Typical Guillain-Barré Syndrome Research Articles

When Guillain–Barré Syndrome Defies Expectations: A Case of Atypical Features

Abstract Guillain–Barré syndrome (GBS) is one of the most common causes of acute flaccid paralysis. In typical GBS, there are no central nervous system (CNS) features. GBS with prominent CNS involvement can be seen in uncommon variants of GBS (such as Miller Fisher Syndrome and Bickerstaff brainstem encephalitis [BBE]) or rarely, when it coexists with other conditions such as acute disseminated encephalomyelitis (ADEM). We report a case of a 35-year-old male who presented with fever followed by ascending lower motor neuron type paraparesis suggestive of GBS which progressed rapidly to involve the respiratory muscles necessitating invasive mechanical ventilation. The patient had many atypical features, namely, persistent fever, early bladder and bowel involvement, facial twitching, ophthalmoplegia with ptosis, and a comatose state. Based on these clinical features and the investigations done, a diagnosis of GBS with BBE with a possibility of ADEM was made. The patient was managed intensively, but there was no improvement in the neurological manifestations and the patient succumbed to the illness due to ventilator-associated complications.

Two case reports and a literature review of typical GBS and rare GBS variants associated with COVID-19

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) emerged in late 2019, and is the infectious agent that caused the coronavirus disease 2019 (COVID-19) pandemic. Although respiratory and gastrointestinal manifestations of SARS-CoV-2 are well defined, the spectrum of neurological involvement is less defined. The classic type of Guillain–Barré syndrome (GBS) progresses over days to weeks and has a monophasic course. Areflexia/hyporeflexia and ascending and symmetrical paralysis are observed clinically in patients. It is an autoimmune process that typically leads to the destruction of myelin after infection. There have been numerous reports of adult patients with the coexistence of GBS disease and active COVID-19 illness, but this number is lacking for children. In this study, we present a literature review of the etiological correlation between SARS-CoV-2 and GBS and describe the cases of two pediatric patients with acute monophasic Guillain–Barré syndrome (GBS) during active COVID-19 infection.

Mortality and disability reported after immunoglobulins or plasmapheresis treatment of Guillain-Barré syndrome.

To compare the clinical results (28-day mortality and disability at discharge) in patients with Guillain-Barré Syndrome (GBS) treated with immunoglobulin or plasmapheresis at the Regional Lambayeque in Peru Hospital. Retrospective Cohort Study. Brighton criteria was used for diagnosing GBS, and modified Rankin scale (MRS) was employed for evaluating functional outcome. We used logistic regression for data analyses. A total of 142 cases of GBS diagnosed from 2011 to 2020 were included. GBS presented in a seasonal pattern; 60% of cases occurred in winter and spring. Motor variants (AMAN and AMSAN) accounted for 60% of cases, whereas only 8.5% of cases were typical GBS (AIDP). About 60% of patients were males, and 56% of cases corresponded to the age range of 20-59 years. Patients aged ≥60 years accounted for only 24% of total cases of GBS, but this group had a lethality of 58.3%. Although 28-day mortality was statistically similar in both treatment groups (plasmapheresis or immunoglobulin), we found a trend toward lower mortality in the plasmapheresis group (OR 0.78; 95% CI 0.62-0.97; p=0.062). We found no differences in terms of disability at discharge in GBS patients treated with plasmapheresis or immunoglobulin. Mortality and functional outcome were statistically similar between patients treated with immunoglobulin or plasmatic exchange. However, there was a trend toward lower mortality in patients treated with plasmapheresis.

Incidence of Guillain-Barré syndrome in an Uruguayan population. A prospective cohort study.

