Home About us Contact
|
Home About us Contact | ||
|
|
||
Miller Fisher Syndrome (miller + fisher_syndrome)
Selected AbstractsGuillain,Barré syndrome in southern Taiwan: clinical features, prognostic factors and therapeutic outcomesEUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2003B.-C. Cheng To determine the clinical features, prognostic factors, and therapeutic results of Guillain,Barré syndrome (GBS) in order to improve the therapeutic strategy for this disease. We retrospectively reviewed the electrodiagnostic study and medical records of patients with GBS admitted to Chang Gung Memorial Hospital, Kaohsiung, between January 1986 and December 2000. Outcomes and prognosis were followed-up after 1 year. Ninety-six patients were enrolled in this study. According to the clinical and electrophysiological findings, 77 patients were acute inflammatory demyelinating polyradiculoneuropathy, seven were Miller Fisher syndrome, and six were axonal forms, and six were unclassified. At a follow-up of 1 year, 61 patients (64%) recovered, 30 (31%) had residua and five (5%) died. Amongst these 30 had residua, including unassisted gait in 19, assisted gait in four, and wheel/bed bound in seven. According to the statistical analysis, disabilities at the nadir (P < 0.0001) and at admission (P = 0.014) were significant prognostic factors. Variables used for the stepwise logistic regression, and the results revealed that after analysis for all the above variables, only disability at the nadir (P < 0.0001) was independently associated with the treatment failure rate. Our study revealed 27% of cases in need of respiratory support during hospitalization, and 5% of hospital-treated patients die from the complications. Furthermore, 31% had residua at a follow-up of 1 year or more. If prognostic factors are considered, disability at the nadir during hospitalization demonstrates consistently poor therapeutic outcomes. Therefore, early diagnosis, choice of appropriate treatment, and preventing complications during acute stages are essential to maximize the potential for survival. [source] Anti-disialosyl antibodies mediate selective neuronal or Schwann cell injury at mouse neuromuscular junctionsGLIA, Issue 3 2005Susan K. Halstead Abstract The human paralytic neuropathy, Miller Fisher syndrome (MFS) is associated with autoantibodies specific for disialosyl epitopes on gangliosides GQ1b, GT1a, and GD3. Since these gangliosides are enriched in synaptic membranes, anti-ganglioside antibodies may target neuromuscular junctions (NMJs), thereby contributing to disease symptoms. We have shown previously that at murine NMJs, anti-disialosyl antibodies induce an ,-latrotoxin-like effect, electrophysiologically characterized by transient massive increase of spontaneous neurotransmitter release followed by block of evoked release, resulting in paralysis of the muscle preparation. Morphologically, motor nerve terminal damage, as well as perisynaptic Schwann cell (pSC) death is observed. The relative contributions of neuronal and pSC injury to the paralytic effect and subsequent repair are unknown. In this study, we have examined the ability of subsets of anti-disialosyl antibodies to discriminate between the neuronal and glial elements of the NMJ and thereby induce either neuronal injury or pSC death. Most antibodies reactive with GD3 induced pSC death, whereas antibody reactivity with GT1a correlated with the extent of nerve terminal injury. Motor nerve terminal injury resulted in massive uncontrolled exocytosis with paralysis. However, pSC ablation induced no acute (within 1 h) electrophysiological or morphological changes to the underlying nerve terminal. These data suggest that at mammalian NMJs, acute pSC injury or ablation has no major deleterious influence on synapse function. Our studies provide evidence for highly selective targeting of mammalian NMJ membranes, based on ganglioside composition, that can be exploited for examining axonal,glial interactions both in disease states and in normal NMJ homeostasis. © 2005 Wiley-Liss, Inc. [source] Clinical presentation and prognosis of childhood Guillain,Barré syndromeJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 7-8 2008Jung Hwan Lee Aim: Guillain,Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterised by rapidly progressive, symmetric weakness and areflexia. This study is to assess the clinical characteristics of paediatric GBS, as well as its long-term functional prognosis. Methods: We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 56 children diagnosed with GBS. Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy ([AIDP]n = 34), acute motor axonal neuropathy ([AMAN]n = 14), acute motor and sensory axonal neuropathy (n = 1) and Miller Fisher syndrome ([MFS]n = 7). Results: Upper respiratory infection was the most frequent preceding event, and limb weakness was the most frequent symptom at GBS onset. There was no significant difference in the mean time from the onset of illness to nadir between any of these groups. Both the AIDP and AMAN groups showed significantly poorer functional status, measured by the Hughes scale, than the MFS group. Two years after nadir, however, the three groups did not differ significantly. Functional status at nadir, as estimated by the Hughes scale, is a more important factor than electrophysiological types in predicting long-term outcome. Conclusion: The most common symptom at onset in paediatric GBS