Acute Motor Axonal Neuropathy Research Articles

A rare kind of Guillain-Barre syndrome triggered by acute hepatitis A infection in a pediatric patient: a case report and review of literature.

Guillain-Barre Syndrome (GBS) is a rare but serious neurological disorder characterized by acute flaccid paralysis and areflexia, usually after an infectious disease. This case report describes a previously healthy 9-year-old boy who developed GBS following an acute hepatitis A infection. The patient presented with rapidly progressive weakness, ascending paralysis, and areflexia, confirmed by clinical and electrophysiological findings. Results were consistent with the GBS subgroup of Acute Motor Axonal Neuropathy. Treatment with intravenous immunoglobulin (IVIG) led to gradual improvement, highlighting the importance of early recognition and intervention. This report reviews the current literature on the association between GBS and hepatitis A, emphasizing the rarity of such cases in pediatric populations. The report aims to raise awareness among clinicians about this potential complication of hepatitis A, underscoring the need for prompt diagnosis and treatment to improve outcomes in similar cases. The report emphasizes the need for prompt diagnosis and treatment to improve outcomes in similar cases.

Clinical characteristics and functional outcomes of pediatric Guillain-Barré syndrome admitted to the Neuro-intensive care unit: a decade-long retrospective observational study.

Guillain-Barré Syndrome (GBS) remains a significant contributor to acute flaccid paralysis in pediatric patients worldwide. Despite its impact, studies focusing on pediatric GBS requiring intensive care unit (ICU) management are limited. This study aimed to address this gap by exploring the clinical and outcome characteristics of pediatric GBS necessitating ICU care. This retrospective observational study, spanning a decade, analyzed the records of 75 pediatric GBS patients admitted to the Neuro-ICU of a tertiary care center in South India. Data included demographics, prodromal symptoms, clinical features, investigations, treatment modalities, and outcomes. The majority (55/75) of patients were male, with a median age of 12 years. The highest incidence of GBS requiring ICU admission was in the monsoon season. Prodromal symptoms were observed in 56%. Most patients (93.33%) presented with typical GBS symptoms, and 40% had respiratory distress on ICU admission. Acute motor axonal neuropathy (AMAN) was the most common subtype. Approximately 80% required mechanical ventilation, with a median duration of 22.5 days. No in-hospital mortality was recorded. At discharge, most patients had a GBS disability score of 4, improving to 2 at a median follow -up of 228 days. Pediatric GBS patients requiring ICU care exhibit distinctive characteristics, including a higher prevalence of AMAN subtype, seasonal clustering, and favorable outcomes with intensive treatment. The absence of in-hospital mortality underscores the effectiveness of prompt ICU admission and dedicated Neuro-intensive care.

Epidemiological and clinical aspects of Guilain-Barré syndrome, 2012-2022

Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy, generally post-infectious, and is the leading cause of acute flaccid paralysis worldwide. To describe the epidemiological, clinical and behavioral characteristics of Guillain-Barré syndrome. An observational, cross-sectional and descriptive study was carried out. The records of all patients with GBS from 2012-2022 were reviewed, and information on epidemiological and clinical variables was obtained. The incidence was calculated for each of the study years and the trend for the study period using the Pearson correlation coefficient, considering p ≤ 0.05 statistically significant. A total of 477 cases were obtained, 67% were men, among the comorbidities, drug addictions were present in 31% of the cases. Most cases were preceded by a gastrointestinal infectious condition. The most common phenotype was acute motor axonal neuropathy. 29% showed up in summer. A statistically significant tendency to increase was observed (p ≤ 0.05) and a mortality of 2.5% with predominance in the axonal variety. The results of this study cover a relatively long period compared to other reviews, the findings agree with previous studies. GBS showed a statistically significant tendency to increase.

Potential advantage of Therapeutic Plasma Exchange over Intravenous Immunoglobulin in children with axonal variant of Guillain-Barré syndrome: A report of six paediatric cases

Guillain-Barre syndrome (GBS) is a disease entity described in literature since 1859. It is associated with various etiological, clinical and immunological factors with prognostic predictive value. Both Intravenous immunoglobulin (IVIG) and Therapeutic Plasma Exchange (TPE) have been regarded as the first-line treatment for GBS. Certain diagnostic tools help us in early identification of GBS subtypes that may aid clinical management. Here, we have discussed six paediatric cases of GBS of Acute Motor Axonal Neuropathy (AMAN) subtype that were considered for TPE. 5 out of 6 patients were eventually weaned from mechanical ventilation and discharged from the hospital. This study emphasizes the role of TPE in management of severe IVIG refractory GBS with axonal involvement that can be beneficial to the patient. TPE may be considered early in GBS cases with axonal involvement.