Guillain-Barre syndrome (GBS) is an acute autoimmune peripheral inflammatory neuropathy and the most frequent cause of non-poliovirus acute flaccid paralysis worldwide. Background annual GBS incidence rates (IRs) in Latin America (LA) varies from 0.40 to 2.12/100000 persons per year. We performed a prospective population-based epidemiological study to determine the incidence and clinical profile of GBS in the most densely populated regions in Uruguay. The incidence of GBS in the population living in Montevideo and Canelones was studied in the period between June 01, 2018 and May 31, 2020. Patients older than 16 years of age diagnosed with GBS were prospectively enrolled. The mean global annual IR in the Uruguayan population was 1.7/100000 persons (95% CI 1.25-2.25). The highest rate was observed in the 65 to 74 age group among men (5.25/100000 per year) and in the 55 to 64 age group among women (2/100.000 per year). The mean age was 53.9 ± 19.5, years, without difference by sex (53.5 women, 54.5 men). The in-hospital mortality rate was 5.8%. A total of 51 patients were diagnosed with GBS: 42 (82%) had typical GBS, 5 (10%) Miller-Fisher syndrome (MFS), 3 (7%) a bilateral facial nerve palsy, 1 patient had a GBS-MFS overlap (2.3%). This is the first population-based GBS incidence study in LA using a prospective design. Our IR can be a useful tool in establishing the background rate to examine future disease trends caused by the introduction of new viruses or vaccines in Uruguay.

Guillain-Barré syndrome associated with SARS-CoV-2 infection. A systematic review.

Guillain‐Barré syndrome (GBS) incidence can increase during outbreaks of infectious illnesses. A few cases of GBS associated with coronavirus disease 2019 (COVID‐19) infection have been reported. The aim was to identify specific clinical features of GBS associated with COVID‐19. PubMed, Embase and Cochrane were searched from 1 November 2019 to 17 May 2020 and included all papers with full text in English, Spanish, French or Italian, reporting original data of patients with GBS and COVID‐19. Data were extracted according to a predefined protocol. A total of 18 patients reported in 14 papers were included in this review. All the patients were symptomatic for COVID‐19, with cough and fever as the most frequently reported symptoms. The interval between the onset of symptoms of COVID‐19 and the first symptoms of GBS ranged from −8 to 24 days (mean 9 days; median 10 days). Most of the patients had a typical GBS clinical form predominantly with a demyelinating electrophysiological subtype. Mechanical ventilation was necessary in eight (44%) patients. Two (11%) patients died. Published cases of GBS associated with COVID‐19 report a sensorimotor, predominantly demyelinating GBS with a typical clinical presentation. Clinical features and disease course seem similar to those observed in GBS related to other etiologies. These results should be interpreted with caution since only 18 cases have been heterogeneously reported so far.

A lesson learnt from the emergence of Zika virus: What flaviviruses can trigger Guillain-Barré syndrome?

While Zika virus outbreaks raised the concern about rare viral infections in human, attention should also be paid to other Guillain-Barré syndrome (GBS) inducing viruses. This study aims to search for other flaviviruses likely to be associated with GBS. Amino acid (aa) sequence matching analysis was conducted to identify viruses molecularly similar to the Zika virus and human GBS-related proteins. A systematic review of clinical literature was performed to summarize the clinical findings of the GBS-associated flaviviruses identified in the aa sequence matching analysis. It was found that more than 10 pentapeptides were shared between 9 flaviviruses, Zika virus, and human GBS-related proteins. Twenty-six articles totaling 42 clinical cases were eligible for inclusion in the systematic review concerning the nine flaviviruses identified. While some patients showed signs of encephalitis, 5 out of 42 cases demonstrated typical GBS symptoms. Public health professionals should be aware of other GBS-associated flaviviruses and GBS cases with mild symptoms.

