Acute Motor Axonal Neuropathy Research Articles

Pattern of Autonomic Involvement in Adult Patients with Guillain Barre Syndrome in a Tertiary Hospital

Background: Guillain Barre Syndrome (GBS) is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis. Autonomic dysfunction is one of the important manifestations of GBS which may lead to significant morbidity and mortality. Objective: The aim of the study is to assess the autonomic involvement, to determine its frequency and pattern of involvement in adult patients with GBS Methods: An observational, descriptive, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS and 35 apparently healthy controls were recruited as the study population. On the basis of nerve conduction study patients were classified into different groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) and other variants. Disability status at the time of autonomic testing was measured by Hughes functional grading scale. The following tests of autonomic nervous system were performed in both patients and controls 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire. Results: The mean age of patients was 35±12 years (range18 to 65 years) and 58.1% were male. Around 88.4 % of patients showed some sort of autonomic dysfunction. Variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. Among them 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (2.7%), sinus arrhythmia (4.7%), constipation (30%), urinary retention (7%) and urinary incontinence (4.7%). Conclusion: In this study different patterns of autonomic dysfunction was found in 88.4% of patients with GBS involving both sympathetic and parasympathetic components. The present study found no significant association between autonomic dysfunction and motor disability scores. Thus autonomic function assessment is essential in every patient with GBS in addition to motor & sensory function. Bangladesh Medical Res Counc Bull 2022; 48(3): 174-179

Post‐COVID‐19 vaccination Guillain–Barré syndrome with sensory ataxia, gaze‐evoked nystagmus, mental‐status change and positive pathological reflex

AbstractBackgroundVarious neurological disorders have been reported after vaccination against coronavirus disease 2019, one of which is Guillain–Barré Syndrome (GBS).Case PresentationWe report a case of a 73‐year‐old woman who developed GBS and extra‐GBS manifestations 19 days after the second dose of BNT162b2 mRNA vaccine. She presented lower limb predominant muscle weakness and loss of tendon reflexes. Nerve conduction study showed acute motor and sensory axonal neuropathy. In addition, she developed notable deep sensory ataxia, and showed positive pathological reflex, gaze‐evoked nystagmus and altered consciousness, which suggested brainstem involvement.ConclusionsThis is the first coronavirus disease 2019 vaccine‐related GBS complicated with such central nervous system manifestations.

Guillain-Barre Syndrome linked to SARS-CoV-2 infection – meta-analysis and literature review

Background
 The novel coronavirus disease-2019 (COVID-19), which is caused by Severe Acute Respiratory Distress Syndrome coronavirus 2 (SARS-CoV2), was originally detected in Wuhan, China in December 2019. In this meta-analysis and literature review, we compared and summarized the clinical presentation, cerebrospinal fluid (CSF) and electromyography (EMG) findings and outcomes in SARS-CoV-2 patients with Guillain-Barre Syndrome (GBS) and its variants.
 Methods
 We conducted a literature review in February 2023 searching for terms “Guillain-Barre Syndrome and COVID-19", “SARS neurology”, “COVID-19 complications”. We used PubMed and Google Scholar databases inquiring case reports or series of cases published between April 1, 2020, and September 14, 2023.
 Results
 Of the 52 GBS cases 61,5% (n=32) were male and 39,5% (n=20) were female. The mean age was 57 years old. A total of 75% (n=33) patients presented acute inflammatory demyelinating polyneuropathy (AIDP) variant, 6,8% (n=3) presented acute motor axonal neuropathy (AMAN) variant, 15,9% (n=7) presented acute motor-sensory axonal neuropathy (AMSAN) variant. A total of 85,7% (n=42) of patients were diagnosed with albuminocytological dissociation. During the hospitalization, a total of 30,8% (n=16) required mechanical ventilation. A total of 61,5% (n=32) of patients were treated with a 5-day regimen of intravenous immunoglobulin (IVIG) in dose 0.4 g/kg/day. There were 46,1% (n=24) complete recoveries from GBS, 32,7% (n=17) partial recoveries and 9,6% (n=5) of patients did not respond to treatment. A total of 11,5% (n=6) of patients died.
 Conclusion
 It is crucial to follow patients with COVID-19 and GBS over time to estimate properly the efficacy of treatment and evaluate the real percentage of recovery and complications.

