Case 23

Fisher Syndrome ◽

Immune Mediated ◽

Diagnostic Sensitivity And Specificity ◽

Guillain-Barré syndrome is the prototype of acute immune-mediated neuropathies. Guillain-Barré syndrome has several subtypes including acute inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, and acute motor sensory axonal neuropathy. Guillain-Barré syndrome has also several variants including Miller Fisher syndrome, ataxic form, and pharyngeal–cervical–brachial form. This case highlights the clinical findings in Guillain-Barré syndrome and discusses in details the diagnostic criteria that are essential in confirming the diagnosis and excluding mimickers of the disorder. This is followed by a detailed discussion on the electrodiagnostic findings in Guillain-Barré syndrome during the acute presentation and recovery phase. The diagnostic sensitivity and specificity of the various findings seen on nerve conduction studies are included.

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 ◽

Fisher Syndrome ◽

Acute Inflammatory Demyelinating Polyneuropathy ◽

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.

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 ◽

Fisher 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.

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 ◽

Cross Sectional ◽

Female Ratio ◽

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

Atypical Clinical Presentations of Pediatric Acute Immune-Mediated Polyneuropathy

Journal of Child Neurology ◽

10.1177/0883073818825213 ◽

2019 ◽

Vol 34 (5) ◽

pp. 268-276 ◽

Cited By ~ 2

Author(s):

Naama Yosha-Orpaz ◽

Sharon Aharoni ◽

Malcolm Rabie ◽

Yoram Nevo

Keyword(s):

Axonal Neuropathy ◽

Significant Proportion ◽

Respiratory Muscles ◽

Clinical Findings ◽

Guillain Barre Syndrome ◽

Presenting Symptoms ◽

Tertiary Center ◽

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. During the acute phase, the disorder can be life-threatening by involving the respiratory muscles and the autonomic nervous system. Nevertheless, the prognosis is good, and most children achieve full recovery. The aim of this study was to characterize the clinical and electrophysiologic findings in children with Guillain-Barré syndrome referred to a tertiary center in Israel. A retrospective database review from 2009 to 2015 identified 39 children. Data on clinical presentation, respiratory complications, and long-term neurologic outcomes were collected. Atypical clinical findings at admission included asymmetric weakness in 23%, nonascending weakness in 30%, and normal deep tendon reflexes in 28%. Eight children were later diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Electrophysiologic findings, available in 12 patients with Guillain-Barré syndrome, revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 4 (33.5%), AIDP with secondary axonal changes in 3 (25%), and acute motor axonal neuropathy (AMAN) subtype in 4 (33.5%); 8% had no abnormal findings. On follow-up, 71% of the children with Guillain-Barré syndrome fully recovered compared to 14% of the children with CIDP. Corresponding rates of neurologic sequelae were 29% and 86%. Clinicians should be alert to the atypical presenting symptoms of Guillain-Barré syndrome, which occur in a significant proportion of children.

A Predictive Model for Guillain-Barré Syndrome Based on Single Learning Algorithms

Computational and Mathematical Methods in Medicine ◽

10.1155/2017/8424198 ◽

2017 ◽

Vol 2017 ◽

pp. 1-9 ◽

Cited By ~ 2

Author(s):

Juana Canul-Reich ◽

Juan Frausto-Solís ◽

José Hernández-Torruco

Keyword(s):

Predictive Model ◽

Axonal Neuropathy ◽

Classification Performance ◽

Guillain Barre Syndrome ◽

Fisher Syndrome ◽

Adequate Treatment ◽

Subtype Identification ◽

Background. Guillain-Barré Syndrome (GBS) is a potentially fatal autoimmune neurological disorder. The severity varies among the four main subtypes, named as Acute Inflammatory Demyelinating Polyneuropathy (AIDP), Acute Motor Axonal Neuropathy (AMAN), Acute Motor Sensory Axonal Neuropathy (AMSAN), and Miller-Fisher Syndrome (MF). A proper subtype identification may help to promptly carry out adequate treatment in patients. Method. We perform experiments with 15 single classifiers in two scenarios: four subtypes’ classification and One versus All (OvA) classification. We used a dataset with the 16 relevant features identified in a previous phase. Performance evaluation is made by 10-fold cross validation (10-FCV). Typical classification performance measures are used. A statistical test is conducted in order to identify the top five classifiers for each case. Results. In four GBS subtypes’ classification, half of the classifiers investigated in this study obtained an average accuracy above 0.90. In OvA classification, the two subtypes with the largest number of instances resulted in the best classification results. Conclusions. This study represents a comprehensive effort on creating a predictive model for Guillain-Barré Syndrome subtypes. Also, the analysis performed in this work provides insight about the best single classifiers for each classification case.

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 ◽

Elevated Protein ◽

A Cell ◽

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)

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 ◽

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.

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 ◽

Bickerstaff Brainstem Encephalitis

Guillain Barré ◽

A case of guillain-barré syndrome associated with post-covid infection during 2nd wave of coronavirus in india

Indian Journal of Case Reports ◽

10.32677/ijcr.v7i8.2982 ◽

2021 ◽

pp. 342-344

Author(s):

Anil Kumar Behera ◽

Syed Naqueeb ul Hassan ◽

Chaitanya Challa ◽

K Divya Madhusudhan Reddy ◽

V Bharat R Bhardwaj ◽

...

Keyword(s):

Axonal Neuropathy ◽

Inflammatory Cells ◽

Multiple Organ ◽

Guillain Barre Syndrome ◽

Polymerase Chain Reaction Test ◽

Immune Mediated ◽

Organ Systems ◽

Patient Reported ◽

The novel coronavirus-COVID-19 was detected in Wuhan city of Hubei Province in China on December 31, 2019. Coronaviruses are known to infect multiple organ systems, with respiratory complications being the most obvious symptoms. Although, neurological manifestations are quite rarely reported in cases of COVID-19. In this report, we present the case of a 57-year-old male patient reported with complaints of acute progressive symmetric quadriparesis and recently recovered from COVID-19. Two weeks prior to hospitalization, the patient suffered from cough and fever. The reverse transcriptase-polymerase chain reaction test for COVID-19 infection was positive. Electrodiagnostic tests showed that the patient had acute motor and sensory axonal neuropathy variant of Guillain-Barré Syndrome (GBS). COVID-19 virus stimulates the inflammatory cells and as a result, creates immune-mediated processes. GBS is an immune-mediated disorder. It is not clear whether COVID-19 infection induces the production of antibodies against specific gangliosides. Further investigations should be conducted in regards to the mechanism of GBS in patients with COVID-19 in the future.