Raras
Buscar doenças, sintomas, genes...
Siringomielia
ORPHA:3280CID-10 · G95.0CID-11 · 8D66DOENÇA RARA

A siringomielia é caracterizada por cavidades cheias de líquido cefalorraquidiano (LCR) (siringe) dentro da medula espinhal, seja como resultado de uma causa conhecida (siringomielia secundária, SS) ou, mais raramente, devido a uma causa desconhecida (siringomielia primária, PS).

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Introdução

O que você precisa saber de cara

📋

A siringomielia é caracterizada por cavidades cheias de líquido cefalorraquidiano (LCR) (siringe) dentro da medula espinhal, seja como resultado de uma causa conhecida (siringomielia secundária, SS) ou, mais raramente, devido a uma causa desconhecida (siringomielia primária, PS).

Pesquisas ativas
6 ensaios
18 total registrados no ClinicalTrials.gov
Publicações científicas
4.597 artigos
Último publicado: 2026 Apr 15
Medicamentos
2 registrados
THALIDOMIDE, BETAINE

Tem tratamento?

2 medicamentos registrados
Ver detalhes, fases e interações →
THALIDOMIDEBETAINE

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
8.4
Europe
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: G95.0
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Entender a doença

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
6 sintomas
🦴
Ossos e articulações
3 sintomas
💪
Músculos
2 sintomas
🫘
Rins
1 sintomas
👁️
Olhos
1 sintomas
🫃
Digestivo
1 sintomas

+ 26 sintomas em outras categorias

Características mais comuns

Parestesia
Escoliose
Síndrome de Horner
Sinal de Babinski
Hiperreflexia do membro inferior
Siringomielia
41sintomas
Sem dados (41)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.

ParestesiaParesthesia
EscolioseScoliosis
Síndrome de HornerHorner syndrome
Sinal de BabinskiBabinski sign
Hiperreflexia do membro inferiorLower limb hyperreflexia

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico4.597PubMed
Últimos 10 anos200publicações
Pico2025150 papers
Linha do tempo
2026Hoje · 2026🧪 1992Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 25
1Fase 11
·Pré-clínico11
Medicamentos catalogadosEnsaios clínicos· 2 medicamentos · 15 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Siringomielia

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

4 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

18 ensaios clínicos encontrados, 6 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
1.533 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.533

#1

Morvan syndrome in a patient with progressing metastatic thymoma and myasthenia gravis.

BMJ case reports2026 Mar 19

Morvan syndrome (MoS) is a rare paraneoplastic syndrome that is predominantly characterised by autonomic dysfunction, peripheral nerve hyperexcitability and altered mental status. Cases in literature often describe pre-existing autoimmune diagnoses in patients with MoS, alluding to its association with systemic immune dysregulation.We report a woman presenting with hypersomnolence and fluctuating mental status consistent with MoS who had been previously diagnosed with metastatic thymoma, acetylcholine receptor-positive myasthenia gravis and multiple other chronic medical conditions. The report emphasises the importance of prompt recognition of symptoms and accurate diagnosis in rare diseases, allowing for timely initiation of the appropriate immunotherapy that ultimately led to rapid and significant clinical improvement.

#2

MRI-morphometric characterization of Chiari malformation types 0 and 1 with syringomyelia: implications for diagnosis and pathogenesis.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Mar 13

Primary Chiari malformation type 1 (CM1) and type 0 (CM0) appear to share features of overcrowding of the posterior cranial fossa (PCF) neural elements. We carried out the present study to identify anatomical features differentiating primary Chiari malformation type 1 (CM1) from Chiari malformation type 0 (CM0) and normal subjects, and then used those anatomic features for CM1 and CM0 phenotyping and diagnosis. This study included two groups of adult patients: CM0 with syringomyelia (SM) (CM0-SM) having cerebellar tonsillar descent ≤ 2 mm below the foramen magnum (FM), CM1 with syringomyelia (CM1-SM) with tonsillar herniation (TH) > 3 mm, and normal controls. Clinical data and MRI parameters were analyzed. CM1 and CM0 associated with SM share anatomic characteristics of PCF hypoplasia, size reduction, and flattening, and share clinical findings of occipital headache and cervical central myelopathy. CM1 has more pronounced PCF hypoplasia, narrower FM CSF pathways, and more expansive and extensive syringes than CM0, while CM0 has reduced FM size. Combining two MRI morphometric markers predicted CM1 with a sensitivity of 94%, and three markers predicted CM0 with a sensitivity of 90%. In CM1 and CM0, the descent of the PCF neural structures, assessed by TH and obex descent relative to the FM, correlated with PCF flattening and FM size but not with PCF size. Four PCF morphometric markers independent of TH can accurately predict the presence of CM0 and CM1 associated with syringomyelia. The online version contains supplementary material available at 10.1007/s10072-026-08820-z.

