CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome.

BACKGROUND AND OBJECTIVES: To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study. METHODS: Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/µL). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%). RESULTS: In 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/µL in 1,005 patients (83%), 5-49 cells/µL in 200 patients (16%), and ≥50 cells/µL in 13 patients (1%). DISCUSSION: ACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/µL, is compatible with GBS after a thorough exclusion of alternative diagnoses. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.

Recurrent Miller-Fisher syndrome overlapping Guillain-Barrè syndrome and Bickerstaff brainstem encephalitis: A case report.

Miller-Fisher syndrome (MFS) together with Guillan-Barré syndrome (GBS) and Bickerstaff brainstem encephalitis (BBE) are considered to form a continuous clinical spectrum of the same disease, possibly affecting the peripheral and/or central nervous systems, with monophasic symptoms. The frequency of overlapping clinical signs and the risk of recurrence are independent and very low, but no cases of GQ1b-seropositive recurrent MFS overlapping with GBS and BBE have been described so far. Here, we describe for the first time an atypical case of recurrent GQ1b-seropositive MFS overlapping GBS and BBE, 12 years after a previous GQ1b-seronegative typical MFS episode. Our case expands the clinical spectrum of recurrent MFS, and it should prompt clinicians to investigate the presence of anti-ganglioside antibodies in recurrent MFS even when these were negative in the previous episode, especially in those presenting with overlapping spectrum symptoms and a critically ill picture during the second episode.

Bilateral vocal cord paralysis in Miller Fisher syndrome/Guillain-Barre overlap syndrome and a review of previous case series.

Miller Fisher syndrome (MFS), an acute demyelinating neuropathy, is characterised by a triad of areflexia, ataxia and ophthalmoplegia. It is the most common variant of Guillain-Barre Syndrome (GBS). In about 5.6%-7.1% of MFS cases, patients also suffer from progressive motor weakness of the limbs. This condition is termed MFS/GBS overlap syndrome. Whether it is in MFS or GBS, bilateral vocal cord paralysis (BVCP) is a rare manifestation with limited cases reported in the literature. We report an extremely rare case where a 65-year-old man developed BVCP in an MFS/GBS overlap syndrome. We have also reviewed previous case reports in the literature for comparison.

Diagnosis of Guillain-Barré syndrome and validation of the Brighton criteria in Malaysia.

We aimed to evaluate the key diagnostic features of Guillain-Barré syndrome (GBS) in Malaysian patients and validate the Brighton criteria. This was a retrospective study of patients presenting with GBS and Miller Fisher syndrome (MFS) between 2010 and 2019. The sensitivity of the Brighton criteria was evaluated. A total of 128 patients (95 GBS, 33 MFS) were included. In the GBS cohort, 92 (97%) patients presented with symmetrical limb weakness. Reflexes were depressed or absent in 90 (95%) patients. Almost all patients (94, 99%) followed a monophasic disease course, with 5 (5%) patients experiencing treatment-related fluctuations. Cerebrospinal fluid (CSF) albuminocytological dissociation was seen in 62/84 (73%) patients. Nerve conduction study (NCS) revealed neuropathy in 90/94 (96%) patients. In GBS patients with complete dataset (84), 56 (67%) patients reached level 1 of the Brighton criteria, 21 (25%) reached level 2, 3 (4%) reached level 3, and 4 (5%) reached level 4. In MFS, the clinical triad was present in 25 (76%) patients. All patients had a monophasic course. CSF albuminocytological dissociation was present in 10/25 (40%) patients. NCS was normal or showed sensory neuropathy in 25/33 (76%) patients. In MFS patients with complete dataset (25), 5 (20%) patients reached level 1 of the Brighton criteria, 14 (56%) reached level 2, 2 (8%) reached level 3, and 4 (16%) reached level 4. Inclusion of antiganglioside antibodies improved the sensitivity of the Brighton criteria in both cohorts. In the Malaysian cohort, the Brighton criteria showed a moderate to high sensitivity in reaching the highest diagnostic certainty of GBS, but the sensitivity was lower in MFS.

