Purpose To review recent knowledge on the clinical features, pathology and pathophysiology, diagnosis and treatment of Miller Fisher syndrome (MFS) Data sources Clinical and laboratory studies on MFS in the past 1...Purpose To review recent knowledge on the clinical features, pathology and pathophysiology, diagnosis and treatment of Miller Fisher syndrome (MFS) Data sources Clinical and laboratory studies on MFS in the past 10 years were included Results A viral infection preceded neurological symptoms in 71 8% of MFS patients Typical MFS consists of the triad of ataxia, areflexia and ophthalmoplegia Other cranial nerves are also involved, which may overlap with limb weakness in typical Guillain Barre syndrome (GBS) Lower cranial nerve variants of GBS, atypical MFS and ataxic neuropathies may overlap, and are thought of as variant forms of MFS Recurrence and CNS involvement is found more frequently in MFS than in GBS Antibody to GQ1b, a tetrasyaloganglioside (GQ1b antibody) which is found in close relation to ophthalmoplegia in MFS, is also associated with Campylobacter jejuni (C jejuni) serotype Penner 2 This suggests that C jejuni may induce MFS via the GQ1b structure The GQ1b antibody may lead to the failure of acetylcholine release from motor nerve terminals, which has been confirmed by clinical neurophysiological results Conclusions Many studies have shown similarities in the pathogenesis of MFS and GBS However, there are still some differences between them, especially in the areas of sensory and CNS involvement The GQ1b antibody is thought of as one of the key factors in the pathogenesis of MFS, especially with ophthalmoplegia, and it may prove a useful clinical marker in the diagnosis of MFS展开更多
Miller Fisher综合征(MFS)和Bickerstaff脑干脑炎(BBE)为临床少见疾病,两者是否为两种不同的疾病一直存在着争议。抗GQ1b抗体综合征的提出,将有着共同血清抗体的不同临床表现的这两种疾病概括为同一连续性疾病谱,促进了对MFS、BBE及相...Miller Fisher综合征(MFS)和Bickerstaff脑干脑炎(BBE)为临床少见疾病,两者是否为两种不同的疾病一直存在着争议。抗GQ1b抗体综合征的提出,将有着共同血清抗体的不同临床表现的这两种疾病概括为同一连续性疾病谱,促进了对MFS、BBE及相关疾病的认识。本文就有关MFS、BBE及抗GQ1b抗体综合征的发病机制、临床特征、诊断、治疗及预后等进行综述。展开更多
BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial rol...BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial roles in the development of MFS.The positive rate of ganglioside antibodies is exceptionally high in MFS patients,particularly for anti-GQ1b antibodies.However,the presence of other ganglioside antibodies does not exclude MFS.CASE SUMMARY We present a 56-year-old female patient who suddenly developed right blepharoptosis and progressively worsening vision in both eyes.There were flu symptoms prior to onset,and a coronavirus disease 2019 test was positive.On physical examination,the patient exhibited bilateral extraocular muscle paralysis,weakened reflexes in both limbs,and impaired coordination.The cerebrospinal fluid examination results showed no obvious abnormalities.Bilateral peroneal nerve F-waves were not extracted.Serum anti-GD1b IgG and anti-GT1a IgG antibodies were positive.The patient received intravenous methylprednisolone(1000 mg/day),with the dosage gradually decreased.Additionally,intravenous high-dose immunoglobulin treatment was administered for 5 days(0.4 g/kg/day)from day 2 to day 6 of hospitalization.The patient’s symptoms improved after treatment with immunoglobulins and hormones.CONCLUSION Positive ganglioside antibodies may be used as supporting evidence for the diagnosis;however,the diagnosis of MFS is more reliant on clinical symptoms.展开更多
文摘Purpose To review recent knowledge on the clinical features, pathology and pathophysiology, diagnosis and treatment of Miller Fisher syndrome (MFS) Data sources Clinical and laboratory studies on MFS in the past 10 years were included Results A viral infection preceded neurological symptoms in 71 8% of MFS patients Typical MFS consists of the triad of ataxia, areflexia and ophthalmoplegia Other cranial nerves are also involved, which may overlap with limb weakness in typical Guillain Barre syndrome (GBS) Lower cranial nerve variants of GBS, atypical MFS and ataxic neuropathies may overlap, and are thought of as variant forms of MFS Recurrence and CNS involvement is found more frequently in MFS than in GBS Antibody to GQ1b, a tetrasyaloganglioside (GQ1b antibody) which is found in close relation to ophthalmoplegia in MFS, is also associated with Campylobacter jejuni (C jejuni) serotype Penner 2 This suggests that C jejuni may induce MFS via the GQ1b structure The GQ1b antibody may lead to the failure of acetylcholine release from motor nerve terminals, which has been confirmed by clinical neurophysiological results Conclusions Many studies have shown similarities in the pathogenesis of MFS and GBS However, there are still some differences between them, especially in the areas of sensory and CNS involvement The GQ1b antibody is thought of as one of the key factors in the pathogenesis of MFS, especially with ophthalmoplegia, and it may prove a useful clinical marker in the diagnosis of MFS
文摘BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial roles in the development of MFS.The positive rate of ganglioside antibodies is exceptionally high in MFS patients,particularly for anti-GQ1b antibodies.However,the presence of other ganglioside antibodies does not exclude MFS.CASE SUMMARY We present a 56-year-old female patient who suddenly developed right blepharoptosis and progressively worsening vision in both eyes.There were flu symptoms prior to onset,and a coronavirus disease 2019 test was positive.On physical examination,the patient exhibited bilateral extraocular muscle paralysis,weakened reflexes in both limbs,and impaired coordination.The cerebrospinal fluid examination results showed no obvious abnormalities.Bilateral peroneal nerve F-waves were not extracted.Serum anti-GD1b IgG and anti-GT1a IgG antibodies were positive.The patient received intravenous methylprednisolone(1000 mg/day),with the dosage gradually decreased.Additionally,intravenous high-dose immunoglobulin treatment was administered for 5 days(0.4 g/kg/day)from day 2 to day 6 of hospitalization.The patient’s symptoms improved after treatment with immunoglobulins and hormones.CONCLUSION Positive ganglioside antibodies may be used as supporting evidence for the diagnosis;however,the diagnosis of MFS is more reliant on clinical symptoms.
