Acute Motor Axonal Neuropathy Research Articles

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).

Differential diagnosis of acute flaccid paralysis in children with Guillain—Barré syndrome and neuromyelitis optica spectrum disorder: Clinical cases

Background. Acute flaccid paralysis is a clinical syndrome characterized by a sudden onset of weakness in one or more limbs with decreased or absent tendon reflexes in the affected limbs. This condition may be a manifestation of such pathologies as Guillain-Barré syndrome and neuromyelitis optica spectrum disorder. Clinical cases description. We describe two clinical cases of Guillain-Barré syndrome in patient M., 7 years old, and neuromyelitis optica spectrum disorder in patient D., 3 years old. In both children, the main clinical manifestation was acute flaccid paralysis. Patient M. was admitted to the Neurological Department of the Moscow Regional Center for Maternity and Childhood Protection with complaints of sharp pronounced weakness in the limbs, inability to walk. According to the disease history, the patient had an acute respiratory viral infection in October 2022. The conducted neurologic examination revealed decreased muscle tone and muscle strength in all limbs, absence of reflexes from the lower limbs. A liquor test was conducted, which revealed increased protein contents. Electroneuromyography detected a pronounced axonal lesion of all motor fibers in the arms and legs. The formulated diagnosis was “Guillain-Barré syndrome, variant of acute motor axonal neuropathy.” The treatment with plasmapheresis and intravenous immunoglobulins showed positive dynamics. In the second case, patient D. was admitted to the Pediatric Infectious Disease Department of Naro-Fominsk Hospital with similar complaints of sharp weakness in the limbs. Neurological examination showed a diffuse decrease in muscle tone, absence of reflexes from all limbs. Elevated protein contents were determined in the liquor. MRI of the cervical spinal cord showed myelitis of segments C3–C7. Negative antibodies to aquaporin-4 were found. The patient was treated with ceftriaxone, methylprednisolone, and plasmapheresis. Then he was transferred to the Neurological Department of the Moscow Regional Center for Maternity and Childhood Protection, where a repeated MRI of the cervicothoracic spinal cord revealed, in addition to myelitis at the level of C3–C7, a lesion of segments from the level of C2 to the medulla oblongata. The formulated diagnosis was “Neuromyelitis optica spectrum disease, seronegative form. Flaccid tetraparesis.” The continued treatment with prednisolone and intravenous immunoglobulin produced positive effect. Conclusion. The presented clinical cases will help neurologists to improve timely diagnosis and treatment of causes of acute flaccid paralysis in children, thereby reducing possible complications, disability, and mortality.

A study on the role of serum uric acid in differentiating acute inflammatory demyelinating polyneuropathy from acute-onset chronic inflammatory demyelinating polyneuropathy.

Clinical symptoms and laboratory indices for acute inflammatory demyelinating polyneuropathy (AIDP), a variant of Guillain-Barré syndrome, and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) were analyzed to identify factors that could contribute to early differential diagnosis. A retrospective chart review was performed on 44 AIDP and 44 A-CIDP patients looking for any demographic characteristics, clinical manifestations or laboratory parameters that might differentiate AIDP from acutely presenting CIDP. In Guillain-Barré syndrome patients (N = 63), 69.84% (N = 44) were classified as having AIDP, 19.05% (N = 12) were found to have acute motor axonal neuropathy, 6.35% (N = 4) were found to have acute motor and sensory axonal neuropathy, and 4.76% (N = 3) were found to have Miller Fisher syndrome. Serum uric acid (UA) was higher in A-CIDP patients (329.55 ± 72.23 μmol/L) than in AIDP patients (221.08 ± 71.32 μmol/L) (p = 0.000). Receiver operating characteristic analyses indicated that the optimal UA cutoff was 283.50 μmol/L. Above this level, patients were more likely to present A-CIDP than AIDP (specificity 81.80%, sensitivity 81.80%). During the follow-up process, serum samples were effectively collected from 19 AIDP patients during the rehabilitation phase and 28 A-CIDP patients during the remission stage, and it was found that UA levels were significantly increased in A-CIDP (remission) (298.9 ± 90.39 μmol/L) compared with AIDP (rehabilitation) (220.1 ± 108.2 μmol/L, p = 0.009). These results suggest that serum UA level can help to differentiate AIDP from A-CIDP with high specificity and sensitivity, which is helpful for early diagnosis and guidance of treatment.