was limb weakness. Functional status at nadir in AMAN was not significantly different from that of AIDP, and both types achieved good functional outcome for ambulation after 2 years. Functional status at nadir was more important than the electrophysiological type in predicting long-term outcomes. [source] A child with Miller Fisher syndromeJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 4 2002J Garrett Abstract: We describe the case of a six-year-old girl who presented with a 3-day history of diplopia and gait disturbance following a febrile flu-like illness. On examination she was found to have ataxia, areflexia and ophthalmoplegia, and a diagnosis of Miller Fisher syndrome was made after the exclusion of other conditions. This report outlines the frequency of Miller Fisher syndrome and lists the differential diagnoses that should be considered in Australia. In addition, the occurrence of pupillary dysfunction is discussed. [source] Structure Of Campylobacter Jejuni Lipopolysaccharides Determines Antiganglioside Specificity And Clinical Features Of Guillain-Barre, And Miller Fisher PatientsJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 3 2002CW Ang Ganglioside mimicry in the lipopolysaccharide (LPS) fraction of Campylobacter jejuni isolated from Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) patients was compared with isolates from patients with an uncomplicated enteritis. The antibody response to C. jejuni LPS and gangliosides in neuropathy patients and controls was compared as well. LPS from GBS and MFS-associated isolates more frequently contained ganglioside-like epitopes compared to control isolates. Almost all neuropathy patients showed a strong antibody response against LPS and multiple gangliosides in contrast to enteritis patients. Isolates from GBS patients more frequently had a GM1-like epitope than isolates from MFS patients. GQ1b-like epitopes were present in all MFS-associated isolates and was associated with anti-GQ1b antibody reactivity and the presence of oculomotor symptoms. These results demonstrate that the expression of ganglioside mimics is a risk factor for the development of post-Campylobacter neuropathy. This study provides additional evidence for the hypothesis that the LPS fraction determines the antiganglioside specificity and clinical features in post-Campylobacter neuropathy patients. [source] Improvement of respiratory compromise through abductor reinnervation and pacing in a patient with bilateral vocal fold impairment,THE LARYNGOSCOPE, Issue 1 2010Michael Broniatowski MD Abstract Objectives/Hypothesis. To determine whether respiratory compromise from bilateral vocal fold impairment (paralysis) can be objectively alleviated by reinnervation and pacing. Methods. A patient with paramedian vocal folds and synkinesis had a tracheotomy for stridor after bilateral laryngeal nerve injury and Miller Fisher syndrome. One posterior cricoarytenoideus (PCA) received a nerve-muscle pedicle fitted with a perineural electrode for pacemaker stimulation. The airway was evaluated endoscopically and by spirometry for up to 1 year. Results. Bilateral vocal fold patency during quiet breathing was reversed to active vocal fold adduction during tracheal occlusion. Peak inspiratory flows (PIFs) were significantly higher (P < .001) after reinnervation. PIFs and glottic apertures increased further under stimulation (42 Hz, 1,4 mA, 42,400 ,sec). although the differences were not significant. Conclusions. Based on our preliminary data, PCA reinnervation and pacing offer promise for amelioration of respiratory compromise after paradoxical adduction in bilateral vocal fold impairment. Laryngoscope, 2010 [source] Clinical, electrophysiological, and serological overlap between Miller Fisher syndrome and acute sensory ataxic neuropathyACTA NEUROLOGICA SCANDINAVICA, Issue 5 2002H. Shimamura We report a patient with severe sensory ataxia, areflexia, and ophthalmoplegia with preservation of limb muscle strength. Electrophysiological examinations revealed peripheral sensory nerve involvement. A serological examination showed the elevation of IgG antibodies to various b-series gangliosides as well as GT1a. These indicated that this case is an overlap between acute sensory ataxic neuropathy and Miller Fisher syndrome. Autoantibody is implicated as potential pathogenic agents in some cases of acute sensory ataxic neuropathy. [source] Is Campylobacter lipopolysaccharide bearing a GD3 epitope essential for the pathogenesis of Guillain,Barré syndrome?ACTA NEUROLOGICA SCANDINAVICA, Issue 2 2000N. Yuki The hypothesis has been proposed that the GD3 ganglioside-like lipopolysaccharide (LPS) is essential for and functions in the development of Guillain,Barré syndrome (GBS) and Miller Fisher syndrome (MFS) subsequent to Campylobacter jejuni enteritis. Our study showed that patients with GBS or MFS who had previously suffered diarrhea had anti-GD3 antibodies less often than those who had not had diarrhea. Sera from patients who showed GBS or MFS with the serologic evidence of prior C. jejuni infection had anti-GD3 antibodies less frequently than sera from those without evidence of infection. Statistical analysis showed that anti-GD3 antibodies were less frequent in patients with GBS or MFS from whom C. jejuni had been isolated than were other anti-ganglioside antibodies, such as anti-GM1 antibodies. These results could not support the above hypothesis. [source] | ||||||||||