Prognostic Value of Early Nerve Conduction Studies in Suspected Guillain-Barré Syndrome in Pediatric Age Group: An Observational Study.

Guillain-Barré syndrome (GBS) is a rare but potentially life-threatening autoimmune disorder affecting the peripheral nervous system. In pediatric patients, early diagnosis and intervention are crucial for improved outcomes. This observational study aimed to evaluate the prognostic value of early nerve conduction studies (NCS) in pediatric patients diagnosed with GBS. The study was conducted at All India Institute of Medical Sciences (AIIMS) Patna from January 2022 to March 2023, involving 11 patients aged between five and 14 years. All patients underwent NCS on three occasions: within three days, between three to sevendays, and between 7 and 14 days after admission. The findings indicated that 63.64% of the patients exhibited a demyelinating pattern (acute inflammatory demyelinating polyneuropathy, AIDP), while 36.36% showed an axonal pattern (acute motor axonal neuropathy, AMAN). Early NCS findings were critical in predicting the severity and guiding the timely initiation of appropriate treatment, leading to improved outcomes. This study underscores the importance of early electrophysiological assessment in pediatric GBS for optimal patient management and prognosis.

Temporal Pattern of Individual Neurological Function Recovery in Guillain-Barré Syndrome.

Background: There is paucity of studies on the temporal pattern of recovery of facial, bulbar, sensory, motor, and autonomic dysfunction in Guillain-Barré syndrome (GBS), although many studies have reported short- and long-term functional outcomes. We report the temporal pattern of recovery of various neurological functions in GBS, and compare the pattern of recovery between acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Methods: Forty-two patients with GBS were prospectively included, and their clinical details, including peak disability on a 0-6 scale, were noted. The day of complete recovery in motor, sensory, facial, bulbar, and autonomic functions during 3 months of follow-up was recorded. Results: Complete recovery of autonomic function occurred in all (median, 12 days), bulbar weakness in 91.3% (median, 15 days), facial weakness in 86.2% (median, 19 days), and sensory functions in 82.1% (median, 20 days). Only 9.5% of patients achieved normal motor function within 3 months. The days of complete recovery of bulbar, facial, autonomic, and motor deficits were comparable between AIDP and AMAN. Demyelinating GBS had an earlier recovery of bulbar and sensory functions. Conclusions: The neurological recovery in GBS occurs first in the autonomic, followed by the bulbar, facial, sensory, and motor functions. The demyelinating type had an earlier recovery of bulbar and sensory functions.

Guillain-Barrè Syndrome-Retrospective Analysis of Data from a Cohort of Patients Referred to a Tertiary Care Pediatric Neuromuscular Center from 2000 to 2017: Electrophysiological Findings, Outcomes, and a Brief Literature Review.

Background and Objectives: Guillain-Barré syndrome (GBS) is the most frequent cause of acute flaccid paresis in children. The aim of this study was to describe the clinical and electrophysiological findings and outcomes of children with GBS diagnosed in our unit. Moreover, the literature on pediatric GBS cases from the past 5 years was reviewed. In this retrospective study, we reported data on 12 patients (9 male and 3 female patients; mean age: 5 y, 4 mo; range: 9 mo-11 y) clinically diagnosed at the Child Neurology Unit of the AUSL-IRCCS of Reggio Emilia, Italy, between 2000 and 2017 and a brief analysis/comparison with data from the literature. Materials and Methods: Data were collected from medical charts. Results: In our cohort, male patients were more frequent than female ones (9 vs. 3), and upper respiratory tract infection (n = 8, 66.7%) was the most frequent triggering factor. The main clinical symptoms on admission were distal lower limbs' weakness with gait difficulties (83.3%), pain (50%), upper limbs' weakness (50%), and dysphagia for liquids (25%). Peripheral neurophysiological studies revealed acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 66.6% of the children, acute motor and sensory axonal neuropathy (AMSAN) in 25%, and acute motor axonal neuropathy (AMAN) in 8.3%. Ten individuals (83.3%) received timely treatment with intravenous immunoglobulins (IVIG), and, out of these ten patients, 58% received concomitant treatment with IV methylprednisolone because of a progressive disease course. Complete remission was observed in the majority of individuals (91.6%) within 6 months of symptom onset. Conclusions: Different subtypes of GBS can affect children; however, the outcome is usually positive. Early treatment appears to be important for a favorable outcome.