Factors Associated with Respiratory Insufficiency in Children with Guillain-Barré Syndrome.

The risk factors for respiratory insufficiency in children with Guillain-Barré syndrome (GBS) are poorly known. This study aimed to investigate the factors associated with respiratory insufficiency in children with GBS. This retrospective study included children diagnosed with GBS by pediatric neurologists and admitted at the Wuhan Children's Hospital and other hospitals from January 2013 to October 2022. The patients were divided into the respiratory insufficiency and nonrespiratory insufficiency groups according to whether they received assist breathing during treatment. The median (interquartile range) age of onset of 103 patients were 5 (3.1-8.5) years, 69 (67%) were male, and 64 (62.1%) had a history of precursor infection. Compared with the nonrespiratory insufficiency group, the respiratory insufficiency group showed more facial and/or bulbar weakness (p = 0.002), a higher Hughes Functional Grading Scale (HFGS) at admission (p < 0.001), and a shorter onset-to-admission interval (p = 0.017). Compared with the acute motor axonal neuropathy (AMAN) subtype, the acute inflammatory demyelinating polyneuropathy (AIDP) subtype showed longer days from onset to lumbar (p = 0.000), lower HFGS at admission (p = 0.04), longer onset-to-admission interval (p = 0.001), and more cranial nerve involvement (p = 0.04). The incidence of respiratory insufficiency between AIDP and AMAN showed no statistical difference (p > 0.05). In conclusion, facial and/or bulbar weakness, HFGS at admission, and onset-to-admission interval were associated with respiratory insufficiency and might be useful prognostic markers in children with GBS.

Pattern of Clinical Profile and Outcome of Gillian Barre Syndrome in Sudanese Patients

Introduction: Guillian Barre'-Syndrome (GBS) is an acute, autoimmune disease affecting spinal roots and peripheral nerves, characterized by a rapidly progressive, ascending weakness of the extremity, trunk and even respiratory and facial muscles and with minor sensory and autonomic dysfunction. As in Sudan there is no enough data regarding the pattern of presentation and outcome of GBS, our research aimed to study clinical profile, electrophysiological variant, and outcome of Sudanese patients diagnosed with (GBS). Methods: A multi centers cross-sectional hospital-based study conducted in Khartoum from March to September 2020, 62 patients enrolled in the study, structured questionnaire consists of personal data, clinical history, examination findings, electrophysiological study result, management and outcome is used to assess the clinical profile and outcome of the disease. Results: The study revealed that males and females are equally represented, 70% patients are between 18 years and 50 years. The onset of the weakness was less than or equal 3 days in 33.9% of patients, from 4 to 7 days in 41.9%, from 8 to 14 days in 11.3% and above 14 days in 12.9%.40.3% patients had Evidence of autonomic dysfunction. The majority of patients presented with weakness that started in the lower limbs (93.5%), in (4.8%) the weakness started in the upper limbs and then descend. In (1.6%) the weakness started in the pharyngeal and respiratory muscles from the start. Sensory complains were observed in 54 patients (87.1%), 3 patients (4.8%) developed urinary bladder incontinence and 36 patients (58.1%) had back pain at the onset of the disease. On clinical examination; (98.4%) of patients had absent reflexes, (35.5%) had bilateral facial nerve palsy and (4.8%) had unilateral facial nerve pals. Based on the electrophysiological studies, (37.09 %) were recorded as acute motor axonal neuropathy (AMAN), (29.03%) as acute inflammatory demyelinating poly neuropathy (AIDP) and (29.03%) as acute motor-sensory axonal neuropathy (AMSAN). Regarding the outcome, 30 patients (48.4%) fully recovered, 25 patients (40.3%) recovered with motor deficit, and 7 patients (11.3%) unfortunately died. Conclusion: This study concluded that Guillian Barre'-Syndrome (GBS) in Sudanese population mainly affects age group from 18 to 50 years with variable maximum onset of weakness, presentation and outcome. Lower limb weakness and absent reflexes were the most presenting features. NCS showed axonal type of GBS found to be in nearly 66% of patients and 40% showed clinical evidence of dysautonomia.

