A neuropatia axonal sensório-motora aguda (AMSAN) é uma forma sensório-motora, axonal do síndrome de Guillain-Barré (GBS; ver este termo).
Introdução
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A neuropatia axonal sensório-motora aguda (AMSAN) é uma forma sensório-motora, axonal da síndrome de Guillain-Barré (GBS; ver este termo).
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Publicações mais relevantes
A rare presentation of CASPR2-associated Morvan syndrome overlapping with GM1-positive AMSAN: a case report.
Morvan syndrome is a rare autoimmune disorder characterized by peripheral nerve hyperexcitability with autonomic and central nervous system involvement, most commonly associated with antibodies against contactin-associated protein-like 2 (CASPR2). Acute motor and sensory axonal neuropathy (AMSAN) is an axonal variant of Guillain-Barré syndrome linked to anti-ganglioside antibodies and often manifests as severe limb weakness. Their concurrent presentation is unusual and raises the possibility of shared immune targets within peripheral nerve microdomains. A 70-year-old man presented with a relapsing course of progressive lower-limb weakness accompanied by widespread muscle twitching, severe insomnia with nocturnal hyperarousal, and refractory constipation. He had a prior episode diagnosed as AMSAN that improved after immunotherapy but relapsed four months after treatment was discontinued. Neurological examination demonstrated bilateral lower-limb weakness with reduced tendon reflexes. Moreover, electrophysiological studies confirmed diffuse multifocal peripheral nerve injury with superimposed peripheral nerve hyperexcitability. In addition, immunologic testing revealed serum anti-GM1 antibodies and anti-CASPR2 IgG in both serum and cerebrospinal fluid. Collectively, these findings supported a diagnosis of recurrent AMSAN coexisting with CASPR2-associated Morvan syndrome. Combined immunotherapy with corticosteroids and intravenous immunoglobulin, alongside symptomatic management, resulted in marked clinical improvement. This case report describes a rare overlap of relapsing AMSAN and Morvan syndrome. This antibody-defined coexistence is hypothesis-generating and may reflect synergistic immune injury involving nodal and paranodal regions. This case underscores the importance of recognizing overlapping phenotypes to guide diagnostic profiling and immunomodulatory therapy.
Phenotypic continuum in IGHMBP2-related disorders: a portfolio of cases from typical to Guillain-Barré syndrome-like presentation.
IGHMBP2-related disorders comprise a clinical spectrum from spinal muscular atrophy with respiratory distress type 1 (SMARD1) to Charcot-Marie-Tooth disease type 2S, with increasingly recognized atypical and overlapping phenotypes. We report four pediatric cases from three unrelated families with biallelic pathogenic variants in IGHMBP2. Case 1, a premature infant represents SMARD1. Case 2 had infantile-onset neuropathy without respiratory symptoms. Case 3 and 4, two siblings, presented with a Guillain-Barré syndrome-like phenotype, cauda equina enhancement on spinal neuroimaging, elevated cerebrospinal fluid protein, and electromyography revealing acute motor and sensory axonal neuropathy. Despite an initial response to intravenous immunoglobulin, previous symptoms in Case 3 led to consideration of an immune-mediated neuropathy superimposed on a genetic background. Genetic analysis identified a homozygous nonsense variant in Cases 1 and 2, and novel compound heterozygous missense variants in Case 3 and 4. Thus, the list of overlapping genetic and acquired neuropathies now also includes IGHMBP2-related CMT2S.
EBV and Concomitant Acute Motor and Sensory Axonal Neuropathy in a Healthy 15-Year-Old Female.
Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe form of acute axonal injury caused by immune damage to the axonal membrane. AMSAN is an axonal variant of GBS. GBS occurs from immune injury to the myelin sheath, axonal variants of GBS (AMSAN and AMAN) differ in that insult is to the axonal membrane. AMSAN is seldom seen, especially in pediatric and adolescent patients. Unlike acute motor axonal neuropathy (AMAN), which has been well-described in literature to be secondary to Campylobacter jejuni infection, there is no known etiology of AMSAN. Here, we present a case of an otherwise healthy 15-year-old female who presented with new-onset facial and bulbar weakness that rapidly progressed to functional paralysis requiring intubation. With no clear diagnosis and after failure in improvement with high-dose steroids, Intravenous Immunoglobulin (IVIG), and plasma exchange transfusion, diagnosis was finally made with electromyography (EMG) and nerve conduction study (NCS). In addition, extensive laboratory work was completed and was only notable for primary acute EBV infection. This case represents a new presenting symptom of AMSAN, a unique finding of concomitant primary EBV infection, the possibility of primary Epstein-Barr virus (EBV) infection as the triggering event in AMSAN and stresses the importance of EMG and NCS when evaluating patients with weakness.