#3

Intraoperative ultrasonography in the surgical management of Chiari I malformation: A systematic review and meta-analysis of outcomes and applications.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Mar 09

Chiari malformation type I (CM-I) is a neurological disorder characterized by cerebellar tonsillar herniation and is often associated with syringomyelia. Posterior fossa decompression (PFD) is the primary treatment, although the optimal extent of decompression remains a topic of debate. Intraoperative ultrasonography (IOUS) offers real-time visualization to support informed surgical decision-making. This study aimed to assess the effectiveness and safety of IOUS-guided surgery in CM-I. This systematic review followed PRISMA guidelines and searched the literature up to July 5, 2025. Studies that utilized IOUS and reported related outcomes were included. A random-effects meta-analysis was employed to pool proportions, and a meta-regression analysis was conducted to explore heterogeneity. Twenty-one non-randomized observational studies, including 1,576 patients, 54.4% of whom were female, were included. The pooled clinical improvement rate was 88% (95% CI: 81%–93%), and the syrinx improvement/resolution rate was 87% (95% CI: 77%–95%). The pooled reoperation rate was 6% (95% CI: 3%–9%), and the complication rate was 6% (95% CI: 3%–8%). Meta-regression revealed that longer disease duration, motor/sensory deficits, and duraplasty were associated with improved syrinx outcomes. IOUS-guided surgery in CM-I is linked to positive clinical and radiological improvements and low complication rates. IOUS can serve as a helpful tool for customizing decompression during CM-I surgery. Larger, prospective, multi-center studies are needed to confirm these findings. [Image: see text] The online version contains supplementary material available at 10.1007/s10072-026-08879-8. 

#4

Pediatric intramedullary spinal tumors: Pathological and clinical outcomes in a 96-case single-institution cohort study.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Feb 02

Pediatric intramedullary spinal cord tumors (PIMSCTs) seriously threaten children's growth and development. This study aims to evaluate the pathological features of PIMSCTs and analyze related factors influencing clinical outcomes. A retrospective analysis was conducted on 96 children with PIMSCT who underwent surgical treatment at our hospital from January 2015 to June 2024. Relevant clinical data were collated to comprehensively analyze clinical and pathological characteristics, and to explore factors potentially affecting prognosis and neurological function. Among the 96 included patients, the thoracic spine was the most common tumor location (n = 43, 44.8%). Most patients presented with mild neurological deficits (MMS Ⅱ) at admission. Overall survival was unrelated to tumor length but correlated with the WHO grade of the tumor. No significant difference in survival was observed between H3K27M mutant and wild-type tumors (p = 0.087), while both Ki-67 and p53 expression were significantly associated with postoperative survival. Short-term postoperative neurological function correlated with tumor length, initial symptoms, and preoperative neurological status. For long-term neurological function, influencing factors included age, WHO grade, syringomyelia, postoperative MMS, and changes in the platelet-to-lymphocyte ratio (PLR). Astrocytoma was the most prevalent pathology in this study. H3K27M mutation did not significantly affect survival in high-grade spinal astrocytoma, while high Ki-67 and p53 expression correlated with poorer prognosis. Tumor length was associated with short-term but not long-term neurological function. Long-term neurological outcomes were mainly linked to inherent tumor properties and postoperative neurological status; postoperative PLR changes may partly indicate long-term neurological function.

#5

A retrospective single-center series on the surgical management and postoperative outcomes of pediatric Chiari malformation type I. Part 1: presentation, operative management, and complications.

Journal of neurosurgery. Pediatrics2026 Jan 16

Posterior fossa decompression is the main surgical treatment for Chiari malformation type I (CM-I). The authors present findings on patient presentation, surgical methods, and postoperative complications from a large cohort of pediatric patients with the aim of utilizing this institutional experience to guide evidence-based management of pediatric CM-I. A departmental operative database was queried for patients who underwent a posterior fossa decompression for CM-I between 1992 and 2021. The charts of identified patients were reviewed. Data regarding demographics, presentation, operative details, and complications were collected. A total of 510 patients were identified. The mean patient age was 10.2 ± 5.2 years, and 57% of patients were female. The most common presenting clinical symptoms or associated signs were exertional suboccipital headaches (65%), syringomyelia (55%), and scoliosis (18%). The mean cerebellar tonsillar descent was 14.2 ± 6.4 mm below the foramen magnum. At surgery, 20% of the patients with a preoperative syrinx had documented arachnoid veils obstructing the outflow tract of the fourth ventricle. Operatively, 99% of patients underwent cervical laminectomy; of these laminectomies, 89% were C1 only. Ninety-seven percent of all patients underwent suboccipital craniectomy with expansile duraplasty (PFDD), while 3% underwent bone-only decompression (PFD). Eighty-seven percent of the patients with PFDD received tonsillopexy and/or tonsillar resection. The median postoperative hospital stay was 2 days for PFD patients and 3 days for PFDD patients (p < 0.01). Postoperative complications developed in 12% of cases, with CSF leakage (5%) and aseptic meningitis (4%) being most common. CSF leakage and aseptic meningitis were also the most common reasons for early readmission after surgery. There were no intraoperative or postoperative deaths. The authors demonstrate an institutional preference for PFDD and tonsillopexy. Exertional suboccipital pain and syringomyelia were the most common indications for surgery. Complications tended to be more common with PFDD. There was a significant difference in postoperative length of stay between patients who underwent PFD (median 2 days) versus PFDD (median 3 days). CSF leakage and aseptic meningitis were the most common reasons for readmission.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC2.775 artigos no totalmostrando 199

2026

Clinical Diversity in Currarino Syndrome: Insights From Monozygotic Twins and a Single-Center Retrospective Study.