Reversible Conduction Failure in Guillain-Barré Syndrome

Gangliosides are the primary antigenic target in acute motor axonal neuropathy. When anti-ganglioside antibodies bind to gangliosides near the motor axon node of Ranvier, complement system activation and Wallerian degeneration occur. Nevertheless, debate persists regarding the impact of anti-ganglioside antibodies on Guillain-Barré syndrome (GBS). Certain patients with these antibodies experience a swift recovery or exhibit conduction abnormalities indicative of demyelination in nerve conduction studies. The concept of reversible conduction failure was introduced by Kuwabara et al. in 1998. They proposed that this could result from compromised physiological conduction at the node of Ranvier. Auto-antibodies that bind to GM1 or GD1a gangliosides at this node can activate the complement system and disrupt sodium channel and axo-glial junctions, causing conduction failure. In 2003, Cappaso et al. described two cases of rapidly improving flaccid paralysis following Campylobacter jejuni infection. Initial nerve conduction studies indicated motor conduction block, which resolved quickly within 2 to 5 weeks. The authors termed this phenomenon acute motor conduction block neuropathy and considered it a form of arrested or partial acute motor axonal neuropathy. Since acute motor conduction block neuropathy could be misclassified as acute inflammatory demyelinating polyneuropathy based on existing electrophysiological criteria, several suggestions were made to refine the classification of GBS subtypes.

Nodopathy: clinic, diagnosis, treatment. Clinical description

AIM: Evaluation of the significance and possibilities of laboratory-instrumental diagnostic methods in establishing the diagnosis and selection of targeted therapy in patients with nodopathies.&#x0D; MATERIALS AND METHODS: System analysis of data from foreign and domestic literature with the presentation of a clinical case.&#x0D; RESULTS: Polyneuropathies are classified as demyelinating or axonal based on electrophysiological studies. However, in 2015, in addition to axonal and demyelinating neuropathies, it was proposed to distinguish a separate pathophysiological group — nodopathies. The pathogenesis of nodopathies may differ depending on the type of ion channels involved in the process, but always leads to a loss of excitability of the axon membrane; in the nodal region the membrane becomes inexcitable. Such neuropathies are characterized by transient conduction blocks followed by the development of axonal degeneration. Typical examples of nodopathies are acute motor axonal neuropathy, as well as multifocal motor neuropathy. Current pathophysiological understanding of specialized nodal regions (nodes of Ranvier) and associated axoglial proteins is growing. Hypotheses have been put forward about their role in the pathogenesis of immune-mediated attack on the peripheral myelinated axon. Recently, high titers of antibodies directed against a number of key adhesion molecules have been identified in both acute and chronic inflammatory neuropathies. These facts add to the differences in differential diagnosis between axonal and demyelinating peripheral neuropathies. New disease classification schemes based on seropositivity, improved electrophysiological and ultrasound classification, and identification of putative underlying pathological targets and mechanisms are being rapidly developed.&#x0D; CONCLUSION: Using our clinical example, we demonstrated the capabilities of laboratory and instrumental diagnostic methods in establishing a diagnosis in a patient with one of the forms of nodopathies — multifocal motor neuropathy.

Dengue-induced Guillain–Barre syndrome: a case series

BackgroundDengue virus is an RNA virus that is associated with a myriad of neurological manifestations. Less than 5% of patients develop neuromuscular complications. Guillain–Barre syndrome (GBS) is an uncommon neurological sequelae of dengue fever. Studies have shown acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) to be the most common variants associated with dengue fever in Asian countries. Very few cases have been reported from Pakistan to date.Case presentationWe present four adult patients with dengue-associated GBS. The time interval between onset of symptoms of dengue fever and development of lower limb weakness in all patients was between 6 and 20 days. Dengue was diagnosed based on either serology or dengue NS1 antigen testing. Acute inflammatory demyelinating polyneuropathy (AIDP) (n = 1), AMAN (n = 1), and AMSAN (n = 2) variants were identified. One patient had a coexisting myopathy which has not been previously reported. All patients showed good recovery after treatment with plasmapheresis or intravenous immunoglobulins.ConclusionsOur case series is valuable in contributing to the limited pool of reported cases of dengue fever complicated by GBS. Early detection of new onset symmetrical limb weakness during or after a dengue infection is imperative for early treatment and to limit disability.

