Quantitative cardiovascular autonomic function study in Fisher syndrome

Rk, Lyu; Lm, Tang; Hsu, Wei‐Chung; St, Chen

doi:10.1136/jnnp.73.3.333

Cited by 14 publications

(8 citation statements)

References 16 publications

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“…Four MFS patients progressing to severe tetraparesis showed reduced amplitudes of compound muscle action potentials, additionally suggesting electrophysiological differences from classic demyelinating GBS 70. Quantitative cardiovascular autonomic function tests may be subclinically abnormal in both the parasympathetic and sympathetic components 94…”

Section: Electrophysiological Aspectsmentioning

confidence: 90%

Clinical and immunological spectrum of the Miller Fisher syndrome

2007

Muscle and Nerve

155

132

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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: Electrophysiological Aspectsmentioning

confidence: 90%

Clinical and immunological spectrum of the Miller Fisher syndrome

2007

Muscle and Nerve

155

132

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“…This is consistent with autonomic dysfunction observed in disorders such as Guillain-Barré syndrome and Miller-Fisher syndrome, which are thought to be on the GQ1b Ab spectrum of disease. [17][18][19] Interestingly, these 2 patients had more mild clinical courses during the acute phase, with rapid improvements following IV methylprednisolone therapy monotherapy. Further, GQ1b Ab titer was 1:6400 at diagnosis, which was the median in our study making it difficult to draw conclusions regarding the severity of Bickerstaff brainstem encephalitis and subsequent symptoms of orthostatic hypotension.…”

Section: Discussionmentioning

confidence: 99%

“…Rare reports of continued autonomic dysfunction in Guillain-Barré syndrome and Miller-Fisher syndrome exist but this has not been described in Bickerstaff brainstem encephalitis. 7,8 Beyond description of focal neurologic deficits after diagnosis and treatment, existing literature is limited on the post-acute neurologic and immunologic clinical courses of patients with Bickerstaff brainstem encephalitis. [2][3][4][5][6][7][8][9] This study investigates the long-term neurologic and immunologic outcomes of 5 previously published cases of pediatric Bickerstaff brainstem encephalitis and identify areas of clinical investigation in the follow-up of this population.…”

mentioning

confidence: 99%

Long-term Sequelae of Pediatric Bickerstaff Brainstem Encephalitis Includes Autonomic and Sleep Dysregulation

Santoro

2019

J Child Neurol

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Bickerstaff brainstem encephalitis is a rare, immune-mediated disorder of the brainstem and peripheral nervous system. Published knowledge of pediatric Bickerstaff brainstem encephalitis focuses on the acute phase of the disease process. This study evaluated longterm neurologic and immune sequelae of Bickerstaff brainstem encephalitis in children. A single-center retrospective chart review was performed. Clinical data, neuroimaging, polysomnograms, and serum data were reviewed. Five patients were included in this study. Four patients had no neurologic residua, and 1 patient continued to have mild bulbar dysfunction. There was neither recurrence of symptoms nor development of other neurologic or immunologic disorders at a median of 3 years after diagnosis. Review of systems was largely negative, although 2 patients endorsed symptoms consistent with mild orthostatic hypotension for 1 year after diagnosis, but these findings were not sustained. Four of 5 patients endorsed sleep dysregulation. Three patients met criteria for pediatric obstructive sleep apnea. Prognosis following pediatric Bickerstaff brainstem encephalitis is excellent although posttreatment autonomic and sleep dysregulation may reflect residua from acute phase inflammation in the peripheral nervous system and connections of the reticular activating formation of the brainstem, although this was time limited. Further prospective, multicenter, analysis is warranted.

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“…MFS causes autonomic dysfunction4 and pupil dilatation,5 thereby precipitating AACG in susceptible eyes. There is one previous report of acute glaucoma in a patient with known MFS6, but we are the first to report MFS presenting with BSAACG.…”

Section: Discussionmentioning

confidence: 99%