[Anti-GQ1b syndrome. A child with Miller-Fisher-Bickerstaff syndrome]. / Síndrome anti-GQ1b en un niño con síndrome de Miller-Fisher-Bickerstaff.

Miller-Fisher syndrome and Bickerstaff brainstem encephalitis, among others, constitute the anti-GQ1b syndrome, with a common immune pathophysiologic pathway characterized by the presence of anti-GQ1b antibodies, which react against the different nervous system GQ1b sites according to their different accessibility. The Miller-Fisher syndrome has a prevalence of 0.09 cases per 100 000 people-year but there are not epidemiological studies about Bickerstaff brainstem encephalitis, that it seems to be less frequent. In spite of having a good natural outcome, the immunoglobulin administration has been established as efficacious at improving it. A twelveyear- old boy suffering from Miller-Fisher-Bickerstaff syndrome after an acute Campylobacter jejuni diarrhea with positive titers of anti-GQ1b and anti-QGT1a antibodies is presented. We communicate a very uncommon pediatric disease with the aim of warning about the importance of its early suspicion and the need of specific laboratory determinations.

El síndrome anti-GQ1b reúne el síndrome de Miller-Fisher y la encefalitis del tronco cerebral de Bickerstaff, entre otras entidades. Tienen etiopatogenia común, constituida por la presencia de anticuerpos anti-GQ1b que reaccionan contra los sitios GQ1b del sistema nervioso según sea su accesibilidad. La prevalencia anual del síndrome de Miller-Fisher es de 0,09 casos por 100 000 habitantes por año y no existen estudios epidemiológicos sobre la encefalitis del tronco cerebral de Bickerstaff, que sería menos frecuente. De evolución natural hacia la mejoría, se beneficia del tratamiento con gammaglobulina endovenosa. Se presenta a un paciente de 12 años con síndrome de Miller- Fisher­Bickerstaff tras un episodio de diarrea aguda por Campylobacter jejuni en el que los anticuerpos anti-GQ1b resultaron positivos. Es nuestro objetivo comunicar sobre un síndrome de presentación poco habitual en pediatría a fin de advertir acerca de la necesidad de su sospecha precoz y solicitud de estudios de laboratorio específicos.

Miller Fisher syndrome and polyneuritis cranialis in COVID-19.

OBJECTIVE: To report 2 patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who presented acutely with Miller Fisher syndrome and polyneuritis cranialis, respectively. METHODS: Patient data were obtained from medical records from the University Hospital "Príncipe de Asturias," Alcalá de Henares, and the University Hospital "12 de Octubre," Madrid, Spain. RESULTS: A 50-year-old man presented with anosmia, ageusia, right internuclear ophthalmoparesis, right fascicular oculomotor palsy, ataxia, areflexia, albuminocytologic dissociation, and positive testing for anti-GD1b-immunoglobulin G antibody. Five days previously, he had developed a cough, malaise, headache, low back pain, and fever. A 39-year-old man presented with ageusia, bilateral abducens palsy, areflexia, and albuminocytologic dissociation. Three days previously, he had developed diarrhea, a low-grade fever, and poor general condition. Oropharyngeal swab test for SARS-CoV-2 by qualitative real-time reverse transcriptase PCR assay was positive in both patients and negative in the CSF. The first patient was treated with IV immunoglobulin and the second with acetaminophen. Two weeks later, both patients made a complete neurologic recovery, except for residual anosmia and ageusia in the first case. CONCLUSIONS: Our 2 cases highlight the rare occurrence of Miller Fisher syndrome and polyneuritis cranialis during the coronavirus disease 2019 (COVID-19) pandemic. These neurologic manifestations may occur because of an aberrant immune response to COVID-19. The full clinical spectrum of neurologic symptoms in patients with COVID-19 remains to be characterized.

Clinical and electrophysiological characteristics of Guillain-Barré syndrome in Colombia.