Systemic Lupus Erythematosus Initially Presenting as Acute Motor andSensory Axonal Neuropathy Variant ofGuillain-Barre Syndrome ina Healthy Active Duty Female.

Guillain-Barre syndrome (GBS) is an acute monophasic immune-mediated polyradiculoneuropathy characterized by rapidly evolving ascending weakness, mild sensory loss, and hypo- or areflexia, typically progressing to peak symptoms over the course of 4 weeks. The precise mechanism is unclear but is proposed to be an immune-mediated reaction with the generation of antibodies against peripheral nerves triggered by a preceding viral infection. Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe variant of Guillain-Barre syndrome with limited published literature. Discussion of risk factors for this subtype has not been done in a systematic way. This case report involves a 34-year-old, active duty, West African female, who immigrated to the United States in 2019. She presented with worsening diplopia, bilateral distal upper and lower extremity paresthesias as well as progressively worsening bilateral upper extremity weakness. Her clinical picture was complicated by constitutional symptoms, diffuse lymphadenopathy, no preceding viral illness, and marked clinical deterioration. Ultimately, she was diagnosed with acute motor and sensory axonal neuropathy in the setting of a new diagnosis of systemic lupus erythematosus, a rarely described association emphasizing the importance of a thorough evaluation for underlying causes of acute neurologic pathologies.

Clinical Profile and Predictors of Outcome of Guillain Barre Syndrome Variants among Children Admitted in the Pediatric Department of a Tertiary Care Hospital, Karachi

Background: Guillain Barre Syndrome (GBS) is one of the leading cause of Acute Flaccid Paralysis presenting in pediatric emergency, marking incidents in Asian countries as 3-4 per 100, 000 population. To improve outcome, an accurate and early recognition of predictors of bad prognosis is required. Objective: To identify requirement of intensive monitoring and aggressive treatment to help the health system in decreasing morbidity and mortality associated with Guillain Barre Syndrome. Materials and Methods: This is a cross-sectional study, conducted at Dr. RKMP Civil Hospital Karachi. Medical records of patients &gt;3 months to 12 years of age, admitted with diagnosis of Guillian Barre syndrome from Jan 2017 to June 2022 were reviewed. Data was analyzed in SPSS version 23. For independent variables, frequency and percentage was used. Results: A total of 46 patients were enrolled in the study, mean age of study participants was 80.26 ± 31.7 months. The most common variant of GBS identified was AIDP in 19 (41.3%) followed by ASMAN 14 (30.4%). A positive association between winter season with 2.7 (95% CI 1.4-5.8) and progression within 7 days with 3.2 (95% CI 1.9-6.7) was found with mortality. Conclusion: Acute inflammatory demyelinating polyneuropathy was the most common variant of Gullian-Barre syndrome with male predominance. Presentation in winter seasons and progression of disease in less than 7 days are risk factors for mortality. Bulbar palsy is an independent risk factor for mechanical ventilation, irrespective of GBS variant. Keywords: Acute inflammatory demyelinating polyneuropathy (AIDP), Guillain Barre Syndrome (GBS), Pediatric population, Bulbar palsay, Areflexia, Acute motor axonal neuropathy (AMAN).

