The “Child in the Barrel Syndrome” – Severe Pharyngeal-cervical-brachial Variant of Guillain-Barre Syndrome in a Toddler

Rousseff, Rossen Т.; Khuraibet, Adnan J.; Neubauer, David

doi:10.1055/s-0029-1202768

Cited by 6 publications

(3 citation statements)

References 11 publications

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“…There can be difficulty in distinguishing between conduction block and axonal degeneration (21,22). There are other GBS variants with specific restricted phenotypes; as well as Fisher syndrome (FS), a syndrome of ophthalmoplegia, ataxia and areflexia (23), there is a phenotype of isolated facial diplegia (24) and a pharyngeal-cervical-brachial variant (25).…”

Section: Gbs and Variantsmentioning

confidence: 99%

Sex differences in Guillain Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and experimental autoimmune neuritis

et al. 2022

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Guillain Barré syndrome (GBS) and its variants, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP and its variants, are regarded as immune mediated neuropathies. Unlike in many autoimmune disorders, GBS and CIDP are more common in males than females. Sex is not a clear predictor of outcome. Experimental autoimmune neuritis (EAN) is an animal model of these diseases, but there are no studies of the effects of sex in EAN. The pathogenesis of GBS and CIDP involves immune response to non-protein antigens, antigen presentation through non-conventional T cells and, in CIDP with nodopathy, IgG4 antibody responses to antigens. There are some reported sex differences in some of these elements of the immune system and we speculate that these sex differences could contribute to the male predominance of these diseases, and suggest that sex differences in peripheral nerves is a topic worthy of further study.

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Section: Gbs and Variantsmentioning

confidence: 99%

Sex differences in Guillain Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and experimental autoimmune neuritis

et al. 2022

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“…His voice improved dramatically and he could swallow food with no nasal regurgitation. PBC variant of GBS should be considered in patients with bulbar, cervical and upper limb weakness with predominantly persistent lower motor neuron signs [6].…”

Section: Discussionmentioning

confidence: 99%

Acute pharyngeal-cervical-brachial type of Guillain-Barré syndrome, masquerading brainstem stroke in a child

Syed

2015

J Pediatr Neurol

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Guillain-Barr? syndrome (GBS) manifests clinically as an acute onset of generalized symmetrical and ascending weakness of the limb muscles with areflexia. Several variant forms of the disease with unusual distribution of muscles involvement have been described. Pharyngeal-brachial-cervical form of the disease is one of rare variants of GBS and only a few of cases have been reported in children. Here we report a 13-month-old boy, who developed acute weakness of pharyngeal, bulbar, cervical and upper limb weakness and the initial diagnostic work up were done to rule out acute brain stem stroke. We concluded that, while taking all other etiologies into consideration, pharyngeal-cervical-brachial variant of GBS should be considered in patient's symptoms with acute bulbar and upper extremity weakness in order to institute appropriate early management.

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“…Several clinical reports have revealed the different variants of GBS. Pharyngeal-cervical-brachial (PCB) variant of GBS is characterized by muscle weakness involving oropharyngeal, neck, and upper extremity muscles, and was first reported by Ropper in 1986 [4][5]. Many physicians and neurologists are unfamiliar about PCB variant of GBS, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism.…”

Section: Introductionmentioning

confidence: 99%

Pharyngeal–Cervical–Brachial variant of Guillian–Barre Syndrome in Children

Pradhan

Yadav²,

Yadav

2020

Cureus

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Guillian-Barre Syndrome (GBS) typically presents as symmetrical ascending flaccid muscle weakness with areflexia, and with or without sensory symptoms. However, some patients may present atypically, and accordingly, different variants of GBS have been reported in the literature. Pharyngeal-cervical-brachial variant is one of the rare variants and is characterized by muscle weakness extending from the oropharyngeal and neck area to the proximal upper extremities. Many physicians and neurologists are unfamiliar about pharyngeal-cervicalbrachial variant, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. Herein, we report a case of pharyngeal-cervical-brachial variant of GBS. To the best of our knowledge, this is the first reported case of pharyngeal-cervical-brachial variant of GBS in children from Nepal. A 14-year-old Asian male presented with weakness of bilateral upper limb, dysphagia, and nasal intonation of voice. A diagnosis of pharyngeal-cervical-brachial variant of GBS was made after excluding all other possible differentials and based on cerebrospinal fluid analysis and nerve conduction study. The patient improved following conservative management. Pharyngeal-cervical-brachial variant of GBS should always be considered in any patient presenting with symmetrical upper limb weakness and bulbar palsy. This is to ensure early diagnosis, treatment, and follow-up of the potential complications.

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The “Child in the Barrel Syndrome” – Severe Pharyngeal-cervical-brachial Variant of Guillain-Barre Syndrome in a Toddler

Cited by 6 publications

References 11 publications

Sex differences in Guillain Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and experimental autoimmune neuritis

Sex differences in Guillain Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and experimental autoimmune neuritis

Acute pharyngeal-cervical-brachial type of Guillain-Barré syndrome, masquerading brainstem stroke in a child

Pharyngeal–Cervical–Brachial variant of Guillian–Barre Syndrome in Children

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