Despite the wide literature describing the features of Guillain-Barré syndrome (GBS) in different populations worldwide, Colombian data are very scarce. We aim to characterize patients with GBS in a general hospital setting in Colombia. We conducted a retrospective chart review of GBS cases managed at the Hospital Universitario Fundación Santa Fe de Bogotá, from 2011 to 2016. Twenty-three patients were included. The most commonly reported symptoms were paresthesias (65%), pain (61%), proximal (22%) and distal (74%) limb weakness, and facial palsy (30%). 9% of patients had Fisher syndrome and 21% had other variants: Bickerstaff, pharyngeal-cervical-brachial pattern, and facial diplegia. There was a predominance of the demyelinating form (70%), with only 22% of patients presenting with the axonal variants. Our results are concordant with previous studies in Colombia.

Miller Fisher syndrome developing as a parainfectious manifestation of dengue fever: a case report and review of the literature.

BACKGROUND: Although dengue viral infections have emerged as one of the most important mosquito-borne diseases, neurological manifestations of dengue infections are uncommon. Guillain-Barré syndrome and Miller Fisher syndrome have been reported to occur as immune-mediated complications following dengue infection. We report the case of a patient who developed Miller Fisher syndrome during the acute phase of dengue fever suggesting that Miller Fisher syndrome may arise as a result of direct neurotropism of the dengue virus. CASE PRESENTATION: A 70-year-old Sri Lankan man with well-controlled diabetes mellitus and hypertension presented with fever of 3 days' duration, drooping of eyelids, dysarthria, and unsteady gait. He developed bilateral asymmetric partial ptosis, complete external ophthalmoplegia, bilateral palatal palsy, unilateral tongue weakness, ataxia, and areflexia from the second day of illness. He did not have limb weakness. He had evidence of acute dengue infection including progressive thrombocytopenia and leukopenia, positive dengue non-structural protein 1 antigen, dengue immunoglobulin M antibodies, and polymerase chain reaction detection of dengue virus genome in serum. Magnetic resonance imaging of his brain and cerebrospinal fluid analysis were normal. Polymerase chain reaction for dengue virus and immunoglobulin M antibodies in cerebrospinal fluid were negative. Nerve conduction studies showed axonal neuropathy. Antibodies (immunoglobulin G, immunoglobulin M, and immunoglobulin A) against GQ1b and GT1a were negative. He was treated with intravenously administered immunoglobulins and a recommended fluid regimen for dengue fever. He made a complete recovery from dengue fever in 7 days and Miller Fisher syndrome in 20 days. CONCLUSIONS: This case report highlights the rare occurrence of Miller Fisher syndrome during the acute phase of dengue fever. Neurological manifestations may occur as a consequence of direct neurotropism of dengue virus.

A Simplified, Graded, Electrodiagnostic Criterion for Guillain-Barré Syndrome That Incorporates Sensory Nerve Conduction Studies.

Traditional electrodiagnostic (EDX) criteria for Guillain-Barré Syndrome (GBS), e.g. those delineated by Ho et al. and Hadden et al., rely on motor nerve conduction studies (NCS), and focus on differentiating GBS subtypes instead of the accurate diagnosis of GBS. Sensory studies, including the sural-sparing pattern, are not routinely used in GBS EDX. We studied the utility of a simplified criterion that utilizes sensory NCS. Motor and sensory NCS abnormalities were defined by comparing against age and height adjusted norms derived from 245 controls. We considered the sural-sparing pattern a positive diagnostic feature. We analyzed 109 prospectively validated GBS patients and graded them as "Definite", "Probable" and "Possible" based on the number of motor and sensory abnormalities detected. Using proposed EDX criteria, 35.8%, 43.1%, 11.9% of all GBS patients were considered "Definite", "Probable" or "Possible" respectively; whereas traditional EDX criteria only diagnosed 49.5% of cases. 27.5%, 35.3% and 21.6% of patients with the Miller-Fisher Syndrome (MFS) subtype of GBS were considered "Definite", "Probable" or "Possible" respectively. In comparison, traditional criteria only detected 15.7% of cases. Our proposed EDX criterion, that includes sensory NCS, improves and grades the diagnostic certainty of GBS, especially MFS.

A rare mimic of acute stroke: rapidly progressing Miller-Fisher Syndrome to acute motor and sensory axonal neuropathy variant of Guillain-Barre Syndrome.