A retrospective study on misdiagnosis and prognosis of acute pregnancy complicating Guillain-Barré syndrome

Objective: To investigate the factors of first misdiagnosis, treatment and prognosis of acute pregnancy complicating with Guillain-Barré syndrome (GBS) in order to improve the first diagnosed rate. Methods: A total of 45 acute pregnancy complicating with GBS patients were retrospectively analyzed recruited from January 2009 to October 2017 at the Tianjin Fourth Central Hospital.Patients were divided into the first diagnosis group and the first misdiagnosis group, and GBS clinical types were classified into classic and variant types to analyze the misdiagnosis factors of the first diagnosis. All patients received intravenous immunoglobulin (IVIG) treatment, and the therapeutic effect and prognosis were compared and analyzed. Results: There were 20 cases in the first diagnosis group, 25 cases in the first misdiagnosis group, 35 cases in the typical GBS group, and 10 cases in the variant GBS group.There was no statistically significant difference in the baseline data of the patients(P>0.05).The misdiagnosis factors are divided into four categories: physician factors, patient factors, disease itself factors and laboratory factors. Variant GBS is more likely to lead to misdiagnosis in the first diagnosis than typical GBS. The therapeutic effect of the first diagnosis group was better than that of the first misdiagnosis group(P<0.05).Three patients died in the first misdiagnosis group, and the rest of the pregnant patients gave birth normally, and the babies were born without congenital malformation. Conclusions: Pregnancy is one of the inducing factors of GBS. Early diagnosis and correct treatment can improve maternal and infant clinical outcomes.

Clinical Features of Post-Vaccination Guillain-Barré Syndrome (GBS) in Korea.

Guillain-Barré syndrome (GBS) is the most common immune-mediated polyradiculoneuropathy and it is also the most commonly reported severe adverse event following immunization in adults. To evaluate the results of clinical and laboratory features of GBS after vaccination in Korea, we analyzed the claims-based data from 2002 to 2014 using materials collected for the Advisory Committee Vaccination Injury Compensation (ACVIC) meeting including, clinical features, nerve conduction studies (NCSs), cerebrospinal fluid (CSF) profiles, treatment, and outcomes. Forty-eight compensated GBS cases (median age, 15 years; interquartile range [IQR], 13–51; male:female ratio, 1:1) of 68 suspected GBS were found following immunization and all of them with influenza immunizations with either monovalent (n = 35) or trivalent (n = 13). Among them, 30 cases fulfilled the Brighton criteria level 1–3 (62.5%). The median duration between the onset of symptoms to nadir, duration of the nadir, and total admission period were 3 (IQR, 2–7 days), 2 (IQR, 1–5 days), and 14 (IQR, 6–33 days) days, respectively. The most frequently reported symptom was quadriparesis which was present in 36 cases (75%) at nadir. CSF examination revealed albuminocytologic dissociation in 25.0% and NCS was abnormal in 61.8%. After treatment, most of them showed improvement. Clinical features were similar to typical post-infectious GBS and there were both demyelinating and axonal forms suggesting heterogeneous pathogenic mechanism. In order to improve the diagnostic certainty of post-vaccination GBS, careful documentation of clinical features and timely diagnostic work-up with follow-up studies are needed.

Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy, which has various clinical presentations and both axonal and demyelinating forms. The original description of "ascending paralysis" encompasses the most common varieties: the primary demyelinating form, acute inflammatory demyelinating polyneuropathy (AIDP), and some of the axonal forms, acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN). However, there are now well-documented acute "monophasic" polyneuropathies that have a different clinical phenomenology than that described originally by Guillain, Barré, and Strohl: Miller Fisher syndrome, pure sensory neuropathy/neuronopathy, pandysautonomia, and oropharyngeal variant. Here the authors review both typical GBS (AIDP, AMAN, and AMSAN), and variant syndromes with a focus on clinical and diagnostic features, pathologic findings, pathogenesis, and treatment.

