Atypical Miller Fisher Syndrome Research Articles

A Case of Atypical Miller Fisher Syndrome with Headache Accompanied by Unilateral Extraocular Muscle Paralysis and Literature Review

A Case of Atypical Miller Fisher Syndrome with Headache Accompanied by Unilateral Extraocular Muscle Paralysis and Literature Review

Acute eye movement-retained internal ophthalmoplegia in atypical Miller Fisher syndrome variants are associated with IgG anti-GQ1b antibodies

Ophthalmoplegia is a common neurological finding in Miller Fisher syndrome (MFS), but acute eye movement-retained internal ophthalmoplegia is a rare variant of MFS. In this report, we present three cases of acute eye movement-retained internal ophthalmoplegia; IgG anti-GQ1b antibodies were detected in all patients. We reviewed a total of 13 cases, including 10 previously reported cases, and revealed that IgG anti-GQ1b antibodies were identified in all but one patient. In addition, we investigated the correlation between acute eye movement-retained internal ophthalmoplegia and antibodies against ganglioside complexes. We found that anti-GQ1b antibodies in our patients reacted with GQ1b or GT1a but not GQ1b/GM1 or GQ1b/GD1a complexes, indicating that antibodies against disialosyl residues have a pathogenetic role in acute eye movement-retained internal ophthalmoplegia. This is the first study describing that acute eye movement-retained internal ophthalmoplegia is associated with IgG antibodies specific to GQ1b or GT1a.

Bilateral Sixth nerve palsy in a pediatric patient as first presentation of atypical miller fisher syndrome

AbstractPurposeTo describe a case of bilateral sixth nerve palsy in the setting of an atypical Miller Fisher Syndrome (MFS), extremely rare in the pediatric population and barely documented in the literatureMethodsWe present a case of a 12‐year‐old girl who came to the emergency room for diplopia of 24 hours. She had no history of a previous infectious or febrile process, except for an episode of abdominal pain 8 days earlier. The examination revealed bilateral sixth nerve palsy. Hospital admission was decided. Two days later the clinical picture worsened, appearing swallowing difficulty, decreased strength and muscle tone in all 4 extremities. The tendon reflexes were preserved. Craniospinal MRI and lumbar puncture were normal. The electroneurogram was consistent with early stage of polyradiculoneuritis. Autoimmunity tests showed positive anti‐GT1a antibodies and negative anti‐GQ1b antibodies.ResultsBased on the findings, treatment with immunoglobulin and intravenous corticosteroids was decided. Clinical resolution was progressive and the oculomotor alteration completely remitted at 2 months.ConclusionsMFS is a rare autoimmune disease characterized by the classic triad of ophthalmoplegia, ataxia, and areflexia. It is considered a variant of Guillain‐Barré syndrome. There are few cases or series of cases described in the literature on MFS and its behavior in pediatrics. Therefore, almost all clinical and laboratory findings have been described in adults, but in pediatrics, there is still uncertainty. This can lead to difficulties in the diagnosis of MFS in these patients. MFS should always be suspected in the context of acute diplopia or oculomotor paralysis in pediatrics, especially if it is bilateral; despite that its bilateral character seems to be less than in adults. Fortunately, even in these patients, it maintains its self‐limited behavior, so a full recovery is expected.Mane S, (2020) Miller Fisher in a Child. Journal of Pediatric Neurosciences.

A Case Study of a Patient with Diplopia and Bilateral Facial Palsy Due to Atypical Miller Fisher Syndrome: Treatment with Complex Korean Medicine

Miller Fisher syndrome (MFS) is a rare variant of Guillain?Barr? syndrome characterized by ocular paralysis, ataxia, and insensitivity. This report describes the effect of Complex Korean Medicine Treatment (CKMT) on a patient previously diagnosed with MFS presenting with diplopia and facial palsy. The distance at which diplopia occurs, the diplopia questionnaire, the range of diplopia, the degree of facial paralysis, and the degree of ptosis were evaluated at the time of admission and weekly for 1 month. After receiving CKMT for 4 weeks the 62-year-old female had improved symptoms of diplopia, bilateral facial palsy and ptosis caused by MFS. These results show the significant association of MFS with facial paralysis and the improvement achieved with CKMT.