Congenital anomalies
2026

Posttraumatic syrinx resulting in syringomyelia and syringobulbia: illustrative case.

Journal of neurosurgery. Case lessons
2026

Morvan syndrome in a patient with progressing metastatic thymoma and myasthenia gravis.

BMJ case reports
2026

Minimally Invasive Tubular Posterior Cervical Laminectomy in Medically Complex Patients: A Case Series With Clinical and Radiographic Outcomes.

Cureus
2026

Complex Type Split Cord Malformation: A Two-Center Study.

Pediatric neurosurgery
2026

Extradural foramen magnum decompression with multiple superficial dural incisions for Chiari I malformation: a multicenter series of 91 pediatric patients.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2026

Thoracic intramedullary spinal cord astrocytoma associated with syringomyelia: a case report of gross total resection.

International journal of surgery case reports
2026

MRI-morphometric characterization of Chiari malformation types 0 and 1 with syringomyelia: implications for diagnosis and pathogenesis.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2026

Large terminal syringomyelia associated with terminal lipoma: mid- to long-term outcomes after tethered cord release.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2026

Therapeutic lumbar puncture as a potential treatment for symptomatic paediatric syringomyelia: A single centre cohort study.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2026

En-bloc posterior vault distraction including the foramen magnum for Chiari I malformation in severe syndromic craniosynostosis: First cases and literature review.

Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery
2026

Association of Posterior Fossa Morphometric Analyses with Cerebrospinal Fluid Flow Parameters in Patients with Chiari Malformation Type 1.

Journal of neurological surgery. Part B, Skull base
2026

Flaccid Lower Limb Paraparesis Despite Infiltration of the Spinal Cord by a Secondary Central Nervous System T-cell Non-Hodgkin Lymphoma With Syringomyelia: A Case Report.

Cureus
2026

Intraoperative ultrasonography in the surgical management of Chiari I malformation: A systematic review and meta-analysis of outcomes and applications.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2026

The Impact of Foramen Magnum Decompression With versus Without Duraplasty on Symptom Improvement and Syrinx Reduction in Chiari 1 Malformation Patients.

Asian journal of neurosurgery
2026

Fatal arachnoiditis following accidental intradural injection of chlorhexidine: a case report and literature review.

Regional anesthesia and pain medicine
2026

The efficacy of surgical management in post traumatic syringomyelia: A systematic review.

The journal of spinal cord medicine
2026

Clinical outcomes of resection of thoracic arachnoid webs: patient series.

Journal of neurosurgery. Case lessons
2026

A rare presentation of CASPR2-associated Morvan syndrome overlapping with GM1-positive AMSAN: a case report.

Frontiers in immunology
2026

Development and validation of the Chiari-like malformation and syringomyelia evaluation: the CHASE questionnaire.

Journal of veterinary internal medicine
2026

The effectiveness of optic nerve sheath diameter in surgical decision-making for type I Chiari malformations.

Journal of craniovertebral junction &amp; spine
2026

Chiari Malformation Type I: A Review of Pathophysiology, Cerebrospinal Fluid Flow Dynamics, Diagnosis, Surgical Management, and Its Relationship to Syringomyelia.

Cureus
2025

Hypomagnesemia as a primary clue for the diagnosis of 17q12 deletion syndrome associated with spinal syringomyelia: a case report.

The Turkish journal of pediatrics
2026

Radioresistant but Alectinib-Responsive Isolated Intramedullary ALK-Positive Histiocytosis.

Case reports in hematology
2026

Pediatric intramedullary spinal tumors: Pathological and clinical outcomes in a 96-case single-institution cohort study.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2026

Spinal Deformity Characteristics Associated With Intraspinal Anomalies in a Population of Presumed Adolescent Idiopathic Scoliosis (AIS).

Journal of pediatric orthopedics
2025

Recurrent neurological blisters restricted to the upper limbs: cutaneous alert of syringomyelia.

European journal of dermatology : EJD
2025

Syringomyelia in the Course of Resection of Foramen Magnum Pathology: A Case Report with an Intriguing Therapeutic Solution and Systematic Review.

Brain sciences
2026

Sinking skin flap syndrome masquerading as Chiari malformation: failed syringomyelia treatment rescued by cranioplasty. Illustrative case.

Journal of neurosurgery. Case lessons
2026

Chiari I Malformation: Review and Update of Current Treatment Options.

Clinical anatomy (New York, N.Y.)
2025

Role of Polysomnography in Tracheostomy Decannulation in Neuromuscular Disease: A Case Report.

Cureus
2025

Subpial cerebellar tonsillectomy for decompression of the cervicomedullary junction in children and young adults.

Journal of neurosurgery. Pediatrics
2026

A retrospective single-center series on the surgical management and postoperative outcomes of pediatric Chiari malformation type I. Part 1: presentation, operative management, and complications.

Journal of neurosurgery. Pediatrics
2026

A retrospective single-center series on the surgical management and postoperative outcomes of pediatric Chiari malformation type I. Part 2: symptomatic outcomes and revision surgery.

