A rare case of Syringomyelia with Arnold � Chiari Malformation Type 1 - A case report

Syringomyelia is a pathologic cystic cavity within the spinal cord containing cerebrospinal fluid (CSF). It is commonly seen as a complication of an Arnold-Chiari type 1 malformation, which is the herniation of cerebellar tonsils through foramen magnum into cervical spinal canal. Syringomyelia can also occur as complication of hemorrhage, tumor, meningitis, arachnoiditis, or trauma. Symptoms usually begin to appear in early and middle life. These symptoms usually consist of pain, dissociated sensory loss and weakness that present and progress gradually. We herewith report a rare case of syringomyelia and associated Chiari I malformation presenting with dissociated sensory impairment in neck region with headache and neck pain Treatment in these cases is surgical decompression. Recovery with significant decrease in sensory loss and relief in headache and neck pain.

Research progress of Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Chiari malformation (CM) is a group of congenital cerebellar tonsillar hernia malformations involving the craniocervical junction. Chiari malformation type I (CMI) is the most common in clinic, however its pathogenesis is still unclear, and there is no consensus on the surgical treatment standard of CMI. At present, the most widely accepted is the theory of posterior fossa incompatibility, so doctors at home and abroad use posterior fossa decompression (PFD) and posterior fossa compression with duraplasty (PFDD) as the gold standard for surgical treatment, and have their own experience and technical improvement. However, the volume of the posterior cranial fossa in some patients is no different from that in healthy people, and about 30% of the patients with CMI have poor results after posterior cranial fossa decompression. As a result, this operation cannot treat all patients with CMI. In recent years, with the development of imaging, the progress of diagnostic technology and the deepening of understanding of CM, some studies have shown that CMI may be related to atlantoaxial instability, and proposed that CMI is the secondary factor of atlantoaxial instability, and atlantoaxial fusion is the standard of surgical treatment, which has caused great controversy in academic circles. Different clinical research results of scholars support or oppose this theory: some studies have shown that the clinical symptom relief rate of patients with CMI treated with atlantoaxial fusion is 96.9%; another study showed that 70% of patients with CMI underwent atlantoaxial fusion had improved neurological function, but the overall postoperative effect was not satisfactory. In short, CMI is related to many diseases and its clinical manifestations are complex. Therefore, individualized management and treatment should be carried out in combination with the clinical manifestations and auxiliary examination results of patients.

Lesión difusa de médula espinal, ¿cirugía o tratamiento paliativo?: a propósito de un caso

Las lesiones difusas de médula son raras y un reto diagnóstico. Los gliomas de bajo grado son los de mayor prevalencia y los astrocitomas primarios, el subtipo más frecuente. Presentamos el caso de un varón de 36 años, con tiempo de enfermedad de 13 años de dolor cervical insidioso, progresa con hemiparesia derecha y, posteriormente, paraparesia severa con pérdida de control de esfínteres. Mediante una RMN total de columna se evidenciaron lesiones neoproliferativas difusas abarcando todos los segmentos medulares. Se realizó una biopsia a cielo abierto en segmento T1-T2, la patología diagnosticó glioma de bajo grado. El tratamiento es controversial y se descartó la cirugía por la alta morbilidad. Se optó por radioterapia y quimioterapia por su rol importante en el control de la enfermedad.

Diffuse spinal cord injuries are rare and pose a diagnostic challenge. Low-grade gliomas are the most prevalent type, with primary astrocytomas being the most frequent subtype. We present the case of a 36-year-old man with a 13-year history of squeezing neck pain, which progressed with right hemiparesis and later with severe paraparesis and loss of sphincter control. A whole spine MRI scan showed diffuse neoproliferative lesions that spread throughout all the spinal cord segments. An open biopsy of the lesion in the T1-T2 segment was performed. The pathology report stated low-grade glioma. Treatment is controversial, so surgery was ruled out due to high morbidity. Radiotherapy and chemotherapy were chosen because they play an important role in controlling the disease.

Siringomielia: revisión a propósito de un caso / Syringomyelia: review, case and report

La siringomielia supone un desafío diagnóstico, ya que es una entidad poco reconocida si no se tiene conciencia de su existencia. Al ser un cuadro progresivo, cuya clínica puede presentarse de forma larvada y ser coincidente con otras patologías neurológicas tales como la esclerosis múltiple, su detección suele realizarse en etapas tardías sobre todo en población adulta y más aún cuando se presenta de forma adquirida. Por lo que el estudio imagenológico con Resonancia Magnética adquiere especial relevancia, permitiendo identificar y clasificar la enfermedad, lo que brindará la base para decidir terapía.