Highly Immunoresistant Acute Motor and Sensory Axonal Neuropathy With Concomitant Anti-NF155 Axonal Nodopathy: A Case Report.
Acute motor and sensory axonal neuropathy (AMSAN) is a rare, severe subtype of Guillain-Barré syndrome (GBS) associated with a poorer prognosis and greater resistance to standard immunotherapies. While immunomodulatory therapies are the traditional mainstay therapies for GBS and its variants, as autoimmune-mediated processes, refractory cases can present unique challenges. In addition, chronic inflammatory demyelinating polyneuropathy (CIDP) is a known persistent variant of GBS with its own wide array of variations, including neurofascin IgG4-positive axonal nodopathy, associated with an antibody that provides an additional layer of immunoprotection. This condition has been found in previously documented case series to require non-traditional immunoregimens. We present a unique case of a highly refractory AMSAN with concomitant chronic axonal motor and sensory axonal nodopathy with high multimodal immunotherapy resistance. This case illustrates the importance of considering neurofascin IgG4 in refractory subacute axonal nodopathies and, consequently, the potential role of empiric rituximab, while also highlighting the need for further research into B-cell-depleting therapeutic alternatives in resistant cases.
Antibody Positive Miller-Fisher Syndrome and Acute Motor Sensory Axonal Neuropathy With Respiratory Failure: A Rare Overlap.
Miller-Fisher syndrome (MFS), a rare variant of Guillain-Barré syndrome (GBS), is classically defined by ophthalmoplegia, ataxia, and areflexia and is strongly associated with anti-GQ1b antibodies. We report a 35-year-old man presenting with classical MFS features who rapidly deteriorated with progressive limb weakness and respiratory failure requiring mechanical ventilation. Cerebrospinal fluid analysis showed albuminocytologic dissociation, while nerve conduction studies were consistent with an axonal process. Serologic testing revealed positivity for both anti-GQ1b and anti-GD1a antibodies, confirming an overlap between MFS and acute motor sensory axonal neuropathy (AMSAN). The patient showed marked neurological recovery following intravenous immunoglobulin therapy and intensive supportive care. This case highlights a rare MFS-AMSAN overlap with respiratory failure and underscores the importance of early recognition, vigilant respiratory monitoring, and prompt immunotherapy in preventing morbidity and optimizing patient outcomes in rapidly progressive GBS-spectrum disorders.
Publicações recentes
A rare presentation of CASPR2-associated Morvan syndrome overlapping with GM1-positive AMSAN: a case report.
Phenotypic continuum in IGHMBP2-related disorders: a portfolio of cases from typical to Guillain-Barré syndrome-like presentation.
Highly Immunoresistant Acute Motor and Sensory Axonal Neuropathy With Concomitant Anti-NF155 Axonal Nodopathy: A Case Report.
Antibody Positive Miller-Fisher Syndrome and Acute Motor Sensory Axonal Neuropathy With Respiratory Failure: A Rare Overlap.
A typical presentation of AMSAN variant of guillain-barré syndrome in an elderly female with multisystem involvement: A case report.
📚 EuropePMC30 artigos no totalmostrando 79
A rare presentation of CASPR2-associated Morvan syndrome overlapping with GM1-positive AMSAN: a case report.
Frontiers in immunologyPhenotypic continuum in IGHMBP2-related disorders: a portfolio of cases from typical to Guillain-Barré syndrome-like presentation.
Neuromuscular disorders : NMDHighly Immunoresistant Acute Motor and Sensory Axonal Neuropathy With Concomitant Anti-NF155 Axonal Nodopathy: A Case Report.
CureusAntibody Positive Miller-Fisher Syndrome and Acute Motor Sensory Axonal Neuropathy With Respiratory Failure: A Rare Overlap.
CureusA typical presentation of AMSAN variant of guillain-barré syndrome in an elderly female with multisystem involvement: A case report.
BioinformationGuillain-Barré Syndrome and Variants.
Neurologic clinics[A case report of glycogen storage disease type III combined with Guillain-Barré syndrome and literature review].
Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical geneticsGuillain-Barré Syndrome Following Falciparum Malaria in an 18-Year Male Adolescent: A Rare Case Report and Literature Review.
Clinical case reportsEfgartigimod in the treatment of Guillain-Barré syndrome: case report.
Frontiers in immunologyRare Abdominal Pain Onset in Guillain-Barré Syndrome: A Case Report of Acute Motor Sensory Axonal Neuropathy.
The American journal of case reportsEBV and Concomitant Acute Motor and Sensory Axonal Neuropathy in a Healthy 15-Year-Old Female.
NeuropediatricsGuillain-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.
Medicina (Kaunas, Lithuania)A 'Decrescendo' in a Woman With Ascending Paralysis: A Diagnostic Challenge.
