Pharyngeal-cervical-brachial Research Articles

When Guillain-Barre syndrome took a wrong path

The pharyngeal cervical brachial (PCB) variant of Guillain-Barre-syndrome (GBS) is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in upper limbs. On nerve conduction study PCB represents a localized subtype of GBS characterized by axonal rather than demyelinating neuropathy. PCB is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. Herein we describe a case of symmetric descending type of acute flaccid paralysis with predominant upper limb involvement and also lower limb involvement diagnosed as a PCB- GBS overlap mainly based on nerve conduction study.

Recurrent Guillain-Barré syndrome presenting as pharyngeal-cervical-brachial variant with three species of ganglioside antibodies:case report

BackgroundGuillain-Barré syndrome (GBS) is generally considered to be monophasic, and recurrent GBS (RGBS) is very rare. Pharyngeal-cervical-brachial (PCB) is a less common variant of GBS. There have been no cases reported describing RGBS showing different phenotype presenting as PCB variant with three species of ganglioside antibodies.Case presentation We report a case of a 77-year-old female patient with GT1a, GD1a and sulfatide-seropositive PCB-GBS after prior episode of AMAN-GBS 13 years ago. Our patient showed oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs and partially improved after 5 days of IVIG and physiotherapy.ConclusionThis study reports a rare case characterized as recurrent GBS after a long period, showing different phenotypes in different episodes with three different species of ganglioside antibodies. Further studies are required to obtain better understanding of RGBS and PCB variant.

A Diagnostic Mimicker: A Sixty-Year-Old Male With Ptosis and Left Arm Weakness

We describe a case of a 60-year-old male presenting with ptosis and left arm weakness. We review key localization features, diagnostic studies, including electromyography (EMG) findings, and discuss treatment and prognosis of the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barre syndrome. We explore the differential diagnoses from motor neuron disease, neuromuscular junction disorder, and other atypical Guillain-Barre syndromes that this condition can mimic and how to recognize the PCB variant in the neurologic evaluation. J Neurol Res. 2022;12(1):21-24 doi: https://doi.org/10.14740/jnr605

Pharyngeal-cervical-brachial variant of Landry Guillain Barre syndrome after dengue infection: a case report

Dengue is a common mosquito-borne infection in India. We reported a rare Pharyngeal cervical brachial (PCB) variant of Landry Guillain Barre syndrome (LGBS) associated with the dengue virus infection. The pathogenesis seems to be molecular mimicry between gangliosides and microbial lipo-oligosaccharides. PCB usually presents with oropharyngeal or cervicobrachial weakness. Therefore, it must be recognised early and distinguished from conditions presenting with cephalocaudal progressing weakness, such as Myasthenia Gravis, Miller-Fisher syndrome, botulism, diphtheria, porphyria or brain stem stroke. The aim of the study was to add to the limited literature on the PCB variant of LGBS after dengue infection and shed some light on presentation and management options for this rare entity.

Pharyngeal–Cervical-Brachial Variant: A Rare Form of Guillain–Barre Syndrome in a Paediatric Patient

This case report describes a successfully managed rare variant of Guillain-Barre´ Syndrome (GBS), the pharyngeal-cervical-brachial (PCB) variant, in a 5-year-old child which is characterized by the involvement of oropharyngeal, neck and upper limb muscles, without disturbed consciousness or ataxia. Since early treatment and supportive care yield successful outcome, it is important for the treating physician to be familiar with other diseases that mimic the disease and variants for prompt and accurate diagnosis.

Determining the Utility of the Guillain-Barré Syndrome Classification Criteria.