A Case of Atypical Miller Fisher Syndrome

A Case of Atypical Miller Fisher Syndrome

G.P.412 - Atypical Miller Fisher syndrome presenting as acute isolated bilateral ophthalmoplegia in young children

G.P.412 - Atypical Miller Fisher syndrome presenting as acute isolated bilateral ophthalmoplegia in young children

PP08.12 – 2493: Atypical Miller Fisher syndrome presenting as acute isolated bilateral ophthalmoplegia: Report of two nex patients with serological specificity

Objective Miller Fisher syndrome (MFS) is an acute polyradiculoneuritis regarded as an uncommon clinical variant of Guillain-Barre syndrome (GBS) asociated with antiGQ 1b positvie serology. MFS is characterized by the acute onset of a clinical triad of ophthalmoplegia, cereballar ataxia and areflexia. Atypical forms of MFS presenting as ophthalmoplegia without ataxia, have been scarcely described. Methods A detailed clinical and serological description of 2 young childhood cases of acute isolated bilateral ophthalmoplegia. Results Case 1 6-year-old boy presented with acute onset of diplopia, ten days after he experienced diarrhea and fever. Neurologic examination showed bilateral isolated sixth nerve palsy, more prominent on the left. Investigations including brain imaging, lumbar puncture and fundoscopic exam were normal. C. jejuni serology showed elevated IgG and IgA. Analysis of anti-ganglioside antibodies by ELISA in serum obtained immediately after admission revealed high levels of anti-GQ1b, anti-GT1a and anti-GD3 IgG and IgM. Case 2 4-year-old boy admitted with limited abduction of both eyes, as well as adduction paresis of the right eye. Two weeks before, the boy suffered from a febrile episode with diarrhea. All investigations carried out on admission were normal. ELISA for anti-ganglioside antibodies was performed on the serum obtained 2 weeks after onset and revealed elevated titers of anti-GQ1b and anti-GT1a IgG and IgM. Serology was positive to C. jejuni. We observed a complete spontaneous resolution 8 weeks after onset in both children. Conclusion The two patients we decribed confirm the rare but possible occurrence of atypical MFS in young children a few weeks following gastrointestinal infection. Identification of high titers of anti-GQ1b IgG associated with positive serology for C. jejuni enabled the diagnosis of atypical MFS. This paper highlights the relevance of measuring serum anti-GQ1b IgG level in the diagnostic work-up of acute isolated ophthalmoplegia of unknown aetiology in a child.

Atypical Miller Fisher Syndrome with Anisocoria and Rapidly Fluctuating Pupillary Diameter.

Miller Fisher syndrome is a variant of Guillain-Barre syndrome characterized by the classic triad of ophthalmoplegia, ataxia, and areflexia. Pupillary involvement is common in MFS and has been reported in 35–42% of MFS patients. Although case reports have discussed isolated ophthalmoplegia as a presentation of MFS, anisocoria and rapid fluctuation of pupillary diameter have not been reported in anti-GQ1b antibody positive individuals. Here we describe an individual who presented with diplopia and was found to have progressive internal and external ophthalmoplegia with frequent fluctuations in pupillary diameter and anisocoria. These exam findings are not commonly described even in atypical presentations of MFS. The onset of symptoms was preceded by an upper respiratory infection but no gastrointestinal symptoms. Imaging and CSF studies were unremarkable; however serum levels of immunoglobulin G anti-GQ1b antibody and anti-GAD antibody were elevated confirming the diagnosis of MFS. The patient was treated with IVIG and intravenous steroids with mild resolution of external ophthalmoplegia. He did not go on to develop more typical features of MFS such as ataxia or areflexia. This demonstrates that isolated external and internal ophthalmoparesis with rapidly fluctuating pupillary diameter and associated anisocoria can be the sole manifestation of atypical MFS.

Acute isolated ophthalmoplegia with anti-GQ1b antibodies

Ophthalmoplegia without ataxia, areflexia or both has been designated as atypical Miller Fisher syndrome (MFS) or acute ophthalmoplegia (AO). This entity, first reported by Chiba et al. is associated with anti-GQ1b IgG antibodies.We report a patient with isolated acute ophthalmoplegia with high titer of anti-GQ1b IgG antibody activity in the acute phase in whom treatment with intravenous immunoglobulin (IVIg) led to the clinical recovery and the decrease in antibody titer.