CureusAntineutrophil Cytoplasmic Antibodies Associated Vasculitis Presenting As Neuropathy.
CureusGuillain-barré syndrome (GBS) with antecedent chikungunya infection: a case report and literature review.
Neurological research and practiceConcurrent acute sensorimotor axonal neuropathy and disseminated encephalitis associated with Chlamydia pneumoniae in an adult patient with anti-MOG and anti-sulfatide antibodies: a case report.
Therapeutic advances in neurological disordersA study on the role of serum uric acid in differentiating acute inflammatory demyelinating polyneuropathy from acute-onset chronic inflammatory demyelinating polyneuropathy.
European journal of neurologyEffectiveness of Physiotherapy Intervention in Guillain Barre Syndrome: A Case Report.
CureusReversible splenial lesion syndrome in patient with acute motor and sensory axonal neuropathy.
Clinical neurology and neurosurgerySystemic Lupus Erythematosus Initially Presenting as Acute Motor and Sensory Axonal Neuropathy Variant of Guillain-Barre Syndrome in a Healthy Active Duty Female.
Military medicineSARS-CoV-2 associated acute, motor and sensory, axonal neuropathy requires comprehensive diagnostic work-up.
Neurosciences (Riyadh, Saudi Arabia)Physiotherapy management of a rare variant of Guillain Barre Syndrome, acute motor and sensory axonal neuropathy (AMSAN) along with COVID-19 in a 35-year-old male -a case report.
African health sciencesGuillain-Barré syndrome AMSAN variant in a 90-year-old woman after COVID-19: a case report.
BMC geriatricsGuillain-Barre/Miller-Fisher overlap syndrome or acute, motor and sensory, axonal neuropathy with cranial nerve involvement?
Human vaccines & immunotherapeuticsA Case of Acute Motor and Sensory Axonal Neuropathy Secondary to SGN-LIV1A Therapy.
CureusGuillain-barré syndrome without limb weakness: A rare variant with acute bulbar palsy.
Journal of family medicine and primary careSjogren Syndrome-Associated Autonomic Neuropathy.
CureusSARS-CoV-2 vaccinations reduce the prevalence of post-COVID Guillain-Barre syndrome.
Clinics (Sao Paulo, Brazil)An unusual occurrence of opsoclonus and liver enzymes elevation in a patient with acute motor and sensory axonal neuropathy subtype of Guillain-Barré syndrome.
BMC neurologyGuillain-Barré Syndrome Associated with SARS CoV-2 Infection: Case Report.
Ethiopian journal of health sciencesElectrophysiological Pattern and Predictors of Functional Outcome of Patients with Guillain Barre Syndrome at a Tertiary Care Hospital in Pakistan.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPSevere Guillain-Barré syndrome associated with chronic hepatitis B: A case report and literature review.
MedicineGuillain-Barré Syndrome in an Elderly Patient as a Complication of COVID-19 Infection.
CureusPharyngeal-Cervical-Brachial Variant of Guillain-Barré Syndrome.
CureusElectrophysiologic findings in patients with COVID-19 and quadriparesia in the northwest of Iran, A case series study and literature review.
Caspian journal of internal medicineCovid-19 associated Guillain-Barré syndrome: A series of a relatively uncommon neurological complication.
Diabetes & metabolic syndromeAsymptomatic SARS-CoV-2 infection complicated by acute, motor and sensory, axonal neuropathy (AMSAN).
Clinical neurology and neurosurgeryBilateral Facial Palsy and Hyperreflexia as the Main Clinical Presentation in Guillain-Barré Syndrome.
The American journal of case reportsSARS-CoV-2-associated Guillain-Barré syndrome in four patients: what do we know about pathophysiology?
Acta neurologica BelgicaA variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN.
Ideggyogyaszati szemleNerve ultrasound evaluation of Guillain-Barré syndrome subtypes in northern China.
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Neurological researchAcute motor and sensory axonal neuropathy in association with primary Sjögren's syndrome: a case report.
BMC neurologyAssociation of Guillain-Barre Syndrome With COVID-19: A Case Report and Literature Review.
CureusThe value of sensory nerve conduction studies in the diagnosis of Guillain-Barré syndrome.
Clinical neurophysiology : official journal of the International Federation of Clinical NeurophysiologyClinical and Electrophysiological Outcome Measures of Patients With Post-Infectious Neurological Syndromes Related to COVID-19 Treated With Intensive Neurorehabilitation.
Frontiers in neurologyA case of acute motor and sensory axonal neuropathy mimicking brain death.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyIncreased Cerebrospinal Fluid Uric Acid Levels in Guillain-Barré Syndrome.
Frontiers in neurologyAcute Motor and Sensory Axonal Neuropathy After Mouth Floor Cancer Surgery.
