Distal limb weakness variant of Guillain- Barré syndrome: A mild form of acute motor axonal neuropathy after Campylobacter jejuni enteritis

Objective: This study was designed to determine if the presence of specific ganglioside-like moieties in Campylobacter lipopolysaccharides(LPSs) is related to the development of Guillain-Barré syndrome (GBS), and to discover how frequently such moieties, including GM1, are present in these LPSs.Methods: We studied Campylobacter isolates and sera from seven patients with GBS (five acute motor axonal neuropathy, one acute inflammatory demyelinating polyneuropathy, and one Fisher's syndrome), and compared them with similar specimens from patients with Campylobacter enteritis alone.Results: All GBS patients had antiganglioside antibodies. Anti-GM1 and anti-GD1a titers were significantly elevated in post-Campylobacter GBS, both axonal and demyelinating, compared with normal control subjects or those with uncomplicated Campylobacter diarrhea. Campylobacter isolated from patients with GBS and with enteritis alone had similar ganglioside-like moieties.Conclusions: These results indicate that patients who develop GBS respond differently to the ganglioside-like epitopes on Campylobacter than do non-GBS diarrhea patients. Our findings support a role for host susceptibility as a determinant for the outcome following Campylobacter infection. These findings have important implications for the development of vaccines against Campylobacter jejuni.

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Role of macrophages in Guillain-Barré syndrome: contributes with the outcome

American Journal of BioMedicine ◽

10.18081/2333-5106/016-1-11 ◽

2016 ◽

Vol 4 (1) ◽

pp. 1-11

Author(s):

Robert G. Andrews ◽

Yixin Lin, Limin Lee

Keyword(s):

Axonal Neuropathy ◽

Node Of Ranvier ◽

Guillain Barre Syndrome ◽

Limb Weakness ◽

Acute Motor Axonal Neuropathy ◽

Barr Virus ◽

Guillain Barré Syndrome ◽

Mycoplasma Pneumonia ◽

Epstein Barr ◽

Guillain Barré

Guillain-Barré syndrome (GBS) occurs throughout the world with a median annual incidence of 1.3 cases per population of 100 000, with men being more frequently affected than women. GBS is currently divided into the two major subtypes: acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) and clinically is defined as an acute peripheral neuropathy causing limb weakness that progresses over a time period of days or, at the most, up to 4 weeks. Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus and Mycoplasma pneumonia are commonly identified antecedent pathogens. Strong evidence now exists that axonal subtypes of Guillain-Barré syndrome is caused by antibodies to gangliosides on the axolemma that target macrophages to invade the axon at the node of Ranvier, further, macrophages infiltrate peripheral nerves and may contribute to neural damage in the GBS. This review highlights relevant recent publications, particularly on the outcome of macrophages in Guillain-Barré syndrome.

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Finger drop sign—Characteristic pattern of distal weakness in Guillain-Barré Syndrome: A case report and review of the literature

SAGE Open Medical Case Reports ◽

10.1177/2050313x18773649 ◽

2018 ◽

Vol 6 ◽

pp. 2050313X1877364

Author(s):

Yong Chuan Chee ◽

Beng Hooi Ong

Keyword(s):

Case Report ◽

Axonal Neuropathy ◽

Good Prognosis ◽

Characteristic Pattern ◽

Guillain Barre Syndrome ◽

Demyelinating Neuropathy ◽

Limb Weakness ◽

Acute Motor Axonal Neuropathy ◽

Guillain Barré Syndrome ◽

Guillain Barré

Guillain-Barré Syndrome is an acquired acute autoimmune polyradiculoneuropathy that commonly presents with limb weakness and occasional cranial nerve, respiratory and autonomic involvement. Although the classic description of Guillain-Barré Syndrome is that of a demyelinating neuropathy with ascending weakness, predominant bilateral finger drop as presenting feature has rarely been reported. A characteristic pattern of weakness involving the extensor components of the fingers known as “finger drop sign” has been first described to be specific in acute motor axonal neuropathy form of Guillain-Barré Syndrome in the literature. We report a case of acute motor-sensory axonal neuropathy, which showed characteristic pattern of predominant finger extensor weakness, and provide a summary of all reported cases to date. While previous reports suggested that this is a sign that carries good prognosis, our case report suggested otherwise as the patient succumbed to respiratory and autonomic complications. Further studies are needed to evaluate the clinical significance of this peculiar sign.