Navigating the chemotherapy-induced acute motor and sensory axonal neuropathy and diabetic ketoacidosis in an adolescent girl with acute lymphoblastic leukemia: A case report

Abstract Acute lymphoblastic leukemia (ALL), the most common hematological malignancy of childhood, can cause significant morbidity and mortality due to the tumor burden or toxic effects of chemotherapeutic agents. Early detection and appropriate treatment of these complications are crucial for improving survival and quality of life. We report a case of a 12-year-old girl with B-cell ALL who experienced multiple chemotherapy-related adverse events, namely pancreatitis, hypertriglyceridemia, and a rare combination of steroid and l-asparaginase-induced hyperglycemia with diabetic ketoacidosis, and vincristine-induced acute motor and sensory axonal neuropathy. She was managed by discontinuation of vincristine and l-asparaginase and given intravenous immunoglobulin, fluid resuscitation, and antibiotics. She had a full neurological recovery within 2 months and continued with the modified chemotherapy protocol.

Before blaming SARS-CoV-2 for GBS, other causes should be ruled out.

We read with interest the article by Parikh et al.[1] about a 2.5-year-old female patient who developed progressive, flaccid quadriparesis that resulted in being unable to stand or walk. Work-up revealed positive IgG-antibodies against SARS-CoV-2, demyelinating neuropathy, and dissociation cyto-albumin.[1] Guillain–Barre syndrome (GBS) was diagnosed and intravenous immunoglobulins (IVIG) were given with little benefit.[1] The study is appealing but raises concerns. We disagree with the diagnosis of acute SARS-CoV-2 infection.[1] The patient tested negative for SARS-CoV-2 in the PCR and the rapid antigen test (RAT).[1] Only neutralizing IgG antibodies were elevated.[1] Given that neutralizing antibodies can persist for months,[2] it cannot be ruled out that SARS-CoV-2 infection may have occurred already weeks or months before the onset of GBS, suggesting a causal relationship between SARS-CoV-2 and GBS rather unlikely. Further arguments against acute SARS-CoV-2 infection are that the history was negative for pulmonary or gastrointestinal infections shortly before the onset of GBS,[1] the lymphocyte count was normal, and no other stigmata of COVID-19 were present.[1] Acute SARS-CoV-2 infections are often associated with lymphopenia.[3] We disagree with the interpretation of nerve conduction studies as “demyelinating.” According to Table 1, nerve conduction velocities in the median, ulnar, peroneal, and tibial nerves were within normal limits.[1] The findings in Table 1 are more suggestive of an axonal lesion;[1] therefore, the diagnosis should be acute, motor, axonal neuropathy (AMAN) rather than acute, inflammatory demyelinating polyneuropathy (AIDP). We disagree with the statement in the discussion that there were no abnormalities on follow-up.[1] No results of follow-up investigations were given in the case description.[1] The patient was discharged with severe paraparesis (muscular research council [MRC] 2/5 respectively 3/5).[1] We should know the period in which neurological abnormalities have completely disappeared. Because muscle strength in the lower limbs hardly improved (MRC 2/5 bilaterally at onset, MRC 2/5 respectively 3/5 at discharge), IVIGs can be rated as rather ineffective. We should know if ever plasmapheresis was considered. Additionally, GBS patients who do not respond to treatment should be evaluated for nodopathy.[4] and it should be borne in mind that about one-third of GBS patients have no causative agent. Because the patient had difficulty sitting down,[1] involvement of the axial muscles cannot be ruled out. Because respiratory muscles are often affected along with the axial muscles, we should be informed if there was any evidence of muscular respiratory insufficiency. Were oxygen saturation and lung function tests within normal limits? The results of the virus panels are missing.[1] We should know which viral infections have been ruled out. Given that GBS is often triggered by previous viral infections,[5] ruling them all out is imperative. Even in the absence of a gastrointestinal infection, the patient must be tested for antibodies to Campylobacter jejuni and Mycoplasma pneumoniae.[5] Overall, the interesting study has limitations that challenge the results and their interpretation. Diagnosing COVID-19 requires a positive PCR. A causal connection between SARS-CoV-2 and GBS can only be established if a temporal connection between the infection and the onset of neurological compromise can be demonstrated. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Ethics approval Ethics approval was in accordance with ethical guidelines. The study was approved by the institutional review board. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Immune-mediated polyneuropathy in cats: Clinical description, electrodiagnostic assessment, and treatment.