Asian journal of anesthesiologyElectrophysiological subtypes and associated prognosis factors of Mexican adults diagnosed with Guillain-Barré syndrome, a single center experience.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaComparison of the Clinical Outcomes of Guillain Barre Syndrome Based on Electrophysiological Subtypes in Pakistani Children.
CureusAxonal variants of Guillain-Barré syndrome: an update.
Journal of neurologyClinical, electrophysiological findings and evaluation of prognosis of patients with Guillain-Barré syndrome.
The Turkish journal of pediatricsA Rare Case of Acute Transverse Myelitis (ATM) and Acute Motor and Sensory Axonal Neuropathy (AMSAN) Overlap.
CureusClinical and electrophysiological characteristics of Guillain-Barré syndrome in Colombia.
Journal of the peripheral nervous system : JPNSRole of Anti-ganglioside Antibodies in the Diagnosis of Guillain-Barré Syndrome as an Alternate Investigation.
CureusGuillain Barre syndrome with pulmonary tuberculosis: A case series from a tertiary care hospital.
Journal of family medicine and primary careA rare mimic of acute stroke: rapidly progressing Miller-Fisher Syndrome to acute motor and sensory axonal neuropathy variant of Guillain-Barre Syndrome.
BMJ case reportsPulse-steroid therapy in a 37-year-old man with acute motor and sensory axonal neuropathy: A case report.
Clinical case reportsFulminant Guillain-Barré syndrome in a patient with systemic lupus erythematosus.
BMJ case reportsTreatment of an acute motor and sensory axonal neuropathy with propionate in a 33-year-old male.
Therapeutic advances in neurological disordersAcute motor and sensory axonal neuropathy associated with Sjögren's syndrome.
Ideggyogyaszati szemleA Rare Axonal Variant of Guillain-Barré Syndrome following Elective Spinal Surgery.
Case reports in orthopedicsStudy on Electrophysiological Findings of Guillain Barre Syndrome Patients Attending the Department of Neurology in BSMMU.
Mymensingh medical journal : MMJMiller Fisher syndrome, Bickerstaff brainstem encephalitis and Guillain-Barré syndrome overlap with persistent non-demyelinating conduction blocks: a case report.
BMC neurologyA 2-Year-Old Boy With Difficulty Waking After Bone Marrow Transplantation.
Seminars in pediatric neurologySevere Acute Axonal Neuropathy Induced by Ciprofloxacin: A Case Report.
Case reports in neurologyAtypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy.
Frontiers in neurologyGuillain-Barré-like axonal polyneuropathy associated with Toscana virus infection: A case report.
MedicineBrainstem encephalitis and acute polyneuropathy associated with hepatitis E infection.
BMJ case reportsAcute motor and sensory axonal neuropathy related to treatment with MEK inhibitors in a patient with advanced melanoma.
Melanoma researchConcomitant idiopathic hypertrophic spinal pachymeningitis and Guillain-Barré syndrome in a patient: coincidence or a triggering mechanism?
Journal of neurosurgery. SpineTuberculosis and Guillain-Barre syndrome: A chance association?
Intractable & rare diseases researchAn Overlapping Case of Miller Fisher Syndrome, Bickerstaff's Encephalitis, and the ASMAN Variant of Guillain-Barre Syndrome.
Case reports in neurological medicineFinger drop sign in a child with acute motor and sensory axonal neuropathy form of Guillain-Barré syndrome.
Acta neurologica BelgicaGuillain-Barré syndrome with hyperreflexia and bilateral papillitis in a child.
Journal of pediatric neurosciencesSonographic evaluation of peripheral nerves in subtypes of Guillain-Barré syndrome.
Journal of the neurological sciencesGuillain-Barré syndrome in the elderly.
Journal of the peripheral nervous system : JPNSVariant Guillain-Barré Syndrome in a Patient with Non-Hodgkin's Lymphoma.
Case reports in hematologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- A rare presentation of CASPR2-associated Morvan syndrome overlapping with GM1-positive AMSAN: a case report.
- Phenotypic continuum in IGHMBP2-related disorders: a portfolio of cases from typical to Guillain-Barré syndrome-like presentation.
- EBV and Concomitant Acute Motor and Sensory Axonal Neuropathy in a Healthy 15-Year-Old Female.
- Highly Immunoresistant Acute Motor and Sensory Axonal Neuropathy With Concomitant Anti-NF155 Axonal Nodopathy: A Case Report.
- Antibody Positive Miller-Fisher Syndrome and Acute Motor Sensory Axonal Neuropathy With Respiratory Failure: A Rare Overlap.
- A typical presentation of AMSAN variant of guillain-barré syndrome in an elderly female with multisystem involvement: A case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98917(Orphanet)
- MONDO:0020348(MONDO)
- GARD:19601(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55346106(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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