Acute bilateral mydriasis associated with anti-GQ1b antibody

Miller Fisher syndrome (MFS) is an autoimmune neuropathy characterized by external ophthalmoplegia, ataxia and areflexia. Mydriasis is present in 35% of typical MFS. We report five patients with acute bilateral mydriasis, either isolated or associated with external ophthalmoplegia for which the presumed diagnosis of “atypical MFS” was confirmed by the positivity of anti-GQ1b antibodies. Acute bilateral mydriasis raises important differential diagnoses in clinical practice. This report demonstrates that acute mydriasis can be autoimmune mediated and that anti-GQ1b antibodies are useful to confirm the diagnosis in unexplained cases.

Neuro-ophthalmology and the anti-GQ1b antibody syndromes

The discovery of the association of the anti-GQ1b IgG antibody with the postinfectious clinical syndromes of ophthalmoplegia, ataxia, and areflexia helped house the phenotypes of the Miller Fisher syndrome (MFS), atypical MFS, Guillain-Barré syndrome with ophthalmoplegia, and Bickerstaff's brainstem encephalitis under one roof. The neuro-ophthalmologic signs classically predominate and may vary from case to case, but they maintain clinically recognizable patterns that assist with the diagnosis. The identification of a common lipopolysaccharide on the plasma membrane in human cranial and peripheral nerves at the GQ1b epitope and on infectious particles of bacteria and viruses (ie, Campylobacter jejuni) demonstrates molecular mimicry. The high frequency of oculomotor dysfunction is partially explained by the tissue ganglioside concentration and distribution and the attraction of antibody-stimulating complement activation. Current experimental treatment targets antibody removal and neutralization and prevents membrane attack complex formation through deactivation of complement. This article aims to bring together the historically disparate opinions on the origins of these syndromes as either a purely peripheral nervous system or central nervous system dysfunction, highlight the clinical neuro-ophthalmologic signs, discuss some of the biology of the anti-GQ1b antibody, and review imaging abnormalities and treatment of this fascinating disorder.

A Case of Anti-GQ1b-Positive Atypical Miller Fisher Syndrome With Pupil Involvement

Purpose: To report a case of atypical Miller Fisher syndrome with pupil involvement. Case summary: An 18-year-old woman visited our clinic with a headache and blurred vision. Ophthalmic examination revealed a corrected visual acuity of 20/20 in both eyes, with both pupils dilated to 7.5 mm. There was a loss of light reflex and near reflex in both eyes. Ocular motor examination revealed bilateral abduction, and supraduction limitation, and mild adduction limitation. Neurological examination demonstrated hyporeflexia without ataxia. Brain CT, brain MRI, nerve conduction test, CSF study, and thyroid function tests were normal. The serologic anti-GQ1b IgG test was positive. Conclusions: We confirmed a case of atypical Miller Fisher syndrome with an anti-GQ1 antibody test that had pupil involvement, areflexia, and acute ophthlamoparesis.

Acute Ophthalmoplegia with Tonic Pupils

A 38-year-old female was diagnosed with atypical Miller Fisher syndrome (MFS) after she presented with bilateral ophthalmoplegia and bilateral tonic pupils without ataxia or areflexia following a flu-like infection. She had negative findings for a CSF examination, brain imaging and electrophysiological response; furthermore she was negative for serum ganglioside antibodies. We reported this interesting case and discuss the atypical presentation.

Atypical Miller Fisher syndrome with complete bilateral ophthalmoplegia mimicking brainstem stroke

We report on three elderly patients with stroke-like onset of atypical Miller Fisher syndrome (MFS). The serum titer of anti-GQ1b IgG was markedly elevated in these patients. Their prognoses were sufficiently good with immunoadsorption therapy with or without intravenous immune globulin (IVIg) therapy. However, some neurological findings were not characteristic of typical MFS. Patient 1 suffered from prolonged dysesthesia in her left extremities, and Patients 2 and 3 showed no ataxia. Moreover, complete bilateral gaze limitation is rare in MFS. The sudden stroke-like onset along with the gaze limitation of these patients suggests that the unexpected elevation in the serum titer of anti-GQ1b IgG due to unknown immune dysregulation might have severely affected the third, fourth, and sixth nerves and this potent antibody rapidly attacked these nerves and induced stroke-like clinical features and complete ophthalmoplegia.