Suspected immune-mediated polyneuropathy has been increasingly reported in cats, especially in the last decade, but the condition remains poorly understood. Refine the clinical description and review the classification of this condition based on electrodiagnostic investigation and evaluate the benefit of corticosteroid treatment and L-carnitine supplementation. Fifty-five cats presented with signs of muscular weakness and electrodiagnostic findings consistent with polyneuropathy of unknown origin. Retrospective, multicenter study. Data from the medical records were reviewed. The owners were contacted by phone for follow-up at the time of the study. The male-to-female ratio was 2.2. The median age of onset was 10 months, with 91% of affected cats being <3 years of age. Fourteen breeds were represented in the study. The electrodiagnostic findings supported purely motor axonal polyneuropathy. Histological findings from nerve biopsies were consistent with immune-mediated neuropathy in 87% of the tested cats. The overall prognosis for recovery was good to excellent, as all but 1 cat achieved clinical recovery, with 12% having mild sequelae and 28% having multiple episodes during their lifetime. The outcome was similar in cats with no treatment when compared with cats receiving corticosteroids or L-carnitine supplementation. Immune-mediated motor axonal polyneuropathy should be considered in young cats with muscle weakness. This condition may be similar to acute motor axonal neuropathy in Guillain-Barré syndrome patients. Based on our results, diagnostic criteria have been proposed.

A Case of Acute Motor Sensory Axonal Neuropathy, a Rare Form of Guillain-Barre Syndrome, Developing After Multiple COVID-19 Infections (P13-8.009)

<h3>Objective:</h3> Guillain-Barre syndrome (GBS), an autoimmune polyneuropathy characterized by ascending weakness and characteristic cerebrospinal fluid (CSF) findings, has been identified in patients previously diagnosed with COVID-19, treated for COVID-19 infection, or vaccinated against the same. GBS is divided into axonal and demyelinating subtypes, and confirmatory testing such as electromyography and nerve conduction studies can help differentiate between subtypes. Here we present a case with recurrent COVID-19 infections, most recently treated with bebtelovimab, who developed acute motor sensory axonal neuropathy (AMSAN), a rare variant of axonal GBS. <h3>Background:</h3> A 33-year-old female presented with progressive ascending weakness and sensory loss that started two weeks after COVID-19 infection. She was fully vaccinated, however, had four episodes of COVID-19 infection within the past year and a half. Two weeks after the most recent infection, which was treated with bebtelovimab, she developed rapidly progressive weakness and sensory impairment in bilateral lower extremities quickly involving upper extremities. She received two rounds of intravenous immunoglobulin (IVIG) three and five weeks after symptoms onset. Her symptoms continued to progress requiring hospitalization. Exam showed severe weakness in all limbs with distal muscle atrophy in upper extremities. Imaging was negative. CSF demostrated albuminocytological dissociation. Nerve conduction studies concluded severe motor and sensory axonal neuropathy with active denervation which led to the suspicion of Acute Motor and Sensory Axonal Neuropathy (AMSAN), a rare variant of GBS. She underwent plasmapheresis with only minimal improvement. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> Many cases of GBS have been reported after COVID-19 infection but only a few of them are about AMSAN. This case presents unique features including multiple COVID-19 infections despite prior vaccination, and the development of this rare variant of GBS. It raises the concern of multiple COVID-19 infections having a cumulative impact on peripheral nervous system leading to more severe forms of GBS with poor response to treatment. <b>Disclosure:</b> Dr. Narvaez Caicedo has nothing to disclose. Mr. Rao has nothing to disclose. Dr. Patel has nothing to disclose. Dr. Wu has nothing to disclose.