PEDIATRIC GUILLAIN-BARRÉ SYNDROME IN MÉXICO CLINICAL FEATURES BEFORE AND DURING SARS-COV-2 PANDEMIC

Introduction: Guillain-Barré Syndrome is the most common cause of acute flaccid paralysis in childhood. It is a post infectious disease immune-mediated with a rapidly progressive course, usually without relapse. The main features are progressive weakness of more than one limb, areflexia or hyporeflexia, which gets progressively worst over days-to-weeks, to potentially life-threatening severity requiring mechanical ventilation. Objective: We aimed to describe the clinical features of pediatric Guillain-Barré Syndrome in México before and during SARS-CoV-2 pandemic. Methods: We performed an ambispective, observational, cross-sectional study in a Mexican reference hospital from January 2013 to December 2021. Data were obtained through records: demographic, clinical, laboratories, neurophysiological variants and treatment. Results: Here we show that of the 96 patients, 55 were males with mean age of 9 years, 72% had history of infection; progressive weakness was present in 97%, areflexia/hyporeflexia 97%, progression of symptoms 99%, mean cells of 9/mm3 and mean proteins 88 mg/dL. Admission to Pediatric Intensive Care Unit was 20%. Acute Motor Axonal Neuropathy was the most frequent subtype. Nineteen patients required mechanical ventilation. Immunoglobulin was administered in 88%. Most frequent Disability Score at discharge was bedridden/wheelchair-bound. During 2020-2021 we found two SARS-CoV-2 cases and one associated with BNT162b2 vaccine. Conclusions: Our results demonstrate that clinical features of pediatric Guillain-Barré Syndrome are similar before and during SARS-CoV-2 pandemic, nevertheless the number of cases associated with SARS-CoV-2 infection did not find increase.

Comprehensive Rehabilitation for Acute Motor Axonal Neuropathy (AMAN) in a Paediatric Patient: A Case Report and Functional - Based Intervention Approach

Comprehensive Rehabilitation for Acute Motor Axonal Neuropathy (AMAN) in a Paediatric Patient: A Case Report and Functional - Based Intervention Approach

Electrophysiologic Findings in Pediatric Guillain Barre Syndrome

Objective: To describe electrophysiologic findings in the pediatric GuillianBarre syndrome (GBS).&#x0D; Study design: Comparative prospective study.&#x0D; Place and Duration of Study: Department of Neurology, The Children’s Hospital and Institute of Child Health, Lahore Pakistan, from Jun to Dec 2015.&#x0D; Methodology: Children below 18 years of age, presenting in medical emergency/neurology OPD and fulfilling the clinical case definition of GBS were included for the study. Electrophysiologic studies were performed within 24 hours of admission in all patients.&#x0D; Results: Out of 83 patients with GBS, 59% were male and 80% were between 3-12 years of age. According to the electrophysiological findings, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) was the most common subtype followed by acute motor axonal neuropathy (AMAN). Reduced CMAP and absent F-response were the most common electrophysiologic findingspresented in 70% and 57.8% of patients respectively. However, absent F-response was not specific for any subtype (p&gt;0.05). Prolonged motor DL, reduced NCV, temporal dispersion and abnormal F-wave latency were characteristicelectrophysiologic features of demyelination (p≤0.001). However, prolonged motor DL and absent F-wave occurred early in the course of disease while reduced NCV and temporal dispersion observed later.&#x0D; Conclusion: Electrophysiologic studieswere useful in making the appropriate diagnosis to initiate immunotherapy, particularly during first week after onset of weakness when albuminocytologic dissociation may not be present.

Clinical characteristics and management outcomes of Guillain-Barré syndrome: eight-year experience at a tertiary center in jordan - a retrospective cohort study.

This retrospective study looks at adult patients admitted to a major tertiary referral hospital in the north of Jordan between 2013 and 2021. A total of 30 patients met the inclusion/exclusion criteria. Males were predominantly affected (70%) with a male-to-female ratio of 2.33. Acute inflammatory demyelinating polyradiculoneuropathy variant was encountered in 60% of cases, whereas axonal variants, namely, acute motor axonal neuropathy and acute motor axonal and sensory neuropathy variants were seen in about 23% of cases. ICU admission was reported in 37% of patients and 6.7% required mechanical ventilation. Most patients had a favorable outcome with a GBS disability score of three or better at out-patient follow-up visits. Our cohort of patients showed a significant deviation in disease expression from that reported in other parts of the globe. This deviation was obvious in more prominent male predominance, frequencies of different GBS variants, and more favorable short-term morbidity/mortality outcomes. However, larger multicenter prospective studies are needed for confirmation of these results.