Guillain-Barre Syndrome – rehabilitation outcome, residual deficits and requirement of lower limb orthosis for locomotion at 1 year follow-up

Objective. To analyse long-term functional recovery, deficits and requirement of lower limb orthosis (LLO) for locomotion in patients with Guillain-Barre Syndrome (GBS).Design. Prospective longitudinal follow-up study.Setting. Neurological Rehabilitation unit of university hospital.Patients and Method. Sixty-nine patients of GBS admitted for inpatient rehabilitation. Thirty-five patients (M:F, 19:16) reporting after1 year follow-up (50.72%) were included in study (between September 2005 and July 2009). Their residual deficits and requirement of LLO were recorded and analysed.Results. Age ranged from 4 to 65 year (29.74 ± 15.75). Twenty-seven patients had typical GBS and eight patients had acute motor axonal neuropathy variant. Twenty-eight patients (80%) had neuropathic pain needing medication with 11 required more than one drug. Twenty-one patients (60%) had foot drop and advised ankle-foot orthosis-AFO (20 bilateral AFO). Thirty patients (85.71%) needed assistive devices also for locomotion at discharge. After 1 year, foot drop was still present in 12 patients (34.28%) using orthosis. Modified Barthel Index scores, Modified Rankin Scale and Hughes Disability Scale were used to assess functional disabilities. Significant recovery was observed at the time of discharge and after 1 year (p < 0.001 each).Conclusions. Patients with GBS continue to show significant functional recovery for long period. They have residual deficits even after 1 year with requirement of orthosis in large number of patients.

Guillain-Barre syndrome associated with peginterferon alfa-2a for chronic hepatitis C: A case report

The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.

Guillain-Barré syndrome in child with prolong intubation

Guillain-Barré syndrome (GBS) is an acute demyelinating disorder of the peripheral nervous system that results from an aberrant immune response directed at peripheral nerves. A typical GBS patient presents with rapidly ascending symmetrical weakness, which may progress to respiratory failure in 30% of patients. There are no definite criteria exists in GBS in children regarding prolonged ventilation. Here we report a child of GBS requiring prolonged intubation and ventilation for 60 days who afterward had a complete recovery. We present this case to highlight the importance that even in children prolonged intubation and ventilation of GBS case prognosis can be good.

The Fisher Variant of Guillain-Barré Syndrome (Fisher Syndrome)

Fisher syndrome (FS) is an acute polyneuropathy typically characterized by the triad of ataxia, areflexia, and ophthalmoplegia, although it may present with 2 or even just 1 of these clinical findings. Similarities between FS and other acute polyneuropathies such as Guillain-Barré syndrome (GBS) and Bickerstaff brainstem encephalitis (BBE) suggest that FS is part of a spectrum of autoimmune disorders that may affect the peripheral and/or central nervous system. Anti-GQ1b antibody is present in the serum of more than 85% of patients with FS, but it is not specific to FS. Why some patients develop FS and others develop typical GBS or one of its other variants is still largely unknown.

Guillain–Barré syndrome after rituximab in a patient with idiopathic thombocytopenic purpura: a causal association?