Neurological manifestations of scrub typhus: Encephalitis and Acute Inflammatory Demyelinating Polyneuropathy (AIDP) (P2-10.002)

<h3>Objective:</h3> We report a case of a young male with serologically confirmed scrub typhus presenting with fever, eschar, altered sensorium, and progressive quadriplegia to illustrate the neurological complications (central and peripheral) of scrub typhus. <h3>Background:</h3> A young male presented to the emergency with complaint of high-grade fever for the past 12 days and altered sensorium. From the 10th day of illness, he started complaining of worsening weakness in all four limbs. A black, crusted plaque with an erythematous halo, giving a ‘cigarette-burn’ appearance suggestive of an eschar, was noted on the right forearm. On neurological examination, the patient was disoriented to time and place. Neck rigidity was absent. Reduced power of 3/5 in upper limbs and 1/5 in lower limbs was noted. Deep Tendon Reflexes were absent, and plantars were mute. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> CSF analysis revealed no cells, mildly raised proteins (75 mg/dl), and normal glucose. CSF Grams staining, culture, India Ink preparation, and GeneXpert for Tuberculosis, was negative. Blood cultures were sterile. Scrub typhus IgM antibodies were detected using Enzyme-Linked-Immunosorbent-Assay (ELISA). Abdominal ultrasound revealed hepatomegaly. MRI-Brain revealed diffuse gyral swelling and hyperintensity on T2-weighted and FLAIR sequences involving bilateral lobes with no diffusion restriction, consistent with encephalitis. No abnormal post-contrast enhancement was noted. MRI-Spine was normal. Nerve Conduction Studies (NCS) were suggestive Acute Motor Axonal Neuropathy (AMAN) variant of Guillain-Barré-Syndrome (GBS). The patient received doxycycline. IVIG was added after NCS results. His condition gradually improved, and there was regaining of muscle strength in the following weeks with physiotherapy. <h3>Conclusions:</h3> Scrub typhus induced neurological complications are widely reported, with meningoencephalitis being the most common manifestation. However, PNS complications such as GBS are infrequent, and a high degree of clinical suspicion with prompt appropriate therapy and supportive care may prevent morbidity and mortality. <b>Disclosure:</b> Ms. Sood has nothing to disclose. Mr. Modi has nothing to disclose. Dr. Arora has nothing to disclose. Dr. Moudgil has nothing to disclose.

Severe Porphyric Neuropathy: A Tale of Two Challenging Cases (P13-4.003)

<h3>Objective:</h3> To highlight diagnostic and therapeutic challenges in patients with severe porphyric neuropathy. <h3>Background:</h3> Acute Intermittent Porphyria (AIP) is a rare genetic disorder that leads to the accumulation of toxic metabolites as a result of an enzyme deficiency in the heme biosynthesis pathway. AIP can present with non-specific neurovisceral symptoms and motor weakness that mimics other neurologic disorders including acute inflammatory demyelinating polyneuropathy, vasculitic neuropathy, and lead toxicity. A severe AIP flare can lead to flaccid quadriplegia, respiratory paralysis, and death. While strategies and a novel therapy for preventing recurrent attacks are well-known, no guidelines exist for monitoring treatment response or disease progression in severe porphyric neuropathy. <h3>Design/Methods:</h3> Case series of patients with severe porphyric neuropathy <h3>Results:</h3> We describe two patients, one with known AIP, who initially presented to our hospital with non-specific symptoms. Each went on to develop severe motor weakness resulting in flaccid paralysis with bulbar involvement, and in one case, respiratory failure and death. The rapid progression of weakness in both cases led to concern for acute motor axonal neuropathy. One patient received extensive immunotherapy prior to diagnosis of AIP. Challenges faced in the management of both patients included delays in treatment related to the turnaround time of special laboratory studies, limited availability of intravenous hemin due to the high cost, and lack of treatment guidelines for duration of hemin in severely affected patients. <h3>Conclusions:</h3> Severe porphyric neuropathy can present as flaccid quadriplegia with bulbar symptoms. Though early diagnosis of an AIP flare may be challenging in these patients, recognition of the underlying condition and quick initiation of hemin may limit long-term disability and mortality. <b>Disclosure:</b> Mrs. Gilley has nothing to disclose. Dr. Chapman has nothing to disclose. Dr. Carr has nothing to disclose. Dr. Ladd has nothing to disclose. Dr. Willis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech, Alexion, Biogen. Dr. Willis has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for University of Mississippi.