Opisthotonic posturing in Guillian-Barre syndrome.

Here we report a child of Gullian Barre syndrome (GBS) with opisthotonic posturing and we subsequently detected Scrub typhus in him. An 11-year-old boy presented with progressive motor quadriparesis with transient bladder retention, bilateral facial weakness, diminished gag reflex, absent reflexes and his nerve conduction studies suggested Acute Motor Axonal Neuropathy (AMAN) GBS. His power gradually started recovering after one week. However, he had opisthotonus and signs of meningeal irritation. The child's CSF examination was consistent with GBS. His bacterial, fungal, tubercular microscopy and cultures and viral markers were negative. IgM for Borrelia and Leptospira and HIV ELISA were negative. IgM for scrub typhus, however, came out to be positive. Hence, we gave him azithromycin and he recovered almost completely in 3 months. To our knowledge there is no previous report of opisthotonic posturing in GBS patients. This could be due to radicular involvement in immune mediated etiology of GBS.

Acompanhamento de paciente diagnosticado com Síndrome de Guillain-Barré com lesão axonal do tipo sensitivo-motor (AMSAN)

Guillain-Barre syndrome (GBS) is a rare inflammatory disease of the peripheral nervous system, with a prevalence of 1-4 cases per 100,000 population. It is characterized by acute flaccid paralysis with ascending motor and sensory symptoms, and can be electrophysiologically classified into subtypes such as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute sensory-motor axonal neuropathy (AMSAN). GBS, usually triggered by viral infections, can lead to serious complications like respiratory failure due to phrenic nerve palsy. The reported case study describes a patient with AMSAN, a severe and rare form of the disease, who presented with progressive weakness and areflexia, with a diagnosis confirmed by electroneuromyography and successful treatment with intravenous immunoglobulin. The prognosis is usually favorable with appropriate treatment, but may include complications and the need for prolonged rehabilitation.

Distinguishing Acute Inflammatory Demyelinating Polyneuropathy from Acute Motor Axonal Neuropathy

Distinguishing Acute Inflammatory Demyelinating Polyneuropathy from Acute Motor Axonal Neuropathy

Complete genome sequence of Edwardsiella tarda strain GBS0709 isolated from a Japanese patient with the acute motor axonal neuropathy subtype of Guillain-Barré syndrome.

The complete genome sequence of Edwardsiella tarda strain GBS0709, isolated from an 81-year-old Japanese patient with the acute motor axonal neuropathy subtype of Guillain-Barré syndrome, was determined. It comprised a 3,632,068 bp circular chromosome and a 5,386 bp plasmid. The overall guanine and cytosine content was 57.3%.

Clinical features and treatment of acute motor axonal neuropathy

This paper aims to investigate the clinical features and treatment of patients with acute motor axonal neuropathy (AMAN). The clinical data of 9 patients with AMAN treated in our hospital were retrospectively analyzed from prodromal infections, clinical features, neuroelectrophysiological examination, treatment and the scores of Hughes functional grading scale. Diarrhea was the most common prodromal infection, and weakness of four limbs and muscles atrophy acutely emerged. The patient's condition progressed rapidly. In these cases, therapy of immunoglobulin combined with methylprednisolone was effective. Therefore, early diagnosis and timely treating with immunoglobulin therapy are most important methods to improve the prognosis.

Clinical adverse events to dexmedetomidine: a real-world drug safety study based on the FAERS database.