Anti-GQ1b-negative Miller–Fisher syndrome with lower cranial nerve involvement from parasinusoidal aspergilloma

Miller-Fisher syndrome (MFS) typically presents with ophthalmoplegia, ataxia, and areflexia. Atypical MFS additionally includes bulbar impairment, affection of the limbs, or abortive presentations. Mostly, MFS follows an infection with Campylobacter jejunii. Aspergilloma has not been reported to trigger MFS. In a 48-year-old male tiredness, tinnitus, otalgia, parietal hyperaesthesia, coughing, plugged nose, hypoacusis, globus sensation, epipharyngeal pain, dysarthria, hypogeusia, arthralgia, lid cloni, facial hypaesthesia and tooth ache consecutively developed. There were occasional lid cloni, left-sided facial hypaesthesia, reduced gag reflex, divesting soft palate, and absent tendon reflexes. CSF investigations revealed normal cell-count but increased protein. Antibodies against GM1 and GQ1b were negative. Atypical MFS was diagnosed. Otolaryngological examinations revealed chronic sinusitis maxillaris from an aspergilloma. After immunoglobulins and resectioning of the aspergilloma, neurological abnormalities disappeared within 19d. MFS may manifest as unilateral lower cranial nerve lesions without affection of the upper cranial nerves or ataxia. Atypical MFS may be triggered by parasinusoidal aspergilloma.

Successive yet distinct episodes of isolated ophthalmoplegia associated with anti-GQ1b antibody

Recurrent episodes of the Miller Fisher syndrome (MFS) are uncommon.1 Furthermore, isolated internal ophthalmoplegia is rare in MFS.2,3 We report a patient who presented first with internal/external ophthalmoplegia, then 7 years later with an isolated internal ophthalmoplegia associated with elevated anti-GQ1b IgG antibodies and confirmed Campylobacter jejuni enteritis. A 20-year-old Latino man first presented to an outside neurology group with double vision, photophobia, and ataxia following a bout of diarrhea 2 days prior to symptom onset. On examination there were limited extraocular movements, dilated, non-reactive pupils, impaired vibratory sense of the distal extremities, and normal reflexes. After unremarkable brain imaging and spinal fluid results, the patient was diagnosed with atypical MFS. No treatment was initiated and symptoms resolved over the course of 3 months. At age 27 years he presented again with the gradual onset of double vision, photophobia, and paresthesias of the …

Atypical Miller Fisher syndrome with GQ1b antibodies

An atypical case of Miller Fisher syndrome is described in a patient with ophthalmoplegia and mild ataxia but no areflexia. High titres of acute phase antibodies to gangliosides asialo-GM1 and GQ1b were detected. Asialo-GM1 antibodies have not been previously reported in association with Miller Fisher syndrome. Considerable clinical recovery occurred in association with reduction in the ganglioside antibody titres. Ganglioside antibody assays may be helpful in the diagnosis of atypical cases of Miller Fisher syndrome. Detailed clinical, radiological and laboratory evaluation of suspected cases is warranted to improve our understanding of Miller Fisher syndrome. Such studies and the correlation with ganglioside antibody titres will also provide insights into the relationship between the classical and atypical cases of Miller Fisher syndrome, Guillain-Barré syndrome and Bickerstaff's brain stem encephalitis.

Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies.

To determine the significance of serum anti-GQ1b IgG antibody, we studied the disease spectrum associated with this antibody and GQ1b epitope in the human nervous system. We examined sera from 19 patients with typical Miller Fisher syndrome (MFS), five patients with acute postinfectious ophthalmoplegia without ataxia (atypical MFS), six patients with Guillain-Barré syndrome (GBS) with ophthalmoplegia (GBS-OP[+]), and 23 patients with GBS without ophthalmoplegia (GBS-OP[-]). We also examined sera from 84 patients with other neurologic or non-neurologic disorders and from 16 normal control subjects. Eighteen of the 19 patients with typical MFS, all the patients with atypical MFS, and five of the six patients with GBS-OP(+) had increased anti-GQ1b IgG activity in ELISA, but none of the patients in the other groups, including GBS-OP(-), had it. All the patients' sera that had anti-GQ1b IgG antibody showed anti-GT1a IgG activity. Results of absorption studies suggested that the same antibody reacted with GQ1b and GT1a. An anti-GQ1b mouse monoclonal antibody immunostained the paranodal regions of the extramedullary portion of the human oculomotor, trochlear, and abducens nerves. Biochemical analysis showed that the human oculomotor nerve contained a larger amount of GQ1b than did the ventral and dorsal roots of the spinal cord. We conclude that serum IgG antibody against GQ1b is very closely associated with acute postinfectious ophthalmoplegia in MFS and GBS.