Varicella Zoster Virus Related Myelo-Radiculo-Neuropathy: Case Report

Aim: Varicella zoster virus (VZV) infections can cause various neurological syndromes during primary infection or reactivation periods. We report two rare neurological complications of VZV, acute longitudinally extensive myelitis and acute motor axonal neuropathy due to primary chickenpox infection that occurred concomitantly. Case: A 76-year-old female patient presented with skin lesions consistent with chickenpox all over the body and walking difficulty ten days ago. Neurological examination revealed paraparesis and areflexia. Spinal cord magnetic resonance imaging revealed a long-segment transverse myelitis lesion involving the lower cervical and thoracic levels. Elevated protein and the presence of VZV DNA were detected in the cerebrospinal fluid, and VZV IgM was detected in the serum. Two days later, the patient's weakness in her legs increased. Electromyography showed signs of acute axonal motor polyneuropathy. The patient was treated with intravenous immunoglobulin after high-dose steroid and antiviral therapy. Partial recovery was observed. Conclusion: Transverse myelitis and Guillain Barré syndrome are rare complications of VZV infection. Our case is first case where these two neorological conditions related to VZV were seen in the same patient.

Association between hyperCKemia and axonal degeneration in Guillain–Barré syndrome

BackgroundElevated serum creatine kinase (CK) levels have been reported in patients with Guillain–Barré syndrome (GBS), more frequently in patients with acute motor axonal neuropathy (AMAN) than in those with acute inflammatory demyelinating polyneuropathy (AIDP). However, some patients with AMAN show reversible conduction failure (RCF), characterized by rapid recovery without axonal degeneration. The present study tested the hypothesis that hyperCKemia is associated with axonal degeneration in GBS, regardless of the subtype.MethodsWe retrospectively enrolled 54 patients with AIDP or AMAN whose serum CK levels were measured within 4 weeks from symptom onset between January 2011 and January 2021. We divided them into hyperCKemia (serum CK ≥ 200 IU/L) and normal CK (serum CK < 200 IU/L) groups. Patients were further classified into axonal degeneration and RCF groups based on more than two nerve conduction studies. The clinical features and frequency of axonal degeneration and RCF were compared between groups.ResultsClinical characteristics were similar in the hyperCKemia and normal CK groups. Compared with that in the RCF subgroup, the frequency of hyperCKemia was significantly higher in the axonal degeneration group (p = 0.007). Patients with normal serum CK levels showed better clinical prognosis, evaluated by the Hughes score at 6 months from admission (p = 0.037).ConclusionHyperCKemia is associated with axonal degeneration in GBS, regardless of the electrophysiological subtype. HyperCKemia within 4 weeks from symptom onset might be a marker of axonal degeneration and poor prognosis in GBS. Serial nerve conduction studies and serum CK measurements will help clinicians understand the pathophysiology of GBS.