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Electrophysiology in the Guillain-Barré Syndrome: Study of 30 Cases

Journal of Bangladesh College of Physicians and Surgeons ◽

10.3329/jbcps.v24i2.149 ◽

1970 ◽

Vol 24 (2) ◽

pp. 54-60

Author(s):

NC Kundu

Keyword(s):

Cell Count ◽

Electrophysiological Study ◽

Axonal Neuropathy ◽

Guillain Barre Syndrome ◽

Acute Motor Axonal Neuropathy ◽

Elevated Protein ◽

Guillain Barré Syndrome ◽

A Cell ◽

Guillain Barré

Thirty consecutive patients diagnosed clinically as Guillain Barré Syndrome (GBS) were enrolled in this study to see the electrophysiological patterns of GBS in Bangladeshi community. Among 30 patients, 25 were male (M: F = 5:1) and 47% patients were between 16 and 25 years of age. An antecedent event was present in 67% of patients. An elevated protein was present in 90% of cases and a cell count of up to five was present in 94% of patients. Acute inflammatory demyelinating polyradiculopathy (AIDP) was commonest (33.35%) followed by acute motor axonal neuropathy (AMAN) which constitute 26% of patients in electrophysiological study of the enrolled patients. Acute motor sensory axonal neuropathy constitutes 14% of cases in this series. (J Bangladesh Coll Phys Surg 2006; 24: 54-60)

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Child with Guillain-Barré Syndrome Responding to Plasmapheresis: A Case Report

Case Reports in Acute Medicine ◽

10.1159/000505964 ◽

2020 ◽

Vol 3 (1) ◽

pp. 4-11

Author(s):

Sarmad Al Hamdani ◽

Fatema Yusuf Aljanabi ◽

Maryam Isa Abdulrasool ◽

Alaa Haitham Salman

Keyword(s):

Axonal Neuropathy ◽

Cranial Nerves ◽

Lower Limbs ◽

Guillain Barre Syndrome ◽

Upper Limbs ◽

Acute Motor Axonal Neuropathy ◽

Guillain Barré Syndrome ◽

Foley’S Catheter ◽

Guillain Barré ◽

First Session

Intravenous immunoglobulin (IVIG) has long been regarded as the first-line treatment for Guillain-Barré syndrome (GBS), with plasmapheresis only being reserved for severe cases or used as an additional therapy of unproven efficacy. Here, we present the case of a 9-year-old girl with acute motor axonal neuropathy (AMAN), a rapidly progressive subtype of GBS that caused her to fall into respiratory failure. The patient failed to show a response 10 days after starting IVIG, but showed rather quick improvement with plasmapheresis. She received a total of 5 sessions of plasmapheresis on alternate days over a course of 8 days. Before starting plasmapheresis, her muscle strength was 2/5 in both upper limbs and 1/5 in both lower limbs, and she was dependent on mechanical ventilation. Following the first session, her power improved from 2/5 to 4/5 in the upper limbs, and the gag and sucking reflexes were recovered. On day 3, after the second session was initiated, she was extubated successfully (having been on a ventilator for 2 weeks) and remained on continuous positive airway pressure for the next 48 h, after which she was on room air. In addition, she was having hypertension from the first day of the diagnosis (which was due to autonomic instability), which improved after clonidine to maintain her blood pressure. She was also initially having urinary retention, then was off Foley’s catheter. The patient was discharged from the hospital 2 weeks following the first session of plasmapheresis, with power grade 4/5 in both her upper and lower limbs. Her cranial nerves had recovered fully, and she was able to walk with aids.

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Acute Motor Axonal Neuropathy in a 5-Month-Old Child

Journal of Pediatric Neurology ◽

10.1055/s-0039-1698816 ◽

2019 ◽

Vol 18 (03) ◽

pp. 171-174

Author(s):

Federica Sullo ◽

Milena Motta ◽

Pierluigi Smilari ◽

Luigi Rampello ◽

Filippo Greco ◽

...

Keyword(s):

Axonal Neuropathy ◽

Male Infant ◽

Guillain Barre Syndrome ◽

Acute Motor Axonal Neuropathy ◽

Fisher Syndrome ◽

Acute Inflammatory Demyelinating Polyneuropathy ◽

Guillain Barré Syndrome ◽

Guillain Barré ◽

Prior Infection ◽

Prevalent Form

AbstractGuillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy characterized by rapidly progressive, essentially symmetric weakness and areflexia in a previously otherwise healthy child. It is the most common cause of acute flaccid paralysis in children, and its reported incidence is 1 to 2/100,000 population. Prior infection is a well-established predating event in GBS. The commonly recognized variants of GBS are acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy, and Miller–Fisher syndrome. AIDP is the most prevalent form. As Guillain–Barrè syndrome represents an important differential diagnosis in infancy with pronounced and progressive hypotonia, we herein report a case of AMAN in a 5-month-old male infant without known exposure to immunomodulating factors or infections.