Adverse events associated with dexmedetomidine were analyzed using data from the FDA's FAERS database, spanning from 2004 to the third quarter of 2023. This analysis serves as a foundation for monitoring dexmedetomidine's safety in clinical applications. Data on adverse events associated with dexmedetomidine were standardized and analyzed to identify clinical adverse events closely linked to its use. This analysis employed various signal quantification analysis algorithms, including Reporting Odds Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and Multi-Item Gamma Poisson Shrinker (MGPS). In the FAERS database, dexmedetomidine was identified as the primary suspect in 1,910 adverse events. Our analysis encompassed 26 organ system levels, from which we selected 346 relevant Preferred Terms (PTs) for further examination. Notably, adverse drug reactions such as diabetes insipidus, abnormal transcranial electrical motor evoked potential monitoring, acute motor axonal neuropathy, and trigeminal cardiac reflex were identified. These reactions are not explicitly mentioned in the drug's specification, indicating the emergence of new signals for adverse drug reactions. Data mining in the FAERS database has elucidated the characteristics of dexmedetomidine-related adverse drug reactions. This analysis enhances our understanding of dexmedetomidine's drug safety, aids in the clinical management of pharmacovigilance studies, and offers valuable insights for refining drug-use protocols.

Guillain-Barré syndrome: History, pathogenesis, treatment, and future directions.

Since its description by Guillain, Barré, and Strohl in 1916, Guillain-Barré syndrome (GBS) has attracted a large literature. The author reviews the history of research into its pathogenesis and treatment to highlight promising avenues for future research. This is a nonsystematic personal review. Since the early 1900s, the clinical picture of GBS has been illustrated in multiple series culminating in the ongoing International Guillain-Barré Syndrome study of 2000 patients. In the 1950s and 1960s, the inflammatory nature of the commonest form, acute inflammatory demyelinating polyradiculoneuropathy (AIDP), was described. In the 1990s, two axonal forms, acute motor-sensory axonal neuropathy and acute motor axonal neuropathy, were recognized. In the 1990s and early 2000s, these forms were shown to be due to antibodies against Campylobacter jejuni glycans cross-reacting with glycolipids on axonal membranes. The pathogenesis of AIDP remains unknown, but T-cell responses to the compact myelin proteins, P2 and P0, which cause experimental autoimmune neuritis, suggest that T cells are important. Randomized controlled trials in the 1970s and 1980s showed no benefit from corticosteroids. Trials in the 1980s showed benefit from plasma exchange and in the 1990s from intravenous immunoglobulin. Future research should seek biomarkers to identify subgroups with different treatment responses, define the true natural history of the disease with population-based epidemiological studies, study the pathology in autopsies early in the disease, seek causative antibodies and confirm autoimmune T-cell responses in AIDP, and expand treatment trials to include anti-T-cell agents.

Variegate porphyria onset as neurovisceral manifestation and unusual sensory impairment distribution. Case report

Background: Acute hepatic porphyrias are heme metabolism disorders presenting with a broad clinical spectrum, including neurovisceral manifestations. Peripheral neuropathy is one of the most frequent complications in porphyrias and can correspond about 40 % of the neurovisceral manifestations, and it is mainly attributed to the ALA and PBG accumulation neurotoxicity. The hallmark of an acute porphyric attack is an acute motor axonal neuropathy accompanied by autonomic dysfunction. Sensory impairment is infrequent, and when present, it is classically described as the stocking-glove pattern or, more rarely, a “bathing suit” proximal distribution pattern. Objective: To report a variegate porphyria case in a young patient with unusual sensory manifestations. Methods: A 27-year-old man, previously healthy, present in context of possible arbovirus infeccion with severe abdominal pain, followed by dysautonomia due to orthostatic hypotension, change in urine color and, the emergence of sensory impairment in the trunk and proximal region of the four limbs about twenty days from onset. Dysestesia was severe and marked. Results: Positive clinical features and a urinary exposure test to sunlight corroborate in request genetic testing for acute hepatic porphyrias, with a positive mutation of the PPOX gene in heterozygosis. Discussion: Clinical manifestations with initial presentation of severe dysautonomia followed by sensory changes demarcate neurovisceral involvement of variegate porphyria, adopting an "old-bathing-suit costume" sensory pattern throughout its evolution.