Guillain-Barré syndrome AMSAN variant in a 90-year-old woman after COVID-19: a case report

BackgroundGuillain-Barré syndrome (GBS) is an inflammatory disease of the peripheral nervous system characterized by rapidly evolving polyneuropathy caused by autoimmune demyelination and/or axonal degeneration. Since SARS-CoV-2 outbreak, several GBS cases following exposure to coronavirus disease-2019 (COVID-19) have been reported in literature, raising the concern of the latter being a potential trigger event for GBS.Case presentationWe report the case of a 90-year-old Caucasian woman who was admitted to our hospital because of fatigue, worsening gait and leg strength, dysphonia, dysarthria and dysphagia, started 3 weeks after being exposed to COVID-19. Based on clinical presentation GBS was suspected, so she performed a lumbar puncture and electromyography, which confirmed the diagnosis of acute motor and sensory axonal neuropathy (AMSAN) variant. We administered high dose of intravenous immunoglobulin with slight neurological improvement. However, after 2 weeks of hospitalization with maximization of care, her physical condition worsen, manifesting severe frailty. The patient was discharged with home support services for managing parenteral nutrition and intense scheduled physiotherapy. A few days later, the patient experienced a further decline in her clinical condition and died at home.ConclusionsTo the best of our knowledge, we report the oldest woman with GBS AMSAN variant after COVID-19 described in the existing literature. Our case supports further research aimed at improving recognition, characterization and prompt management of neurological diseases related to COVID-19 in older patients.

707 - Acute motor and sensory axonal neuropathy in a patient with undiagnosed mixed connective tissue disease

707 - Acute motor and sensory axonal neuropathy in a patient with undiagnosed mixed connective tissue disease

Evaluation of Factors Associated with the Clinical Course and Prognosis of Patients with Guillain-Barre Syndrome

Aim: This study aims to investigate the clinical, laboratory, electrophysiological, and demographic characteristics of patients with Guillain-Barre Syndrome (GBS) who were admitted to our clinic and underwent treatment and the factors contributing to the prognosis at discharge.Materials and Methods: The study included 138 patients admitted to our clinic for treatment between January 2013 and December 2017, whose patient records were reviewed retrospectively. The Hughes scores, demographic characteristics, and clinical and laboratory data of the patients at admission and discharge were recorded.Results: The study sample comprised 61 female (44.2%) and 77 male (55.8%) patients with a mean age of 58.1 years. In evaluations of the Hughes scores at admission and discharge, 117 patients were considered to have a good prognosis and 21 patients to have a poor prognosis at discharge. In the poor prognosis group, advanced age (p=0.028), being in the acute motor axonal neuropathy (AMAN) subtype (p=0.001), development of sepsis (p=0.007), need for mechanical ventilation (p&amp;lt;0.001), high Hughes scores on admission (p&amp;lt;0.001), extended hospitalization (p=0.030), increased WBC count (p=0.033), presence of hyponatremia (p&amp;lt;0.001), abnormal liver function test (p=0.08) were higher than the good prognosis group.Conclusion: Early identification of GBS patients who may have a poor prognosis and rapid application of appropriate treatment methods are essential in creating positive effects on the clinical course and prognosis in this patient group.

Long-term Disability and Poor Outcome Predictors of Guillain-Barre Syndrome in Children: A Systematic Review