Journal of neurosurgery. Pediatrics
2026

Spontaneous vocalisation as a presenting clinical sign in dogs: An association with cervical localisation and intervertebral disc disease.

The Veterinary record
2025

A Rare Case of Posterior Fossa Decompression Performed during Pregnancy for Chiari Malformation Type I.

NMC case report journal
2025

Posterior Fossa Decompression Versus Syringo-Subarachnoid Shunt for Chiari I-associated Syringomyelia: A Systematic Review.

Cureus
2026

Surgical outcomes in Chiari malformation type I: A quality review from a Scandinavian medium-volume neurosurgical center.

Brain &amp; spine
2026

Characterization of additional MRI findings in patients with Chiari spectrum disorders: focus on Chiari 0-1.5 subtypes.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2026

Postoperative Blindness Following Posterior Cervical Surgery: Case Reports.

Neurology India
2026

Chiari type 1 malformations and hypermobility spectrum disorder: clinical outcomes and the importance of the differential diagnosis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Longitudinal Syringomyelia, Cervical Dystonia, and Action Tremor in Trichorhinophalangeal Syndrome Type I - A Case Report.

Tremor and other hyperkinetic movements (New York, N.Y.)
2026

Pediatric triad of craniofacial fibrous dysplasia, Chiari malformation type I and syringomyelia: a case report.

Acta neurochirurgica
2026

A comment on cohort structure in Chiari I malformation studies.

Clinical neurology and neurosurgery
2025

Adhesive Arachnoiditis in a Patient Undergoing Lung Cancer Surgery.

Annals of thoracic surgery short reports
2025

Terminal myelocystocele and the chiari connection: a case-based review with a novel pathophysiological hypothesis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

A Conservative Approach to Clinical Management of Syringomyelia.

Cureus
2025

Cerebellar tonsil remodeling for Chiari I malformation with syringomyelia: cisterna magna volume expansion predicts superior symptom relief.

Neurosurgical review
2025

Tailoring the Surgical Approach to Chiari I Malformation with Intraoperative Ultrasounds: Advantages, Limitations, and Controversies.

Pediatric neurosurgery
2025

Scoliosis associated with Chiari I malformation after posterior fossa decompression: a systematic review and meta-analysis of 380 pediatric patients.

Spine deformity
2025

Comparison of Intraspinal Abnormalities Prevalence in Congenital Scoliosis: Is Multiple Hemivertebra Associated with Higher Risk than Single Hemivertebra?

JB &amp; JS open access
2025

Cohort analysis of surgically managed Chiari malformation type 1: real-world data from multi-centres.

Neurosurgical review
2026

Assessment of Cerebrospinal Fluid Flow Patterns Using Time-Spatial Labeling Inversion Pulse Sequence in a Dog With Chiari-Like Malformation and Syringomyelia.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2026

Video-Polysomnography in Peripheral Nerve Hyperexcitability: Clues to Morvan Syndrome in Two Patients and Literature Review.

Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
2026

Demonstration of the importance of MRI in preoperative evaluation of adolescent idiopathic scoliosis.

Spine deformity
2025

Outcomes of 87 small-breed dogs surgically treated for Chiari-like malformation and syringomyelia.

Veterinary surgery : VS
2026

Cerebellar tonsil manipulation in Chiari malformation type I surgery: A systematic review and meta-analysis.

Clinical neurology and neurosurgery
2025

Diagnostic utility of additional spine imaging for syrinx detection in the pre-operative assessment of pediatric Chiari malformation type 1.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

The management of pediatric Chiari I malformation with concomitant hydrocephalus: a multicenter retrospective analysis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Intraoperative Ultrasound in Brain and Spine Surgery: Current Applications, Translational Value and Future Perspectives.

NeuroSci
2025

Early-Onset Syringomyelia: A Rare Complication of TB Meningitis.

Cureus
2025

Paediatric isolated foot drop-a rare presentation of Chiari 1 malformation with holocord syrinx (case report and the review of literature).

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Unraveling the Diagnostic Challenge of Arachnoiditis Ossificans in Association with Syringomyelia: A Review of the Literature and Two Case Reports.

Journal of neurological surgery reports
2025

Spatial transcriptomic alterations of the dorsal horn in dogs with neuropathic pain.

Pain reports
2025

Dynamic transition from spinal arachnoid diverticulum to syringohydromyelia in a cat: Correlation between computed tomography myelography and magnetic resonance imaging.

Open veterinary journal
2025

Benediction sign in a patient with intramedullary tumor and syringomyelia.

The Pan African medical journal
2026

Prevalence of sleep-related breathing disorders in children with myelomeningocele and other spinal dysraphisms and risk factors.

Sleep medicine
2025

A 3D non-human primate digital model for pharmacokinetic prediction of intra-cerebrospinal fluid drug neuraxial dispersion.

Fluids and barriers of the CNS
2025

Chiari III malformation with ruptured encephalocele: a case report and review of literature.

Annals of medicine and surgery (2012)
2025

Surgical failures and revision strategies in Basilar invagination: an exploratory analysis of 145 published cases.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

Diagnostic and Prognostic Value of Serum Neurofilament Light Chain in Canine Spinal Cord Diseases.

Veterinary sciences
2025

Matched analysis of traction-assisted posterior spinal correction in severe scoliosis with and without syringomyelia.