Guillain–Barre syndrome (GBS) is an acute demyelinating inflammatory polyradiculoneuropathy characterised by rapidly progressing predominantly motor impairment and areflexia. Studies in patients and animals have provided convincing evidence that GBS is caused by an aberrant autoimmune response that damages peripheral nerves [1]. GBS rarely develops in patients who suffer from other autoimmune conditions, such as idiopathic thrombocytopenic purpura (ITP), which suggests a shared immune response [2–11]. Rituximab is a monoclonal antibody particularly efficacious in the treatment of several haematological cancers, which has also been used for the treatment of some autoimmune conditions [12]. Here we report on a patient with ITP who developed a typical GBS after treatment with rituximab. This 86-year-old man, with the antecedents of arterial hypertension, chronic obstructive lung disease and atrial fibrillation, consulted in December, 2008, due to purpuric lesions in the lower limbs. He was diagnosed as ITP. His platelet count was 5,000/mm. He received intravenous immunoglobulins (ivIG) 1 mg/kg/day for 2 days plus a descending treatment with oral prednisone (beginning with 1 mg/kg/day) for 1 month. His platelet count after this treatment was 250,000. In January, 2009, he was admitted again because his blood sample showed 10,000 platelets. He was treated with oral prednisone 1 mg/kg/day for 16 consecutive days. This treatment was stopped due to persistent thrombocytopenia (20,000 platelets). IvIG 1 g/kg/day for 2 days and intravenous rituximab (750 mg once a week for 4 weeks) were administered. His platelet count before the last rituximab dose (27 February 2009) was 121,000. Thirty-nine days after the last dose of rituximab (8 April 2009) he developed an acutely ascending progressive tetraparesis. On admission, two days after the beginning of the clinical picture, symmetric weakness, 4/5 in the upper extremities and 3/5 in lower extremities, and universal arreflexia were found. Plantar responses were flexor. Sensory examination was normal and sphincters were not affected. Platelet count was 192,000/mm. A lumbar tap was performed on admission. Glucose levels were normal, there were no cells and protein concentration was 57 mg/dl. The remaining routine laboratory determinations, including tumor markers and a complete autoimmune profile, were normal. With the presumptive diagnosis of Guillain–Barre syndrome, treatment with ivIG 0.4 g/kg/day for 5 days was given. An electrophysiological study, performed six days after the beginning of the symptoms, disclosed slowing of motor conduction velocities (MCV) in all studied nerves. MCV were markedly delayed (\32 m/s) in peroneal and tibial nerves, and slightly slowed in median and ulnar nerves (46 and 49 m/s, respectively). Motor distal latencies (MDL) were clearly prolonged in tibial and medial nerves and within normal limits in peroneal and ulnar nerves. F waves could not be obtained in right peroneal nerve, right posterior tibial nerve and right median nerve, and were delayed in right ulnar nerve. There was attenuation of distal compound muscle R. Jaso C. Valero Services of Internal Medicine, University Hospital Marques de Valdecilla, Santander, Spain

Variants of Guillain-Barré syndrome: low incidence but high impact

Guillain-Barre syndrome (GBS) is an acute monophasic immune-mediated neuropathy that still carries significant morbidity and mortality [2, 6, 17]. It is one of the neurological emergencies and places, given the disability associated with it, a significant burden on patients afflicted, their caregivers and families, and the health system. Overall, up to one-third of the patients require mechanical ventilation and 3–10% still die as a consequence of respiratory failure, autonomic dysfunction such as bradyarrhythmia, tachyarrhythmia, and secondary complications of long-term immobility, e.g. thromboembolism and intercurrent infections [8].The classical form of GBS is not difficult to recognize and usually presents with the same clinical symptoms and phenotype, which were already described almost a century ago [5]. However, with more clinical research focused on this disease, it became increasingly clear that GBS is not a single entity, but rather a clinically and pathologically heterogeneous group of neuropathic conditions. This concept first emerged subsequent to the description of the Miller Fisher syndrome in 1956, when Charles Miller Fisher published three cases of acute polyneuritis with the clinical triad of ophthalmoplegia, ataxia and areflexia [1], and was further corroborated by and developed following the discovery of pure axonal variants of GBS in northern China in the 1990s [7]. Over the last two decades other, and rarer variants have been added to that list, including subtypes presenting predominantly with dysautonomia, lower cranial nerve involvement or pure brachio-pharyngeal weakness [11, 16, 18]. In the current issue Susuki and colleagues elaborate the clinical features of another variant of the GBS spectrum, which is characterized by prominent facial diplegia and limb paraesthesiae, with no or only minor motor weakness [14]. Ropper delineated this condition in 1994 in his report of four cases that presented with the symptoms of an acute progressive facial diplegia and numbness in the extremities [13]. Based on clinical and serological data collected from about 8,600 patients, Susuki and colleagues have now identified 22 cases, in which clinical symptoms and disease history were compatible with Ropper‘s initial description. Most of these patients presented with numbness in all limbs and weakness of the facial muscles, while only a minority had mild motor deficits in the extremities or ataxia. However, more important than the recognition of symptoms that differ from those of typical GBS is the identification of features supporting the view that facial diplegia and limb paraesthesiae form indeed part of the GBS spectrum. Susuki and colleagues provide here convincing evidence for this concept, since (1) all of the patients described had albuminocytologic dissociation in the cerebrospinal fluid, (2) most cases had a history of antecedent infection and (3) demyelinating abnormalities were detectable in nerve conduction studies [14]. Although GBS is considered to be the most common flaccid neuromuscular paralysis worldwide, it is not very frequent in comparison to other neurological disorders, with an incidence of 1–2 per 100,000 [8–10, 12]. This raises the question how useful is a splitting of such a rare disease into several subtypes, if each comprises only a small percentage of all GBS cases and especially if the absolute numbers for each subtype become very low? Opinions may be divided between ‘‘splitters’’ and ‘‘lumpers’’. First, the recognition of atypical forms is likely to have a strong clinical impact, since it raises the clinician’s H. C. Lehmann H.-P. Hartung (&) Department of Neurology, Heinrich-Heine-University, Moorenstrasse 5, 40225 Dusseldorf, Germany e-mail: hans-peter.hartung@uni-duesseldorf.de