Background and PurposeSeveral variants of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) exist, but their frequencies vary in different populations and do not always meet the inclusion criteria of the existing diagnostic criteria. However, the GBS classification criteria by Wakerley and colleagues recognize and define the clinical characteristics of each variant. We applied these criteria to a GBS and MFS cohort with the aim of determining their utility.MethodsConsecutive GBS and MFS patients presenting to our center between 2010 and 2020 were analyzed. The clinical characteristics, electrophysiological data, and antiganglioside antibody profiles of the patients were utilized in determining the clinical classification.ResultsThis study classified 132 patients with GBS and its related disorders according to the new classification criteria as follows: 64 (48.5%) as classic GBS, 2 (1.5%) as pharyngeal-cervical-brachial (PCB) variant, 7 (5.3%) as paraparetic GBS, 29 (22%) as classic MFS, 3 (2.3%) as acute ophthalmoparesis, 2 (1.5%) as acute ataxic neuropathy, 2 (1.5%) as Bickerstaff brainstem encephalitis (BBE), 17 (12.9%) as GBS/MFS overlap, 4 (3%) as GBS/BBE overlap, 1 (0.8%) as MFS/PCB overlap, and 1 (0.8%) as polyneuritis cranialis. The electrodiagnosis was demyelinating in 55% of classic GBS patients but unclassified in 79% of classic MFS patients. Anti-GM1, anti-GD1a, anti-GalNAc-GD1a, and anti-GD1b IgG ganglioside antibodies were more commonly detected in the axonal GBS subtype, whereas the anti-GQ1b and anti-GT1a IgG ganglioside antibodies were more common in classic MFS and its subtypes.ConclusionsMost of the patients in the present cohort met the criteria of either classic GBS or MFS, but variants were seen in one-third of patients. These findings support the need to recognize variants of both syndromes in order to achieve a more-complete case ascertainment in GBS.

Post-Vaccination Pharyngeal-Cervical-Brachial Variant of Guillain-Barré Syndrome

The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome (GBS) is very rare. It is characterized by weakness of the upper extremities associated with bulbar symptoms and facial diplegia. Documented cases were post-infectious, a post-vaccination occurrence has not been documented in the available literature. Even rarer is the occurrence of any variant of GBS following the mumps measles rubella (MMR) vaccine. The neurophysiological hallmark of PCB variant of GBS is a combination of myelinopathy and axonopathy, hence, its consideration as a subtype of the acute motor axonal neuropathy (AMAN) variant. It should be suspected in any case of acute-onset flaccid symmetrical weakness of the upper extremities, as early diagnosis and treatment are key to preventing fatal bulbar weakness. Here we report a case of a middle-aged man, who presented with features of PCB a fortnight after being vaccinated for MMR.

Pharyngeal-Cervical-Brachial (PCB) variant of Guillain Barre Syndrome

The Pharyngeal- Cervical-Brachial (PCB) variant of Guillain- Barre Syndrome (GBS) is a rare presentation, characterized by marked weakness of the oro- pharynx, neck and proximal upper limbs sparing power and reflexes of the lower limbs. It presents as rapid progressive pharyngeal weakness and often needs intubation and ventilation due to excessive secretions. Diagnosis is supported by high CSF protein and absent F waves in upper limbs in electro-physiological studies. Diagnosis can be confirmed by doing ant DT1a antibodies. We present the case report of a 6-year-old patient who presented with progressive difficulty in swallowing due to oro-pharyngeal muscle weakness.

Pharyngeal-cervical-brachial syndrome: A rare form of Guillain-Barré syndrome with severe acute bulbar palsy

Guillain-Barré syndrome is clinically characterized by acute onset of generalized, symmetrical, and ascending muscle weakness and areflexia from peripheral nerve involvement. We report a rare case of pharyngeal-cervical-brachial (PCB) variant with predominant bulbar symptoms We describe the case of 39-year-old man presented with acute onset of dysarthria. A few hours later, he developed severe dysphagia as well dysphonia and nasal regurgitation. His initial sensory and motor examinations were normal. Over the next few days of hospitalization, he developed a mildly ataxic gait. Sensory examination showed deep sensory disturbance. Reflexes were later depressed. Routine biochemical tests and brain/ orbit magnetic resonance imaging were normal. PCB variant of GBS is an infrequent disease (approximately 2–4% of GBS cases) that can be misdiagnosed as other neurological disease. Electrophysiological studies and CSF analysis were initially normal, but according to the most recent diagnostic criteria for GBS, evidence of neuropathy and/or CSF albuminocytological dissociation are not required but rather considered to be supportive feature. Repeated EMG showed signs of demyelinating polyneuropathy and immunoglobulin 0.4 g/kg/day was started with good response. In conclusion, PCB weakness should always be considered in patients presenting with multiple cranial nerve palsies. Early recognition of this unusual variant of Guillain-Barré syndrome is important in order to enable appropriate electrodiagnostic testing, anticipation of potential complications, and institution of immunomodulatory therapy where appropriate.