Journal of orthopaedic surgery and research
2025

Spina bifida, diplomyelia, and Chiari-like malformation in an Aberdeen Angus calf.

BMC veterinary research
2025

Case Report: 3D-CISS and PC-MRI in the diagnosis and surgical planning of hydrocephalus secondary to presumptive lateral aperture obstruction in a dog.

Frontiers in veterinary science
2025

Predictive potential of radiographic characteristics on outcomes after Chiari Decompression surgery: A meta-analysis of the literature.

Clinical neurology and neurosurgery
2025

A Conundrum of Colliding Conditions: A Histopathological Case Report of Chiari Type III with Complete Spina Bifida Aperta.

Reports (MDPI)
2025

Could Enlarged Sagittal Cervical Canal Diameter in Conventional Plain Lateral Radiographs Indicate the Presence of Syringomyelia? A Propensity Score Matched Study.

Clinical spine surgery
2025

Efficacy of neurosurgical intervention in syrinx resolution in patients presenting with Chiari malformation type I and syringomyelia: a systematic review and radiological meta-analysis.

Neurosurgical review
2025

A Novel Clinical Insight Into Idiopathic Syringomyelia With Occult Arachnoid Webs: Neuropathological Features, Differential Diagnosis, and Surgical Strategy.

Neurospine
2025

Surgical technique for executing an en bloc resection of a multilevel cervical intramedullary ependymoma: A 2D operative video.

Surgical neurology international
2025

Neuropathic Arthropathy of the Shoulder Secondary to Syringomyelia.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2025

Chiari 1 Malformation, Factors That May Influence Decision Making, and Introducing the Chiari 1 Malformation Severity Classification System.

Journal of clinical medicine
2025

Naked sacrococcygeal teratoma associated with dorsal meningocoele and sacrococcygeal inversion: an atypical presentation of Currarino syndrome.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2026

Syringomyelia in Australian Cavalier King Charles spaniels: prevalence and changes over 16 years of magnetic resonance imaging screening.

Australian veterinary journal
2025

Quality-of-Life Outcomes in Adults and Children With Chiari 1 Malformation and in Those Managed Without Surgery: A Prospective, Multicenter, Observational Study.

Neurosurgery
2025

Spinal cord malformations's epidemiology in French children: National cross sectional study based on medico-administrative database.

Neuro-Chirurgie
2025

Current Approach and Predictors of Clinical Outcomes in Adults With Spinal Ependymomas.

Cureus
2025

Prevalence of Neural Axis Abnormalities in Typical and Atypical Laterality Idiopathic Scoliosis: A Systematic Review and Meta-Analysis of Observational Studies.

Spine
2025

The bregmatic fontanelle alone is an unreliable indicator of health in Pomeranian and other toy-sized dogs.

Schweizer Archiv fur Tierheilkunde
2025

Syrinx in spontaneous intracranial hypotension treated by surgical disconnection of CSF venous fistula.

Practical neurology
2025

Minimally Invasive Decompression for Chiari I Malformation: A Systematic Review and Meta-Analysis.

Cureus
2025

Idiopathic Syringomyelia: Diagnostic Value of Cranial Morphometric Parameters.

Brain sciences
2025

Risk factors associated with rapid progression of scoliosis following intraspinal lesion resection in laminoplasty patients.

Neurosurgical review
2025

Favorable clinical outcomes and complications of endoscopic third ventriculostomy in Chiari Malformation Type I: A systematic review and meta-analysis.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

Pollakiuria: a rare and unique mode of revelation of silent dorsal syringomyelia in a young man-a case report.

Oxford medical case reports
2025

Subarachnoid hemorrhage secondary to Chiari 1 malformation in a patient presenting with recurrent severe headache: A case report, imaging, and anatomic considerations.

Surgical neurology international
2025

Cough syncope and central sleep apnea caused by CM1 with atlas assimilation in adult: An illustrative case.

Surgical neurology international
2025

Fourth ventricular stenting in foramen magnum decompression for type 1 Chiari malformations with syringomyelia.

Surgical neurology international
2025

Mini-open exoscopic surgery for C1 restenosis 7 years after decompression of Chiari malformation type 1 with atlas assimilation.

Surgical neurology international
2025

Follow-up of syringomyelia due to Chiari type I malformation with phase-contrast magnetic resonance imaging in a professional athlete.

Surgical neurology international
2025

Betaine: A Promising Natural Product for Neurological and Psychiatric Diseases.

Current neuropharmacology
2025

Charcot arthropathy of the shoulder joint following Arnold-Chiari malformation: A case report.

International journal of surgery case reports
2025

Arachnoid web-a rare but surgically effectively treatable cause of spinal cord compression and syringomyelia.

Brain &amp; spine
2025

A novel Minimally-Invasive technique for Non-Traumatic postoperative adhesive Syringomyelia.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

The effect of atlantoaxial stabilization on juvenile idiopathic scoliosis involving Chiari malformation and syrinx.

Journal of craniovertebral junction &amp; spine
2025

Pediatric Craniovertebral Junction Anomalies: A Literature Review.

Cureus
2025

Chiari-like Malformation and Syringomyelia in Pomeranians: A Longitudinal Study.