Syringomyelia is a diagnostic challenge, since it is a poorly recognized disease, especially if its existence remains unknown. Being a progressive disease, whose clinic can present in a latent way and be coincident with other neurological pathologies such as multiple sclerosis, its detection is usually conducted in late stages, especially in the adult population and even more when it presents in an acquired way. Therefore, the imaging study with Magnetic Resonance acquires special relevance, allowing to be identified and classified, which will provide the basis for deciding on therapy

Imaging study of simple Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Objective:To investigate the relationship between simple Chiari malformation type I (CMI) and atlantoaxial instability from the imaging point of view.Methods:A retrospective analysis were performed on 46 patients diagnosed with simple CMI from January 2014 to December 2020. Forty-six normal people matched for age and sex were selected as the normal control group, while 30 patients with atlantoaxial dislocation were selected as the dislocation group. The degree of atlantoaxial joint degeneration in each group was assessed according to Weishaupt degeneration grading; the atlantoaxial joint angulation angle was measured in the control group of patients with simple CMI; and the sagittal imaging parameters of cervical spine X-ray were measured, including C 0-C 1 Cobb angle, C 0-C 2 Cobb angle, C 1-C 2 Cobb angle, C 1-C 7 Cobb angle, C 2-C 7 Cobb angle, C 7 Slope, C 2 Tilt, spino cranial angle (SCA), and C 2-C 7 sagittal vertebral axis (SVA). All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. Results:ICC ranged between 0.842 and 0.974 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. There were significant differences in the distribution of Weishaupt degeneration grading of atlantoaxial joints between simple CMI, normal and dislocation group ( H=53.68, P<0.001 on the left side; H=43.39, P<0.001 on the right side). There were significant differences in the degree of atlantoaxial joint degeneration between the normal group and dislocation group (left, Z=6.60, P<0.001; right, Z=6.29, P<0.001); There were significant differences in the degree of atlantoaxial joint degeneration between the normal group and simple CMI patients (left, Z=5.31, P<0.001; right, Z=4.13, P<0.001); There were significant differences in the degree of atlantoaxial joint degeneration between simple CMI and dislocation group (left, Z=3.20, P=0.001; right, Z=3.15, P=0.002). There were significant difference in the angulation angle of the atlantoaxial articular surface between the normal group and simple CMI patients (left, Z=3.32, P<0.001; right, Z=5.74, P<0.001). There were significant differences in C 0-C 1 Cobb angle ( t=2.41, P=0.018), C 1-C 7 Cobb angle ( t=2.88, P=0.005), C 2-C 7 Cobb angle ( t=3.29, P=0.001), and C 2-C 7 SVA ( t=2.87, P=0.005) between the normal group and simple CMI patients, but there was no significant difference in other parameters. Conclusion:The degree of atlantoaxial joint degeneration in patients with simple CMI is higher than that in normal people, the angulation angle is larger, and the cervical lordosis is larger, suggesting that there may be atlantoaxial joint instability. This study provides further evidence that Chiari malformation type I is associated with atlantoaxial instability.

The clinical effect of posterior fossa decompression combined with dural reconstruction in the treatment of Chiari malformation-Ⅰ complicated with syringomyelia / 中国医师进修杂志

Objective:To investigate the clinical effect of posterior fossa decompression combined with dural reconstruction in the treatment of Chiari malformation-Ⅰ(CM-Ⅰ) complicated with syringomyelia (SM).Methods:The clinical data of 50 patients with CM-Ⅰ complicated with SM who were treated in Yan′an University Xianyang Hospital from June 2019 to January 2021 were analyzed. They were divided into the study group (27 cases) and the control group (23 cases) according to the surgical methods. The former received posterior fossa decompression combined with dural reconstruction, while the latter received posterior fossa decompression alone. The clinical symptom improvement, neurological function, cerebrospinal fluid dynamics and syringomyelia changes were compared between the two groups before and after the surgery, and postoperative complications were compared.Results:The overall clinical symptom improvement rate between the two groups had no significant difference ( P> 0.05). After the surgery, the scores of pain, sensory disturbance, dyskinesia and ataxia in the study group were higher than those in the control group: (4.56 ± 0.35) points vs. (4.28 ± 0.43) points, (3.61 ± 0.82) points vs. (3.15 ± 0.73) points, (3.81 ± 0.44) points vs. (3.59 ± 0.50) points, (4.43 ± 0.41) points vs. (4.09 ± 0.53) points, there were statistical significant ( P<0.05). After the surgery, the cerebrospinal fluid stroke volume (SV) and mean flow (MF) in the study group were higher than those in the control group: (0.05 ± 0.02) ml vs. (0.04 ± 0.01) ml, (0.05 ± 0.01) ml/s vs. (0.04 ± 0.01) ml/s; the maximum peak flow velocity (V max) of the head and tail in the study group were lower than those in the control group: (3.14 ± 1.05) mm/s vs. (3.87 ± 1.13) mm/s, (5.56 ± 1.38) mm/s vs. (6.43 ± 1.22) mm/s, there were statistical significant ( P<0.05). There were no significant differences in the rate of reduction or disappearance of syringomyelia, the rate of no change and the rate of increase of syringomyelia after the surgery between the two groups ( P>0.05). There was no significant difference in the incidence of postoperative complications between the two groups ( P>0.05). Conclusions:Posterior fossa decompression combined with dural reconstruction in CM-Ⅰ complicated with SM can better improve cerebrospinal fluid dynamics, and promote the reduction of syringomyelia without increasing postoperative complications.