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An overlapping case of Bickerstaff brainstem encephalitis and acute motor axonal neuropathy variant of Guillain-Barre syndrome associated with Systemic Lupus Erythematosus

Polish Archives of Internal Medicine ◽

10.20452/pamw.4371 ◽

2018 ◽

Cited By ~ 1

Author(s):

Paweł Wańkowicz ◽

Przemysław Nowacki ◽

Marek Brzosko ◽

Danuta Bobrowska-Snarska

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Axonal Neuropathy ◽

Guillain Barre Syndrome ◽

Brainstem Encephalitis ◽

Systemic Lupus ◽

Acute Motor Axonal Neuropathy ◽

Guillain Barré Syndrome ◽

Guillain Barré ◽

Bickerstaff Brainstem Encephalitis

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The acute motor-sensory axonal neuropathy variant of Guillain-Barré syndrome after thoracic spine surgery

Journal of Neurosurgery Spine ◽

10.3171/2011.8.spine1159 ◽

2011 ◽

Vol 15 (6) ◽

pp. 605-609 ◽

Cited By ~ 13

Author(s):

Jocelyn Cheng ◽

D. Ethan Kahn ◽

Michael Y. Wang

Keyword(s):

Spine Surgery ◽

Thoracic Spine ◽

Axonal Neuropathy ◽

Guillain Barre Syndrome ◽

Acute Motor Axonal Neuropathy ◽

Fisher Syndrome ◽

Guillain Barré Syndrome ◽

Classic Form ◽

Guillain Barré ◽

New Onset

Guillain-Barré syndrome (GBS) is the eponym used to describe acute inflammatory polyradiculoneuropathies, which manifest with weakness and diminished reflexes. Although the classic form of GBS is considered to be an ascending demyelinating polyneuropathy, several variants have been described in the literature, including the Miller-Fisher syndrome, acute panautonomic neuropathy, acute motor axonal neuropathy, and acute motor-sensory axonal neuropathy (AMSAN). Few cases of postoperative GBS have been documented, particularly for the AMSAN variant. The authors describe the case of a patient who developed AMSAN after thoracic spine surgery and highlight the importance of investigating new-onset weakness in the postoperative period.

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Electrophysiological patterns in patient with Guillain-Barre syndrome

BIRDEM Medical Journal ◽

10.3329/birdem.v12i1.57220 ◽

2021 ◽

Vol 12 (1) ◽

pp. 16-21

Author(s):

SM Monowar Hossain ◽

Zahed Ali ◽

Mohammad Motiur Rahman ◽

Md Aolad Hossain ◽

Pallab Kanti Saha ◽

...

Keyword(s):

Nerve Conduction Study ◽

Nerve Conduction ◽

Axonal Neuropathy ◽

Clinical Findings ◽

Guillain Barre Syndrome ◽

Acute Motor Axonal Neuropathy ◽

Cross Sectional ◽

Female Ratio ◽

Guillain Barré Syndrome ◽

Guillain Barré

Background: Guillain-Barre syndrome (GBS) is an acute, frequently severe and fulminant polyradiculoneuropathy that is autoimmune in nature. Incidence and predominant subtypes of GBS differ geographically. Electrophysiology has important role in subtyping GBS. This study aimed to evaluate the electrophysiological findings in patient of GBS. Methods: This was a hospital based cross-sectional descriptive study and conducted at the Department of Neurology in Sir Salimullah Medical College & Mitford Hospital, Dhaka and National Institute of Neurosciences and Hospital, Dhaka during July 2017 to June 2018. Clinically diagnosed 53 patients with GBS were enrolled according to prefixed selection criteria. Detail history taking, clinical examination, nerve conduction study and cerebrospinal fluid (CSF) examination was performed in all cases. Clinical findings, nerve conduction study (NCS) parameters, CSF findings and demographic profiles were evaluated. Results: Mean ± SD age of presentation was 41.64 (±14.56) years and median age was 42.0 years. There were total 33(62 %) males and 20 (38 %) females with male: female ratio of 1.7:1. Clinically two-thirds(62.3%) of patients had both upper and lower limb involvement (62.3%), facial weakness was in 32.1% and 13.2% had bulbar involvement. Acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN)were found to be 51%, 32% and 17% respectively. CSFprotein was elevated in most of the patients with a range of 16-725 mg/dl. Highest CSF protein was found in AIDP. Conclusion: Electrophysiological studies play an important role in the early detection; characterization of GBS.In this study, the commonest type of GBS was AIDP. Higher levels of CSF protein, absent H-reflex and Fresponse, sural sparing and unexcitable nerves are more frequently present in AIDP. BIRDEM Med J 2022; 12(1): 16-21

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