Veterinary sciences
2025

Partially calcified giant hemorrhagic syringomyelia and hematomyelia.

Journal of clinical imaging science
2025

Is "routine" magnetic resonance imaging necessary in adolescent idiopathic scoliosis? A retrospective analysis in New Zealand.

Asian spine journal
2025

Syringobulbia and Syringomyelia Associated with Intramedullary Ependymoma.

World neurosurgery
2025

The Efficacy of Atlantoaxial Facet Joint Distraction and Fixation and Foramen Magnum Decompression for Chiari Type 1 Malformation with Basilar Invagination: A Case Report.

NMC case report journal
2025

Resolution of tonsillar herniation and syringomyelia after resection of supratentorial large meningioma.

Brain &amp; spine
2025

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Journal of neurology, neurosurgery, and psychiatry
2025

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Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Effect of Dynamin-2 Gene Modified OECs on Functional and Molecular Neuroprotection After Spinal Cord Injury in Rats.

Neurochemical research
2025

Chiari I malformation with syringomyelia: Exploring novel dermatological markers in pediatrics.

Surgical neurology international
2026

Pediatric Arnold-Chiari I Malformation With Syrinx Presenting With Unilateral Hand Weakness: A Case Report.

Hand (New York, N.Y.)
2025

Not The Classic Waxing and Waning Multiple Sclerosis: A Case Report.

Journal of Brown hospital medicine
2025

Vertebral Metastasis With Syringomyelia Secondary to Breast Adenocarcinoma: A Case Report and Literature Review.

Clinical case reports
2025

Resolution of tension pseudomeningocele complicating foramen magnum decompression for Chiari I malformation after ventriculoperitoneal shunt: A case report.

International journal of surgery case reports
2025

A comparison of prone versus sitting position for the surgical treatment of Chiari malformation type I in children.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

Syringomyelia Associated with Magendie's Foramen Obstruction Due to Probably Congenital Gliomesenchymal Anomaly.

NMC case report journal
2025

Exploring Telitacicept for Neurological Autoimmune Disorders: A Case Study on Morvan Syndrome.

The American journal of case reports
2025

Sacrococcygeal Teratomas in Currarino Syndrome: A Multicenter Review of Tumor Characteristics, Surgical Outcomes, and Recurrence.

Journal of pediatric surgery
2025

First-In-Human Application of Human Umbilical Cord-Derived Extracellular Vesicles in Tethered Spinal Cord Release Surgery.

Journal of extracellular vesicles
2025

Facial onset sensory-motor neuronopathy: diagnostic challenges and insights from a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

Effectiveness of conus lipoma surgery-a case series.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari - I malformation: A case report.

Radiology case reports
2025

Clinical outcome of different surgical approaches for symptomatic Chiari malformation without syringomyelia: a 13-year retrospective study.

Neurosurgical review
2025

A Case Report of an Adverse Outcome: Development of a Dural Arteriovenous Fistula Following Foramen Magnum Decompression for Chiari Malformation.

Cureus
2025

Decompression with Intradural Dissection for the Chiari Malformation Type I: Toward Eliminating Wound Complications.

World neurosurgery
2025

Computational and biological modeling of IGF1R inhibition for multifocal medulloblastoma.

Communications medicine
2025

Dynamic Magnetic Resonance Imaging Changes in Spinal Ependymomas and Their Impact on Surgical Planning.

Neurologia medico-chirurgica
2025

Adult-Onset Syringomyelia in Dandy-Walker Malformation Patients: Clinical Insights and Literature Review.

Brain sciences
2025

Nasopharyngeal Carcinoma with Spinal Cord Metastasis and Secondary Syringomyelia: A Case Report.

Acta neurologica Taiwanica
2025

Intradural-Intramedullary Spinal Teratoma with Syrinx Formation: A Case Report.

Clinical medicine insights. Case reports
2025

Orthopedic Manifestations of Syringomyelia: A Comprehensive Review.

Journal of clinical medicine
2025

Atlantoaxial instability in Chiari formation- an analysis based on static and dynamic head imaging in 35 patients.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

The impact of surgical and medical treatment on headaches in pediatric patients with Chiari malformation type I.

Journal of neurosurgery. Pediatrics
2025

Adult Chiari Type I Malformation Presenting as Emergent Acute Respiratory Insufficiency: A Case Report and Literature Review.

Cureus
2025

Basilar Invagination Diagnosis, Classification, and Radiology: WFNS Spine Committee Recommendations.

Spine
2025

Genomic analyses in Cavalier King Charles spaniels identify loci associated with clinical signs of Chiari-like malformation and Syringomyelia.

BMC veterinary research
2025

Cerebrospinal Fluid Concentrations of Calcitonin Gene Related Peptide in Dogs With Chiari-Like Malformation.

Journal of veterinary internal medicine
2025

Clinical phenotypes among patients with familial forms of Chiari malformation type 1.

Journal of neurosurgery. Pediatrics
2025

Syringomyelia Charcot-arthropathy involving the joints of both hands.

Clinical rheumatology
2025

Epidemiological Data, Clinical Signs, Therapy and Outcome Evaluation in Dogs with Syringomyelia of Different Etiology.