Beyond Binary: Biomarkers Reveal the Coexistence of Acute Motor Axonal Neuropathy (AMAN) and Acute Inflammatory Demyelinating Polyneuropathy (AIDP) in Guillain-Barré Syndrome (P9-11.001)

Beyond Binary: Biomarkers Reveal the Coexistence of Acute Motor Axonal Neuropathy (AMAN) and Acute Inflammatory Demyelinating Polyneuropathy (AIDP) in Guillain-Barré Syndrome (P9-11.001)

Acute Motor and Sensory Axonal Neuropathy (AMSAN) Relapse After COVID-19 Infection: A Case Report and Literature Review (P3-13.010)

Acute Motor and Sensory Axonal Neuropathy (AMSAN) Relapse After COVID-19 Infection: A Case Report and Literature Review (P3-13.010)

The sural-sparing pattern in clinical variants and electrophysiological subtypes of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency.

Antiganglioside antibody frequency in routine clinical care settings.

Antiganglioside antibodies (AGAs) might be involved in the etiopathogenesis of many neurological diseases, such as Miller-Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS). Available comprehensive reference data regarding AGA positivity rates and cross-responsiveness among AGAs (where one line immunoblot is positive for ≥1 AGA) during routine clinical care are scant. In this 10-year monocentric retrospective study, 3560 immunoglobulin (Ig) G and IgM line blots (GA Generic Assays' Anti-Ganglioside Dot kit) obtained using cerebrospinal fluid (CSF) and serum samples from 1342 patients were analyzed for AGA positivity in terms of 14 diagnosis categories and AGA cross-responsiveness. Of all 3560 line blots 158 (4.4%) and of all CSF samples 0.4% (4/924) CSF line blots were AGA positive. For serum IgG, blots with positivity rates higher than the standard deviation of 15.6% were associated with MFS (GD3, GD1a, GT1a and GQ1b) and acute motor axonal neuropathy (AMAN) (GM1, GD1a and GT1a). For serum IgM, blots with positivity rates higher than the standard deviation of 8.1% were associated with AMAN (GM2, GT1a and GQ1b), MFS (GM1, GT1a and GQ1b), multifocal motor neuropathy (MMN) (GM1, GM2 and GQ1b) and chronic inflammatory demyelinating polyneuropathy (CIDP) (GM1). Cross-responsiveness was observed in 39.6% of all positive serum AGA. Testing for AGAs during routine clinical care rarely led to positive findings, both in serum and even less in CSF, except for the diagnoses AMAN, MFS, MMN and CIDP. Nonspecific findings found as cross-responsiveness between different AGA samples occur frequently, impacting the positivity of most AGA subtypes.

TREM2 deficiency impairs the energy metabolism of Schwann cells and exacerbates peripheral neurological deficits

Triggering receptor expressed on myeloid cells-2 (TREM2) has been implicated in susceptibility to neurodegenerative disease. Schwann cells (SCs), the predominant glial cell type in the peripheral nervous system (PNS), play a crucial role in myelination, providing trophic support for neurons and nerve regeneration. However, the function of TREM2 in SCs has not been fully elucidated. Here, we found that TREM2 is expressed in SCs but not in neurons in the PNS. TREM2 deficiency leads to disruption of glycolytic flux and oxidative metabolism in SCs, impairing cell proliferation. The energy crisis caused by TREM2 deficiency triggers mitochondrial damage and autophagy by activating AMPK and impairing PI3K-AKT-mTOR signaling. Combined metabolomic analysis demonstrated that energic substrates and energy metabolic pathways were significantly impaired in TREM2-deficient SCs. Moreover, TREM2 deficiency impairs energy metabolism and axonal growth in sciatic nerve, accompanied by exacerbation of neurological deficits and suppression of nerve regeneration in a mouse model of acute motor axonal neuropathy. These results indicate that TREM2 is a critical regulator of energy metabolism in SCs and exerts neuroprotective effects on peripheral neuropathy.TREM2 deficiency impairs glycolysis and oxidative metabolism in Schwann cells, resulting in compromised cell proliferation. The energy crisis caused by TREM2 deficiency induces mitochondrial damage and autophagy by activating AMPK and impairing PI3K-AKT-mTOR signaling. Moreover, TREM2 deficiency disrupts the energy metabolism of the sciatic nerve and impairs support for axonal regeneration, accompanied by exacerbation of neurological deficits and suppression of nerve regeneration in a mouse model of acute motor axonal neuropathy (by FigDraw).