Acute Inflammatory Demyelinating Polyneuropathy and a Unilateral Babinski/Plantar Reflex

Acquired acute demyelinating peripheral polyneuropathy (AADP) is a general classification of pathologies that could affect secondary the peripheral nervous system. They are characterized by an autoimmune process directed towards myelin. Clinically they are characterized by progressive weakness and mild sensory changes. Acute inflammatory demyelinating polyneuropathy often is referred to as Guillain-Barré syndrome (GBS). GBS is the major cause of acute nontraumatic paralysis in healthy people and it is caused by autoimmune response to viral agents (influenza, coxsackie, Epstein-Barr virus, or cytomegalovirus) or bacterial infective organisms (Campylobacter jejuni, Mycoplasma pneumoniae). A detailed history, with symptoms of progressive usually bilateral weakness, hyporeflexia, with a typical demyelinating EMG pattern supports the diagnosis. Progressive affection of respiratory muscles and autonomic instability coupled with a protracted and unpredictable recovery normally results in the need for ICU management. We present a case report of a patient with a typical GBS presentation but with a unilateral upgoing plantar reflex (Babinski sign). A unifying diagnosis was made and based on a literature search in Pubmed appears to be the first described case of its kind.

Incidence and clinical features of acute inflammatory polyradiculoneuropathy in Lombardy, Italy, 1996.

To assess the annual incidence of typical Guillain-Barré syndrome (GBS) and its main variants (atypical GBS) in a well-defined population from a large area. A population-based prospective survey of GBS was undertaken during the calendar year 1996 in Lombardy, Italy (population 8,891,652). Typical and atypical GBS was diagnosed using the National Institutes of Neurological and Communicative Disorders and Stroke (NINCDS) and Ropper criteria. Eligible cases were hospital inpatients traced through a regional registry, the hospital discharge diagnoses, and an ongoing case-control study. Diagnostic and demographic findings were collected for each case. Complete clinical and laboratory features were available for 80% of cases. A total of 138 patients (males 74; females 64) aged 2-91 years fulfilled the diagnostic criteria for typical GBS (128) or atypical GBS (10). GBS variants included Miller-Fisher syndrome (four cases), cranial polyneuritis (three cases), pure motor GBS (two cases), and sensory loss with areflexia (one case). The crude annual incidence of GBS was 1.55 per 100,000 (typical GBS 1.43; atypical GBS 0.11; male 1.67; female 1.43; age <35 years, 0.79; 35-54 years, 1.33; 55-74 years, 3.22; 75+ years, 4.67). The overall rate was 1.58 when age- and sex-adjusted to the 1996 Italian population. Previous infections were reported for 37% of patients. The electrophysiological findings indicated demyelination in 51%, primary axonopathy in 14%, and mixed myelin and axon involvement in 27%. The incidence of typical GBS is comparable with that in other reports using the NINCDS diagnostic criteria. Atypical GBS accounts for a limited number of cases.

