Detection of Serum Anticerebellar Antibodies in Patients with Miller Fisher Syndrome

Inoue, Akira; Koh, Chang−Sung; Iwahashi, Teruaki

doi:10.1159/000008113

Cited by 14 publications

(6 citation statements)

References 15 publications

(16 reference statements)

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“…A 1‐year study using immunocytochemical staining of human cerebellum described selective staining of the molecular layer with sera from 3 MFS or GBS patients who had elevated IgG anti‐GQ1b antibody levels 80. Western blot analysis also revealed increased levels of anti‐cerebellar antibodies in MFS patients when compared with GBS patients or healthy controls 60. These findings suggest the presence of immunologically mediated cerebellar dysfunction in MFS, but more studies are needed to further define the role of anti‐GQ1b IgG antibody, as well as the underlying immunological mechanism of cerebellar dysfunction in MFS.…”

Section: Clinical Featuresmentioning

confidence: 99%

Clinical and immunological spectrum of the Miller Fisher syndrome

2007

Muscle and Nerve

154

139

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The Miller Fisher syndrome (MFS), characterized by ataxia, areflexia, and ophthalmoplegia, was first recognized as a distinct clinical entity in 1956. MFS is mostly an acute, self-limiting condition, but there is anecdotal evidence of benefit with immunotherapy. Pathological data remain scarce. MFS can be associated with infectious, autoimmune, and neoplastic disorders. Radiological findings have suggested both central and peripheral involvement. The anti-GQ1b IgG antibody titer is most commonly elevated in MFS, but may also be increased in Guillain-Barré syndrome (GBS) and Bickerstaff's brainstem encephalitis (BBE). Molecular mimicry, particularly in relation to antecedent Campylobacter jejuni and Hemophilus influenzae infections, is likely the predominant pathogenic mechanism, but the roles of other biological factors remain to be established. Recent studies have demonstrated the presence of neuromuscular transmission defects in association with anti-GQ1b IgG antibody, both in vitro and in vivo. Collective findings from clinical, radiological, immunological, and electrophysiological techniques have helped to define MFS, GBS, and BBE as major disorders within the proposed spectrum of anti-GQ1b IgG antibody syndrome.

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Section: Clinical Featuresmentioning

confidence: 99%

Clinical and immunological spectrum of the Miller Fisher syndrome

2007

Muscle and Nerve

154

139

View full text Add to dashboard Cite

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“…Muscle spindle bodies [42] and dorsal roots immunostain for anti-GQ1b antibodies (dorsal roots also stain for GD1b and GD3), but the physiological significance of this is unclear [43]. A study looked at immunostained human cerebellum with anti-GQ1b sera from three Miller Fisher patients [44], and western blot analysis has shown an increase in anticerebellar antibodies in Miller Fisher syndrome patients compared to GBS patients and controls [45]. Therefore, although the site of disease process remains unclear, there is evidence to suggest involvement of peripheral nerve, dorsal root ganglion, neuromuscular junction, and even cerebellum.…”

Section: Discussionmentioning

confidence: 99%

Ataxia, Ophthalmoplegia, and Areflexia: What Would You Think?

Karsan

Fletcher

Bódi

et al. 2012

Case Reports in Neurological Medicine

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We present here a case of carcinomatous meningitis presenting as Miller Fisher syndrome (MFS). There are four further cases described in the literature with evidence of tumour invasion within the central nervous system (CNS) shown either in cerebrospinal fluid examination or on histology. There are further five cases described in which an association between cancer and a Miller Fisher phenotype has been shown. Some of these have identified antiganglioside antibodies in the serum and, in one case, also showed antibodies deposited within the primary tumour itself. This raises a question as to whether there is a paraneoplastic form. It would be informative when further cases present in this way to histologically examine for malignant CNS invasion, and the presence of antiganglioside antibodies in both the malignant primary and areas of nervous system thought to be affected by MFS.

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“…Although a brain stem lesion has been suggested, direct pathological evidence of demyelination in the CNS7 8 is lacking. Serum antibody binding to human and mouse cerebellum has been implicated as a cause for ataxia,9 but current immunological evidence has mainly shown binding to cranial nerves involved in eye movements 10. Magnetic resonance imaging studies have shown enhancement in the lumbosacral root, cauda equina, facial nerve, and trigeminal nerves 11-13.…”

Section: Discussionmentioning

confidence: 99%

Transcranial magnetic stimulation studies in the Miller Fisher syndrome: evidence of corticospinal tract abnormality

Ratnagopal²

2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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The Miller Fisher syndrome, clinically defined by the triad of ataxia, areflexia, and ophthalmoplegia, is an uncommon form of acquired inflammatory demyelinating polyneuropathy. 1 An immunological process 2-4 is likely to be involved in its pathogenesis akin to the more common forms of axonal or demyelinating Guillain-Barré syndrome, but its exact site of abnormality remains poorly defined in terms of clinical, radiological, or neurophysiological evidence. In particular, there is minimal evidence to suggest upper motor corticobulbar or corticospinal tract involvement. The disease is thought to be primarily demyelinating in nature, with rapid onset and often spontaneous complete resolution of signs and symptoms. In view of this, its rarity and relatively short disease process, clinical and pathological information are primarily lacking. 5 Transcranial magnetic stimulation (TMS) is a well established method for studying the functional integrity of the corticospinal system electrophysiologically. 6 For the first time we have utilised TMS, a rapid, reproducible, and safe technique, in a serial study of three consecutive patients presenting with classic Miller Fisher syndrome. Central motor conduction times (CMCTs) were studied over a 3 to 6 month period with the aim of demonstrating dynamic corticospinal tract dysfunction in correlation with the resolution of clinical features. The findings are discussed in relation to existing evidence on the pathogenesis of the disease. PATIENT 1 A 39 year old women had no relevant medical history. She developed giddiness, diplopia, and limb numbness of acute onset, worsening over 3 days. A history of upper respiratory infection 1 week before admission was elicited. Physical examination on admission showed diminished eye movements in all directions, mild bilateral ptosis, areflexia, upper limb incoordination, and reduced limb sensation to touch and pain. No detectable motor weakness was present. Brain MRI and CSF examination were unremarkable. An antiGQ1b assay was positive. The patient's eye movements progressed to a near complete ophthalmoparesis over 2 days. Transcranial magnetic stimulation studies were performed on admission. She was discharged 1 week after admission with minimal improvement of eye movements. She continued to improve when reviewed fortnightly. At 1.5 months after admission, she recovered to having near complete eye movements and a repeat TMS study was done. At 7.5 months after admission, there was complete recovery of eye movements and ataxia but reflexes remained slightly diminished. A repeat TMS study was performed then. PATIENT 2 A 55 year old women had no relevant medical history. She experienced double vision on waking as the only presenting complaint. Examination on admission disclosed abduction failure in the left eye with an otherwise unremarkable examination. On the second day of admission, abduction failure in the right eye and generalised hyporeflexia was elicited. This progressed to areflexia and total ophthalmoplegia by the 5th day of ad...

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Detection of Serum Anticerebellar Antibodies in Patients with Miller Fisher Syndrome

Cited by 14 publications

References 15 publications

Clinical and immunological spectrum of the Miller Fisher syndrome

Clinical and immunological spectrum of the Miller Fisher syndrome

Ataxia, Ophthalmoplegia, and Areflexia: What Would You Think?

Transcranial magnetic stimulation studies in the Miller Fisher syndrome: evidence of corticospinal tract abnormality

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