencephalitis and rhombencephalitis

Br. Med. J.

Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome

Ann. Neurol.

Serum anti-GQ1b antibody is associated with impaired eyr movement in Miller Fisher syndrome (abstract)

Ann. Neurol.

An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia)

N. Engl. J. Med.

Syndrome of Fisher: ophthalmoplegia, ataxia and areflexia

Am. J. Ophthalmol.

Serum antibodies to gangliosides in Guillain-Barré syndrome

Ann. Neurol.

Research paper
An immunologic abnormality common to Bickerstaff's brain stem encephalitis and Fisher's syndrome

Serum IgG antibody to ganglioside G_Q1b is a possible marker of Miller Fisher syndrome

Serum anti-G_Q1b antibody is associated with impaired eyr movement in Miller Fisher syndrome (abstract)