Ophthalmoplegia, ataxia and areflexia characterise the clinical triad of Miller-Fisher Syndrome (MFS). When the disease presents acutely, it can mimic posterior circulation stroke. We describe a case of an adult patient presenting with sudden dizziness, diplopia, vomiting, and loss of balance. She was initially managed as a case of a brainstem stroke, but the progression of craniopathies without deterioration in sensorium coupled with areflexia clinched the diagnosis of MFS two days into her admission. On the third day, her MFS progressed rapidly to acute motor and sensory axonal neuropathy (AMSAN) variant of Guillain-Barre Syndrome, a rare occurrence in patients with MFS, with only four reported cases including our own. Among the four cases, ours is the only one still non-ambulatory eight months after the initial onset of symptoms. The case highlights the importance of early recognition of MFS in patients with ophthalmoplegia and ataxia despite initially normal reflexes.

The utility of Guillain-Barré syndrome prognostic models in Malaysian patients.

Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that has variable disease course and outcome. The Erasmus GBS outcome score (EGOS), modified EGOS (mEGOS), and Erasmus GBS respiratory insufficiency score (EGRIS) are prognostic models designed to predict the functional outcome of GBS patients at 6 months (EGOS and mEGOS) and the need for mechanical ventilation within a week of admission (EGRIS). The models were primarily developed in the Dutch GBS population, and thus the usefulness of these models in other GBS cohorts is less clear. In the current study, we aimed to validate mEGOS, EGOS, and EGRIS in Malaysian GBS patients. A total of 107 patients with GBS and its variants were consecutively recruited. Patients with GBS and Miller Fisher syndrome (MFS) were analysed separately. In the GBS cohort, high mEGOS and EGOS scores were significantly correlated with poor outcome at 6 months (mEGOS on admission: r = .381, P = .005; mEGOS at day 7 of admission: r = .507, P < .001; EGOS: r = .484, P < .001). However, there were no significant correlations between mEGOS or EGOS and outcome in patients with MFS (mEGOS on admission: r = .152, P = .523; mEGOS at day 7 of admission: r = .008, P = .973; EGOS: r = .110; P = .644). The score of EGRIS for GBS patients with mechanical ventilation was significantly higher than those patients without mechanical ventilation (4 ± 2 vs 3 ± 1; P < .001). We conclude that mEGOS and EGOS are clinically useful and relevant to the Malaysian GBS population but not in patients with classic MFS. EGRIS could be used to predict the need for mechanical ventilation in our local GBS patients.

[Miller Fischer syndrome: about a paediatric case]. / Le syndrome de Miller Fischer: à propos d'un cas pédiatrique.

Miller Fischer (MF) syndrome is a rare variant of Guillain-Barré syndrome which occurs only in 5% of cases among adults while the pediatric cases described in the literature are even more rare. We here report the case of a 12-year old boy who was diagnosed with Miller Fischer syndrome based on clinical presentation characterized by obvious ocular involvement and anti-ganglioside antibody-positivity. The patient was admitted to our hospital with respiratory distress requiring orotracheal intubation and intravenous immunoglobulins, with good clinical evolution.

Prediction of disease progression in Miller Fisher and overlap syndromes.

Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome (GBS) with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE. The clinical, serological, and electrophysiological features were compared. Twenty-three patients presented with MFS, of which 10 (43%) developed limb weakness (MFS-GBS overlap syndrome). All these transitions occurred in the first week after onset of symptoms. There were no differences in the clinical, electrophysiological and serological features at entry between MFS and MFS-GBS. Twelve patients had ophthalmoplegic GBS and the disease severity at nadir and outcome was worse than in the patients with a MFS-GBS overlap syndrome. No early predictors for progression from MFS to MFS-GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least 1 week.