Veterinary sciences
2025

Difference in clinical presentation and surgical outcomes in pediatric and adult patients with Chiari malformation type 1: a single center retrospective study.

Acta neurochirurgica
2025

Successful treatment of morvan syndrome with efgartigimod: report of two cases.

Journal of neurology
2025

Moebius Syndrome associated with syringomyelia: a case report.

Einstein (Sao Paulo, Brazil)
2025

Substantiated degeneration of rectus capitis posterior minor muscle in Chiari malformation I based on multimodal observations: a prospective cohort study.

Neurosurgical review
2025

Posterior Fossa Decompression Followed by Duraplasty with Arachnoid-Preserving Technique for Primary and Recurrent Adult Chiari Malformation Type-1.5: A Comparative Retrospective Study.

Journal of neurological surgery. Part A, Central European neurosurgery
2025

Surgical Strategy Analysis of Chiari Malformation With or Without Type II Basilar Invagination According to the Morphological Types of the Atlanto-Occipital Joint: A Retrospective Study of 212 Patients.

Neurospine
2025

The Effectiveness of Amitriptyline and Gabapentin in Treating Pomeranians with Chiari-like Malformation and/or Syringomyelia.

Animals : an open access journal from MDPI
2025

Skull Base and Craniocervical Anomalies in Syndromic Craniosynostosis.

Neurosurgery
2025

Chiari malformation type I with extensive syringomyelia presenting as progressive bilateral foot drop: a case report.

Korean journal of family medicine
2025

A silent malformation: Closed spinal dysraphism in a boy with urinary incontinence.

Radiology case reports
2025

Posterior-only hemivertebra bone-disc-bone osteotomy (BDBO) without internal fixation in a 15-day-old neonate with 18-year follow-up.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

Pediatric Spine Ultrasound: Comprehensive Review and Systematic Approach.

Canadian Association of Radiologists journal = Journal l'Association canadienne des radiologistes
2025

Can posterior fossa decompression alone effectively treat Chiari malformation type I patients with even severe syringes? Illustrative cases.

Journal of neurosurgery. Case lessons
2025

[A case of amyotrophic lateral sclerosis complicated by syringomyelia associated with Chiari type I malformation].

Rinsho shinkeigaku = Clinical neurology
2025

Rare Masqueraders of Pure Neuritic Leprosy: A Report of Four Cases.

Indian dermatology online journal
2025

Surgical Correction for Adolescent Idiopathic Scoliosis : A Case Series of 139 Consecutive Patients Treated in Neurosurgical Department.

Journal of Korean Neurosurgical Society
2025

A new concept and surgical approach for Chiari malformation type I based on the protection and strengthening of the myodural Bridge.

Scientific reports
2025

Acute Progressive Spinal Arachnoid Web Without the Scalpel Sign: A Case Report.

Cureus
2025

Clinical efficacy of surgery for patients with Chiari malformation type I with syringomyelia: posterior fossa decompression versus posterior fossa decompression with resection of tonsils.

Frontiers in neurology
2025

Denervation of rectus capitis posterior minor as neglected factor in Chiari malformation type I revealed by double blinded prospective study.

Scientific reports
2025

CT- and MRI-based volumetry for Chiari-like malformation and syringomyelia in Pomeranians.

Frontiers in veterinary science
2025

TNF-α mediates activation of neuronal necroptotic signaling in rat with syringomyelia.

Experimental neurology
2025

A review of cerebrospinal fluid circulation with respect to Chiari-like malformation and syringomyelia in brachycephalic dogs.

Fluids and barriers of the CNS
2024

Atlantoaxial fixation for treatment of syringobulbia and syringomyelia: Case report and literature review.

Journal of craniovertebral junction &amp; spine
2025

Morphometric Evaluation of Posterior Fossa in Patients Operated Due to Chiari Malformation Type 2.

World neurosurgery
2025

A Rare Course of Chiari Malformation With Large Syringomyelia Presenting at 54 Years Old.

Cureus
2025

A Prospective Study of CSF Flow Dynamics Across Foramen Magnum in Adult Chiari Malformation/Syringomyelia Complex and its Clinical Correlation with Outcomes after Surgery.

Asian journal of neurosurgery
2025

A Novel Surgical Technique for Post-traumatic Syringomyelia Progressing to the Medulla Oblongata: Evidence of Upward Drainage of Central Canal Fluid Within the Spinal Cord.

Neurosurgery
2025

Ependymal cilia decline and AQP4 upregulation in young adult rats with syringomyelia.

Fluids and barriers of the CNS
2025

Phase-contrast magnetic resonance imaging-based predictive modelling for surgical outcomes in patients with Chiari malformation type 1 with syringomyelia: a machine learning study.

Clinical radiology
2025

A novel minimally invasive surgical technique for posttraumatic syringomyelia: subarachnoid-subarachnoid bypass.

Journal of neurosurgery. Spine
2025

Impact of intradural abnormalities on symptoms and clinical outcomes in Chiari malformation.

Clinical neurology and neurosurgery
2025

Myoclonic Seizures After Endoscopic Cervical Disc Surgery: A Rare Complication or Just Coincidence?

Asian journal of endoscopic surgery
2025

The range of motion characteristics of atlantoaxial joints with the "sandwich" deformity: a human cadaveric biomechanical study.