PHARYNGEAL-CERVICAL-BRACHIAL VARIANT: AN UNUSUAL PRESENTATION OF GUILLAIN-BARRÉ SYNDROME

Pharyngeal-cervical-brachial (PCB) variant is a rare presentation of Guillain–Barre syndrome (GBS), which is symbolized by oroparhyngeal, cervical and upper limb weakness. It is often wrongly diagnosed as brainstem stroke, myasthenia gravis or botulism. The presence of anti-GT1a and anti-GQ1b ganglioside IgG antibodies and axonal electrophysiological pattern are the hallmark of PCB variant. The treatment is similar to any other variant of GBS. We present here, report of a 20-year-old male, who presented with findings suggestive of PCB variant of GBS but had absent anti-GT1a and anti-GQ1b antibodies and a normal cerebrospinal fluid. He made a remarkable recovery after administration of intravenous immunoglobulins.

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

ObjectivesThis study aimed to evaluate the clinical and electrophysiological characteristics of various distinctive classical and localised Guillain-Barré syndrome (GBS) subtypes. Patients and methodsClinical 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. ResultsAll 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. ConclusionPhenotype recognition based on new GBS clinical classification, supported by electrodiagnostic study permits more precise clinical subtypes determination and outcome prognostication.

Acute bulbar palsy as a variant of Guillain-Barré syndrome.

To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barré syndrome (GBS) and differentiate it from Miller Fisher syndrome (MFS) and pharyngeal-cervical-brachial (PCB) variants. We analyzed cases of ABP without limb motor weakness based on a dataset containing clinical information and the results of antiganglioside antibodies assays for acute immune-mediated neuropathies. Eleven cases with an age at onset ranging from 18 to 65 years (mean 33.8 years) were identified as ABP-plus syndrome. All of the enrolled cases manifested with ABP as the predominant symptom, and with no limb weakness. The following features accompanied ABP in order of decreasing frequency: ophthalmoplegia (n = 9, 82%), ataxia (n = 9, 82%), and facial palsy (n = 6, 55%). An enzyme-linked immunosorbent assay study disclosed that immunoglobulin G (IgG) anti-GT1a antibodies were the most frequent (n = 11), followed by IgG anti-GQ1b antibodies (n = 6). We propose that ABP-plus syndrome without neck or limb weakness is a variant of GBS that is distinct from the MFS and PCB variants. The presence of IgG anti-GT1a antibodies can explain the relationships between the distinct clinical characteristics and the underlying pathomechanisms.

Le syndrome pharyngo-cervico-brachial : un tableau de syndrome de Guillain-Barré atypique avec paralysie bulbaire sévère

The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is rare in children. It is characterized by oropharyngeal, neck, and upper limb muscle involvement, without ataxia and disturbed consciousness. Although associated with anti-GT1a antibodies, there is no single clinical or serological marker of PCB syndrome. We report on two cases in a 14-year-old and a 15-year-old females. The first symptom was acute dysphonia followed by severe bulbar palsy with deglutition disorders, associated with involvement of other cranial nerves and arm and leg weakness. One of the girls had normal deep tendon reflexes. Both had normal cerebral imaging and normal cerebrospinal fluid. No sign of neuropathy was found on nerve conduction studies. The diagnosis of PCB syndrome was established based on the presence of antiganglioside antibodies. Both adolescents had IgG anti-GT1a antibodies. Anti-GQ1b and anti-GT1b antibodies were associated in the first case, anti-GM1 and anti-GD1a in the second case. Clinical improvement was fast after treatment with intravenous immunoglobulin therapy. Recovery was complete. Only a few cases of children and adolescents with PCB syndrome have been reported. The main differential diagnoses were excluded with brain MRI. The neurophysiological findings in PCB syndrome are axonal neuropathy rather than demyelinating neuropathy, which might be normal in the early stages of the disease. Positivity of anti-GT1a IgG antibodies is very helpful for the diagnosis of PCB syndrome. In atypical cases of bulbar palsy with other cranial nerve involvement and normal brain MRI, diagnosis of PCB syndrome should be considered. Recognizing the atypical cases of Guillain-Barré syndrome enables anticipatory monitoring for disease complications and identifies therapeutic options. The short- and long-term outcome of the PCB syndrome after intravenous immunoglobulin treatment seems favorable.

Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome

Investigators from National University Hospital, Singapore, review the clinical features of 13 cases of pharyngeal-cervical-brachial (PCB) variant of Guillain-Barre syndrome (GBS) and outline new diagnostic criteria.

A Rare Form of Guillan Barre Syndrome: A Child Diagnosed with Anti-GD1a and Anti-GD1b Positive Pharyngeal-Cervical-Brachial Variant.

Pharyngeal-cervical-brachial (PCB) variant is a rare form of Guillan-Barre Syndrome (GBS). Antibodies against other membrane proteins like GM1b and GD1a have been found only in a small number of patients with Guillan Barre syndrome variant. Here, we report a 5.5 year-old boy diagnosed early with positive GD1a and GD1b gangliosides of Guillan-Barre syndrome pharyngeal cervical-Brachial variant, who improved and recovered fully in a short period. This is in contrast to those whose recovery period prolongs in spite of early diagnosis and appropriate treatment and/or those who experience incomplete recovery. In summary, diagnosis of PCB variant of GBS should be considered in infants with sudden onset bulbar symptoms and muscle weakness, and it should be kept in mind that early diagnosis and appropriate treatment can give successful outcomes.

Treatment guidelines for Guillain-Barré Syndrome

Guillain–Barre syndrome (GBS) is an acute onset, usually monophasic immune-mediated disorder of the peripheral nervous system. The term GBS is often considered to be synonymous with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), but with the increasing recognition of variants over the past few decades, the number of diseases that fall under the rubric GBS have grown to include axonal variants and more restricted variants, such as Miller Fisher syndrome (MFS) [Table 1].[1] Table 1 Guillain–Barre syndrome—clinical variants Epidemiology The reported incidence rates for GBS are 1–2 per 100,000 population.[2–4] The lifetime likelihood of any individual acquiring GBS is 1:1000.[5] The subtypes of GBS have different incidence rates in different parts of the world. In Europe and North America AIDP is dominant contributing to 90% of the cases. In contrast in China and Japan AMAN being the most common subtype.[6,7] The picture is intermediate when we look at other population. In Indian series the incidence of AIDP and AMAN are virtually equal although AMAN is more common in younger patients.[8] There seems to be a slight preponderance of AIDP in studies by Gupta et al[9] and by Meena et al (unpublished data from NIMS, Hyderabad). Available Indian literature indicates a peak incidence between June–July and Sept–October.[10] In western countries, GBS is common in the 5th decade,[11] but in India it occurs more commonly at a younger age.[10,12] GBS is equally common in men and women and can occur at any age. There is a male preponderance among the hospitalized population.[10,12]

Severe Outcome of Pharyngeal-Cervical-Brachial Pure Motor Axonal Neuropathy

We present two further cases of the pharyngeal-cervical-brachial (PCB) form of GBS, with unfavourable outcome, showing dramatic dissociation between upper and lower body Symptoms. Both patients showed rapidly progressive motor denervation with disappearance of Compound Muscle Action Potentials (CMAPs) in upper limbs muscles. Sensory Nerve Action Potentials (SNAPs) were instead normal. Normal reflexes, F waves and action potentials were elicited in lower limbs. Despite i.v. Immunoglobulin treatment no recovery was observed and both patients died within a year from onset of symptoms.

Optic neuritis and palatal dysarthria as presenting features of post-infectious GQ1b antibody syndrome

A 31-year-old man had optic neuritis 2 weeks after a diarrheal illness, followed by several deficits including palatal dysarthria, diplopia, ataxia, sensory dysfunction, and mild dysautonomia. Brain MRI and CSF were normal. Nerve conduction studies were initially normal and subsequently showed mild reduction in sensory amplitudes. Anti-GQ1b IgG titer was positive. Deficits resolved after treatment with IVIg. This clinical constellation represents an overlap between Miller Fisher syndrome (MFS) and the pharyngeal–cervical–brachial (PCB) variant of Guillain-Barre syndrome (GBS), along with the infrequently reported central feature of optic neuritis. Campylobacter jejuni enteritis may have triggered the syndrome by molecular mimicry. GQ1b antibodies are associated with MFS, GBS, Bickerstaff brainstem encephalitis and PCB; they form an overlapping spectrum of features, hence the anti-GQ1b syndrome.