Acute oropharyngeal palsy associated with anti-GM1b IgG antibody

Sirs: Although patients with Guillain-Barre syndrome (GBS) typically show generalized muscle weakness with areflexia, a few patients have only regional weakness throughout the disease course. O’Leary et al. [4] described three patients who had rapid progression of localized oropharyngeal weakness with or without ataxia. Unlike typical GBS or Fisher syndrome, these patients never developed limb weakness, ophthalmoplegia, or ptosis; they were regarded as having a regional variant of GBS based on detection of anti-GQ1 b and anti-GT1 a IgG antibodies in serum. Another patient with acute oropharyngeal palsy also was reported to have these antibodies [5]. We treated a patient with acute oropharyngeal palsy associated with an isolated anti-GM1 b IgG antibody. This autoantibody has yet to be reported in patients with regional GBS variants, although in more generalized cases it is regarded as a diagnostic marker of GBS [3]. A 47-year-old woman’s voice developed an increasingly nasal quality over a few days. She had no history of a preceding cough, fever, sore throat or diarrhea. Neurological examination on day 5 of her course showed mild dysarthria and mild weakness of neck flexion (Medical Research Council grade, 4). Palatal elevation was diminished and the gag reflex was absent. Pupil size and reactions were normal. No signs of ophthalmoplegia, ptosis, or facial muscle weakness were found. Limb muscle power and tendon reflexes were normal. The patient could not perform tandem gait, but finger-to-nose and heel-to-knee tests were normal. The Romberg test was negative. No sensory or autonomic disturbances were present. The complete blood count and blood chemistry results were normal. Results of serological tests for Campylobacter jejuni, cytomegalovirus, Mycoplasma pneumoniae, varicella-zoster virus, and herpes simplex virus infections were negative. On day 7, serum IgG antibody titer against GM1 b was found to be elevated (1:2,000; normal, less than 1:500). No IgG antibodies against GM2, GM1, GD1 a, GalNAc-GD1 a, GD1b, GT1 a, GT1 b, or GQ1 b were detected. IgM antiganglioside antibodies were absent. Cerebrospinal fluid obtained on day 7 showed normal protein and cell content. Motor nerve conduction studies on day 7 were normal. A sensory nerve action potential was not evoked in the sural nerve, but sensory nerve action potentials and sensory conduction velocities were normal in the median nerve. Cranial MRI with enhancement was normal. From day 10, the patient was given intravenous immunoglobulin (400 mg/kg/day) for 5 consecutive days. She noted some difficulty in swallowing on day 11, but did not require tube feeding. Dysphagia and dysarthria respectively disappeared by days 14 and 28, and all other neurologic abnormalities had resolved completely 2 months after onset. The anti-GM1 b IgG antibody titer had returned to the normal range (less than 500) 8 months following onset of illness. Our patient experienced acute progression of oropharyngeal palsy as well as mild truncal ataxia and neck weakness. She never showed notable limb weakness, ophthalmoplegia, ptosis, or areflexia. These clinical features are simitar to those of O’Leary’s patients with acute oropharyngeal palsy [4]. However, these patients had antiGQ1 b and anti-GT1 a IgG antibodies, while our patient had an antiGM1 b IgG antibody. Anti-GM1 b antibody is closely related to severe limb weakness and slow recovery in generalized GBS [7]. On the whole, GBS patients with anti-GM1 b antibodies are somewhat less likely to have cranial nerve involvement, although this antibody can be detected in half of GBS patients who show oropharyngeal palsy as an initial symptom [2, 6]. Several case reports suggest that anti-GT1 a IgG antibody, with or without cross-reactivity with GQ1 b, is involved in the pathogenesis of oropharyngeal palsy in GBS [1]. Our case supports an association of oropharyngeal palsy with antiGM1 b antibodies. GQ1 b ganglioside in human cranial nerves is believed to be a target of the humoral autoimmune response in patients with Fisher syndrome or GBS with ophthalmoplegia and anti-GT1 a IgG antibody coexists in these conditions. AntiGQ1 b and anti-GT1 a IgG antibodies have also appeared together in acute ophthalmoparesis, Bickerstaff ’s brain stem encephalitis, and the pharyngeal-cervical-brachial variant of GBS [2]. In addition to anti-GT1 a and anti-GQ1 b antibodies, we feel that anti-GM1 b IgG antibody may be a helpful diagnostic marker for acute oropharyngeal palsy in some patients. Since in GBS anti-GT1 a, anti-GQ1 b, and anti-GM1 b IgG antibodies frequently coexist, acute oropharyngeal palsy may represent an early clinical stage of GBS, pharyngeal-cervical-brachial variant of LETTER TO THE EDITORS