Neurosurgical review
2025

Indications for Surgery and Surgical Options in Chiari Malformation: WFNS Spine Committee Recommendations.

Spine
2025

Pediatric Chiari Malformation Management: WFNS Spine Committee Recommendations.

Spine
2025

Maternal, Clinical, and Radiographic Outcomes: A Retrospective Analysis of Anesthesia and Delivery Modality in Chiari I Malformation.

Neurosurgery
2024

A data driven approach for soft tissue biomarker identification linked to Chiari-like malformation and syringomyelia.

Frontiers in veterinary science
2025

Using Artificial Intelligence to Identify Three Presenting Phenotypes of Chiari Type-1 Malformation and Syringomyelia.

Neurosurgery
2025

Destructive arthropathy of the shoulder secondary to syringomyelia.

Joint bone spine
2025

Clinical and surgical characteristic of atlantoaxial reduction and fixation for patients with syringomyelia caused by atlantoaxial dislocation: retrospective study of 101 cases.

The spine journal : official journal of the North American Spine Society
2025

Which type of duraplasty is best for Chiari type I malformation surgery?

Neurosurgical focus
2025

Remdesivir-induced severe hypoglycemia in an elderly man without diabetes: a case report.

Journal of pharmaceutical health care and sciences
2025

Effectiveness of Cerebellar Tonsillectomy Treatment for Revision Chiari Malformation Surgery: A Series of 63 Patients.

World neurosurgery
2024

Syringomyelia with Central Canal Enlargement Caused by Postoperative Inflammation after Surgery for Intradural Lumbar Disc Herniation.

NMC case report journal
2024

Arnold-Chiari Malformation Type I Presenting With Bilateral Scapular Winging.

Cureus
2024

Holocord syringomyelia in 18 dogs.

Frontiers in veterinary science
2024

Case report: Electrical impedance tomography-guided ventilator weaning in an obese patient with severe pneumonia.

Frontiers in medicine
2025

Anesthetic Management of a Patient With Arnold Chiari Malformation and Syringomyelia During Laparoscopic Surgery With Neurologic Monitoring: A Case Report.

A&amp;A practice
2024

Scoliosis in adult Type 1 Chiari malformation with syringomyelia patients: from pathogenesis to treatment.

American journal of translational research
2025

One is not like the other: health quality of life scores vary in neuromuscular EOS by diagnosis.

Spine deformity
2025

Chiari Malformation Type I and Syringomyelia: Outcomes of Arachnoid-Preserving Surgical Technique.

Medical science monitor : international medical journal of experimental and clinical research
2025

Incidence of idiopathic syrinx in pediatric patients diagnosed with VACTERL association.

Journal of neurosurgery. Pediatrics
Ver todos os 2.775 no EuropePMC

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Doenças relacionadas

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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.

  1. Morvan syndrome in a patient with progressing metastatic thymoma and myasthenia gravis.
    BMJ case reports· 2026· PMID 41856686mais citado
  2. MRI-morphometric characterization of Chiari malformation types 0 and 1 with syringomyelia: implications for diagnosis and pathogenesis.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41824146mais citado
  3. Intraoperative ultrasonography in the surgical management of Chiari I malformation: A systematic review and meta-analysis of outcomes and applications.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41796392mais citado
  4. Pediatric intramedullary spinal tumors: Pathological and clinical outcomes in a 96-case single-institution cohort study.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41622362mais citado
  5. A retrospective single-center series on the surgical management and postoperative outcomes of pediatric Chiari malformation type I. Part 1: presentation, operative management, and complications.
    Journal of neurosurgery. Pediatrics· 2026· PMID 41569681mais citado
  6. Impact of Additional Tonsillar Manipulation or Intra-articular Distraction on Syrinx Remission for Type B Basilar Invagination.
    Neurosurgery· 2026· PMID 41983700recente
  7. Letter: Using Artificial Intelligence to Identify Three Presenting Phenotypes of Chiari Type 1 Malformation and Syringomyelia.
    Neurosurgery· 2026· PMID 41983692recente
  8. Nursing care for a patient with syringomyelia complicated by bacterial pneumonia and severe pressure injury: a case report.
    Front Med (Lausanne)· 2026· PMID 41952902recente
  9. Extensive reconstruction of cisterna magna facilitates the outcome of adults with Chiari malformation type 1: A multicenter cohort study.
    Clin Neurol Neurosurg· 2026· PMID 41950723recente
  10. MRI-derived three-dimensional modelling reveals cervicothoracic subarachnoid space narrowing in syringomyelia-affected cavalier king charles spaniels.
    BMC Vet Res· 2026· PMID 41923078recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3280(Orphanet)
  2. MONDO:0017987(MONDO)
  3. GARD:7725(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q1112977(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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Siringomielia
Compêndio · Raras BR

Siringomielia

ORPHA:3280 · MONDO:0017987
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
G95.0 · Siringomielia e siringobulbia
CID-11
Ensaios
6 ativos
Medicamentos
2 registrados
Início
All ages
Prevalência
8.4 (Europe)
MedGen
UMLS
C0039144
EuropePMC
Wikidata
Wikipedia
Papers 10a
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