Encefalitis de Bickerstaff / Bickerstaff encephalitis

La encefalitis de tallo cerebral es un síndrome que se presenta con alteración del estado de conciencia, oftalmoplejia, ataxia y signos piramidales. Esta condición neurológica rara que fue descrita en 1950 por primera vez, presenta similares características clínicas a Síndrome de Guillain-Barré, por lo que representa un reto diagnóstico para el clínico. En este artículo se presenta el caso clínico de una paciente de 51 años de edad que se presenta con alteración del estado de conciencia, es llevada a unidad de cuidado intensivo de adulto donde se considera el diagnóstico de encefalitis de Bickerstaff, tras un exhaustivo abordaje diagnostico; el cual se describe, al igual que sumanejo y evolución...(AU)

Brain stem encephalitis is a syndrome that presents with altered state of consciousness, ophthalmoplegia, ataxia and pyramidal signs. This rare neurological condition that was described in1950 by The first time, it presents similar clinical characteristics to Guillain-Barré syndrome, which represents a diagnostic challenge for the clinician. This article presents the clinical case of a 51-year-old patient who presents with altered state of consciousness, is taken to the adult intensive care unit where the diagnosis of Bickerstaff encephalitis is considered, after an exhaustive diagnostic approach ; which is described, as well as its management and evolution ... (AU)

Guillain-Barré Syndrome, variants & forms fruste: Reclassification with new criteria.

OBJECTIVES: This study aimed to evaluate the clinical and electrophysiological characteristics of various distinctive classical and localised Guillain-Barré syndrome (GBS) subtypes. PATIENTS AND METHODS: Clinical characteristics and electrophysiological data of sixty-one consecutive patients admitted between 2012 and 2015 were systematically analysed and reclassified according to the new GBS clinical classification. Neurophysiology was evaluated with Hadden et al.'s vs recently proposed Rajabally et al.'s criteria. Functional severity and clinical outcome of various GBS subtypes were ascertained. RESULTS: All patients initially identified as GBS or related disorders can be sub-classified into having classical GBS (41, 67%), classic Miller-Fisher Syndrome (MFS) (6, 10%), Pharyngeal-cervical-brachial (PCB) (3, 5%), paraparetic GBS (4, 7%), bifacial weakness with paresthesia (3, 5%), acute ophthalmoparesis (AO) (1, 2%) and overlap syndrome (3, 5%): one (2%) with GBS/Bickerstaff brainstem encephalitis overlap and 2 (3%) with GBS/MFS overlap. Greater proportion of axonal classical GBS (67% vs 55%, p=0.372) seen with Rajabally et al.'s criteria and a predominantly axonal form of paraparetic variant (75%) independent of electrodiagnostic criteria were more representative of Asian GBS cohort. Classical GBS patients had lowest admission and discharge Medical Research Council Sum Score (MRCSS), greater functional disability and longest length of in-patient stay. Twenty (20/21, 95%) patients who needed mechanical ventilation had classical GBS. Patients required repeated dose of intravenous immunoglobulin (5/6, 3%) or plasma exchange (4/4, 100%) more frequently had axonal form of classical GBS. CONCLUSION: Phenotype recognition based on new GBS clinical classification, supported by electrodiagnostic study permits more precise clinical subtypes determination and outcome prognostication.

Serial neurophysiological and neurophysiological examinations for delayed facial nerve palsy in a patient with Fisher syndrome.

The patient was a 47-year-old man who presented with diplopia and gait instability with a gradual onset over the course of three days. Neurological examinations showed ophthalmoplegia, diminished tendon reflexes, and truncal ataxia. Tests for anti-GQ1b antibodies and several other antibodies to ganglioside complex were positive. We made a diagnosis of Fisher syndrome. After administration of intravenous immunoglobulin, the patient's symptoms gradually improved. However, bilateral facial palsy appeared during the recovery phase. Brain MRI showed intensive contrast enhancement of bilateral facial nerves. During the onset phase of facial palsy, the amplitude of the compound muscle action potential (CMAP) in the facial nerves was preserved. During the peak phase, the facial CMAP amplitude was within the lower limit of normal values, or mildly decreased. During the recovery phase, the CMAP amplitude was normalized, and the R1 and R2 responses of the blink reflex were prolonged. The delayed facial nerve palsy improved spontaneously, and the enhancement on brain MRI disappeared. Serial neurophysiological and neuroradiological examinations suggested that the main lesions existed in the proximal part of the facial nerves and the mild lesions existed in the facial nerve terminals, probably due to reversible conduction failure.