Background: Guillain-Barre syndrome (GBS) is an immune-mediated polyneuropathy and a common cause of acute ascending weakness in children. Objectives: This study aims to report long-term disability and poor outcome predictors of GBS in children. Medline (via Pubmed), Embase, Cochrane Library, Web of Science, and Scopus database was searched for relevant studies until April 2022, with a designated search strategy, using MeSH terms and free keywords. Methods: Studies evaluating functional outcomes of GBS in children with at least one year of follow-up were included. All studies achieved acceptable quality for inclusion. After selecting studies based on inclusion criteria, data were extracted based on a modified standardized Joanna Briggs Institute (JBI) data extraction tool, and the methodological quality of studies were reviewed using the Joanna Briggs Institute (JBI) critical appraisal tool. Results: Fourteen studies were included in this systematic review consisting of 1141 patients (647 males, 466 females, and 28 unclassified). Follow-up duration varied from one year to 11 years. The prevalence of the GBS subtype was as follows, acute inflammatory demyelinating polyneuropathies (AIDP) 46.6%, acute motor axonal neuropathy (AMAN) 30.2%, acute motor and sensory axonal neuropathy (AMSAN) 6.8% and Miller fisher 6.1%. The most commonly reported poor outcome after at least one year of follow-up was walking disabilities and gait disorders. Motor deficits and weakness, sensory complaints, including pain or paresthesia and fatigue were other prevalent residual symptoms. Axonal form of GBS was the most reported poor outcome predictor, followed by Hughes disability score &gt;3, a delay in independent walking, artificial ventilation, and rapid progression of symptoms. Conclusions: Despite the good prognosis of GBS in children, they could suffer long-term sequels, especially in walking abilities and gait. The axonal form is considered a crucial poor predictive factor.

Miller Fisher Syndrome Induced by Chemotherapy in Known Case of Acute Lymphocytic Leukaemia: A Case Report

Introduction: Guillain-Barre Syndrome (GBS) is an acute-onset autoimmune-mediated neuropathy. Guillain-Barre Syndrome can be divided into three subtypes: acute inflammatory demyelinating poly-radiculo-neuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). About 20% of patients with GBS develop respiratory failure and require mechanical ventilation. We are presenting a variant of GBS (Miller Fisher Syndrome, or MFS), which has been confirmed by nerve conduction studies along with the triad of ophthalmoplegia, ataxia, and areflexia. The objective of this study is to present a rare case of chemotherapy-induced GBS. Important clinic findings: A 25-year-old gentleman with acute lymphocytic leukemia on active chemotherapy treatment presented with lower limb weakness. This weakness started after his fifth chemotherapy session. After the sixth chemotherapy, he developed complete paralysis of the left lower limb. Later, he developed right lower limb paralysis. He was also complaining of eye dryness and incomplete closure of both eyes. While inpatient, he developed upper-limb weakness. His chemotherapy consisted of MESNA, cyclophosphamide, doxorubicin, vincristine, cyorabine, and methotrexate. He had ptosis and ophthalmoplegia in the left abducent and right oculomotor regions. He had bilateral facial nerve palsy. He was hypotonic with power grade 3 in the upper limbs and grade 0 in the lower limbs with areflexia. His sensation was intact in the upper limbs but lost in the lower limbs. His planter reflexes were mute. Diagnoses and Management: Intravenous immunoglobulins were given for 5 days. A nerve conduction study showed severe demyelinating sensorimotor polyradoculoneuropathy with secondary axonal loss. The triad of ataxia, ophthalmoplegia, and areflexia was consistent with MFS. The patient improved over the course of the hospital stay but did not reach full recovery. Conclusion: Although GBS is uncommon, it must be taken into account when making a differential diagnosis for any patient presenting with progressive weakness. Drug history is important in all GBS cases.

The Association of Acute Motor Axonal Neuropathy (Guillain-Barre' Syndrome Variant) with Coronavirus (SARS-Cov-2) in a Child: A Case Report.

Various reports of neurological manifestations of SARS-COV-2 infection after the virus outbreak are available, including anosmia, seizures, acute flaccid myelitis, Guillain-Barré syndrome (GBS), and encephalitis. Most of the literature has focused on the respiratory manifestation of SARS-CoV-2 infection in adults, but recent evidence showed that it is not confined to the respiratory tract. This report is about a rare variant of GBS acute motor axonal neuropathy (AMAN) in a child due to COVID-19 infection An 11 years old boy was referred to the hospital with a history of three-day lasting mild fever, and gastroenteritis, two weeks before starting symptoms. He was presented with progressive ascending weakness, paresthesia, and areflexia in four limbs four days ago. Nasopharyngeal swab polymerase chain reaction (PCR) was positive for SARS-CoV-2. The electrodiagnostic finding was compatible with acute generalized axonal motor neuropathy, and imaging revealed thoracolumbar syrinx and nerve root enhancement in lumbosacral MRI. Other lab tests were normal. GBS and its variant are one of the manifestations of SARS-CoV-2 in children. Children with an unexplained neurological process should be tested for SARS-CoV-2.