Clinical presentation and outcome of Guillain-Barré and related syndromes in relation to anti-ganglioside antibodies.

We correlated the clinical features of 78 patients with Guillain-Barré syndrome (GBS) or related variants, with the presence of serum antibodies to the gangliosides GM1, GM2, GD1a, GD1b and GQ1b in order to determine whether these antibodies may influence the clinical presentation or outcome of GBS. Sixty-three patients had typical GBS (81%), nine a pure motor form (11%), three a paraparetic form (4%), and three had Miller Fisher syndrome (MFS). IgG or IgM (or both) anti-ganglioside antibodies were found by ELISA in 37% of patients, including 36% with typical, 33% with pure motor and 100% with MFS. Beside the constant occurrence of anti-GQ1b antibodies in patients with MFS (P<0.00001), the other clinical forms were not associated with a specific anti-ganglioside reactivity. Anti-GM1 and anti-GD1a antibodies tended to be associated with a worse disability at 6 month than other or no reactivity and, similarly to anti-GM2 antibodies, with a more frequent respiratory impairment. Anti-GM2 and anti-GD1b antibodies were always associated with typical GBS and, in all but one patient, with a complete recovery; still they were found in only 13 and 3%, respectively, of the patients with this presentation. Anti-GQ1b antibodies, though always associated with ophthalmoplegia and ataxia in both MFS and GBS, were found in only 36 and 26%, respectively, of patients with these symptoms. Even if different anti-ganglioside antibodies tend to be associated with some clinical features possibly suggesting that they may influence the clinical presentation or outcome, with the exception of anti-GQ1b antibodies for ophthalmoplegia and ataxia, they do not permit to predict the clinical presentation or outcome in individual patients.

Neuromuscular blockade by IgG antibodies from patients with Guillain-Barré syndrome: a macro-patch-clamp study.

Guillain-Barré syndrome (GBS) is often associated with serum antibodies to glycoconjugates such as GM1 and GQ1b. The pathogenic role of these antibodies and other serum factors has not yet been clarified. We have investigated the effect of serum, plasma filtrate, and highly purified IgG and IgM from 10 patients with typical GBS on motor nerve terminals in the mouse hemidiaphragm. Quantal endplate currents were recorded by means of a perfused macro-patch-clamp electrode. The plasma filtrate of all GBS patients led to a 5- to 20-fold reduction of evoked quantal release within 7 to 15 minutes of continuous superfusion. In 4 patients, the amplitudes of single quanta were clearly reduced (by 10-66% of control values), indicating an additional postsynaptic action. Blocking effects could be reversed to a variable degree within 15 to 18 minutes after washout. Purified IgG was as effective as native serum, whereas a purified GBS IgM fraction did not block transmission. Sera from convalescent patients and IgG from healthy subjects were without blocking effect. The effects were complement independent and there was no link to the presence (in 6 patients) or absence (in 4 patients) of detectable antibodies to GM1 or GQ1b. In GBS, antibodies to an undetermined antigen depress the presynaptic transmitter release and, in some cases, the activation of postsynaptic channels. We suggest that weakness in the acute stage of GBS may be caused in part by circulating antibodies.