Dose-Effect Relationship of Motor Nerve Inexcitability on Outcome in Guillain-Barré Syndrome: A Prospective Cohort Study.

One or more inexcitable motor (IM) nerves are common during electrodiagnostic (EDx) study in Guillain-Barré syndrome (GBS). This study assessed the dose-effect relationship of IM nerves on outcome in patients with acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor and/or sensory axonal neuropathy (AMAN and AMSAN). Eighty-eight GBS patients admitted during May 2018-June 2023 underwent detailed clinical evaluation and EDx study. Admission and follow-up disability were assessed on a 0-10 Clinical Grading Scale (CGS). Outcome was recovery at 6 months, defined as good (CGS <3) and poor (CGS ≥3). Binary multivariate logistic regression with backward elimination was used to calculate independent predictors of outcome. Proportion of patients with complete recovery decreased significantly with increasing numbers of IM nerves (P < 0.01). Seventy-six patients were followed for 6 months. Among patients with IM nerves (n = 28), complete recovery was similar between AIDP and axonal GBS (70% vs. 50%, respectively; P = 0.40). However, in patients with recordable compound muscle action potentials (CMAPs) in all the motor nerves (n = 26), axonal GBS had significantly poor recovery compared to AIDP (75% vs. 9.1%; P = 0.01). Among patients receiving intravenous immunoglobulin (IVIg; n = 42), poor recovery was seen in 53.6% with IM nerves compared to 35.7% without (P = 0.28), while it was 37.5% versus 5.6% (P = 0.04), respectively, in those who did not receive IVIg (n = 34). However, only admission disability (odds ratio [OR] 0.88, 95% confidence interval [CI] 0.81-0.97; P = 0.007) was found to be an independent predictor of outcome. Although increasing numbers of IM nerves were associated with poor outcome on univariate analysis, they did not predict 6 months' outcome independently. Outcome did not differ between axonal GBS and AIDP among those with IM nerves. IVIg improved outcome in patients with IM nerves.

Markedly Prolonged Latency of Patellar Tendon Reflex in a Patient With Apparent Acute Motor Axonal Neuropathy.

Markedly Prolonged Latency of Patellar Tendon Reflex in a Patient With Apparent Acute Motor Axonal Neuropathy.

Sural Sparing Pattern and Sensory Ratio as Electrodiagnostic and Prognostic Markers in Pediatric Guillain-Barré Syndrome.

We aimed to evaluate the presence of sural sparing pattern (SSP) and sensory ratio in pediatric Guillain-Barré syndrome (GBS), their distribution to subtypes, and their relationship with demographic and clinical features with a focus on the disability and muscle strength. This single-center retrospective study was conducted on pediatric GBS patients of both sexes with 2 years follow-up and two nerve conduction studies in which SSP and sensory ratio were calculated. Three subgroups of SSP were formed by separate calculation of median (SSP-m) and ulnar (SSP-u) and both median and ulnar sensory nerve action potentials (SNAPs; SSP-total). Muscle strength and disability were evaluated with the Medical Research Council (MRC) sum score and Hughes functional grading scale (HFGS), respectively. SSP total was identified in 70.6% (n: 24) of the patients, while sensory ratio >1 was observed in 20 (66.7%) patients. Patients with SSP-m, SSP-u, SSP-total, or sensory ratio >1 had higher HFGS scores, while patients with SSP-m, SSP-u, or SSP-total had lower MRC sum scores. SSP parameters were significantly associated with muscle strength and disability scores in acute motor axonal neuropathy patients. Both SSP and sensory ratio can be used for diagnostic and prognostic purposes. Disability and muscle strength are associated with SSP and sensory ratio in pediatric GBS.