Siringomielia por malformación de Chiari: resolución espontánea. Revisión bibliográfica / Syringomyelia due to Chiari malformation: spontaneous resolution. Bibliographic review

Objetivo: describir y analizar los casos de siringomielia por malformación de Chiari tipo 1 con resolución espontánea. Método: se buscó en Pubmed bajo las palabras clave malformación de Chiari tipo 1, siringomielia y resolución espontánea todos los casos relacionados. Se registraron las características poblacionales de cada caso, evolución y mecanismos propuestos. Resultados: En la población adulta se encontraron 22 casos (edad media: 35,81) y en la población pediátrica se encontraron 37 casos (edad media: 9,82). Las hipótesis sobre los mecanismos que produjeron la resolución espontánea fueron: cambios en la posición de las amígdalas relacionadas con la edad, atrofia del cerebelo, fisura medular, reducción del esfuerzo físico, variaciones de la presión intraespinal, crecimiento del cráneo y disminución de la presión intracraneana. Conclusión: La resolución espontánea es posible en pocos casos. Si bien este hecho invita a realizar controles periódicos y evitar la cirugía, la espera conlleva riesgos ante la posibilidad de un agravamiento agudo o de que surjan secuelas permanentes. La información disponible hoy en día no permite predecir con seguridad la evolución de cada caso

Objective: describe and analyze the cases of syringomyelia caused by Chiari type 1 malformation with spontaneous resolution. Method: we searched in Pubmed with the key words Chiari malformation type 1, syringomyelia and spontaneous resolution all related cases. The population characteristics of each case were recorded along with its outcome and proposed mechanisms. Results: in the adult population there were 22 cases (medium age: 35.81) and in the pediatric population there were 37 cases (medium age: 9.82). The hypothesis about the mechanisms that produce the spontaneous resolution were: changes in tonsils position related to age, cerebellar atrophy, cord fissures, reduction of physical effort, variations in intraspinal pressure, skull growth and decrease in intracranial pressure. Conclusion: the spontaneous resolution of syringomyelia invites to carry out regular check-ups and avoid surgery. However, waiting carries risks with the possibility of acute worsening or permanent sequelae. The information available today does not allow to predict with certainty the evolution of each case.

Spinal cord atrophy-like changes in adolescent Chiari malformation type I patients: a neglected imaging change / 中华骨科杂志

Abstract:To compare the morphology of spinal cord between healthy adolescents with adolescent Chiari malformation type I (CMI) patients and investigate the impact of syringomyelia on the morphology of spinal cord in CMI patients.Methods:The clinical and radiological data of 292 CMI patients diagnosed by our center between June 2012 and March 2019 were retrospectively reviewed. Among them 15 CMI patients without syringomyelia were recruited in the CM group. Among the remaining 277 CMI patients, 274 patients had syringomyelia below the C 3-4 intervertebral disc. According to the principle of best matching, CMI patients with syringomyelia were selected with the closest age to the CM group (±18 months), and 30 CMI patients with syringomyelia were included in the CMS group according to a ratio of 1∶2. Thirty healthy adolescents were enrolled as the control group (NC group) in the same way. The anteroposterior diameters of spinal cord at C 2 (DSCO-C 2), spinal canal at C 2 (DSCA-C 2), midbrain-pontine junction (DPJ), the distance between the tip of cerebellar tonsils and the foramen magnum (AB) and the maximal diameter of the syrinx (D-syrinx) were measured on MRI. All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. One-way ANOVA and SNK- q test were used to compare the above radiographic parameters and age between CM, CMS and NC group. The distribution of genders was compared between the three groups using Chi-square tests. Pearson correlation analysis were conducted to demonstrate the relationship between radiographic parameters in CM and CMS group. Results:ICC ranged between 0.91 and 0.95 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. Patients in CM and CMS groups showed similar DSCO-C 2 values ( P=0.254), both of which were significantly lower than that in NC group ( P<0.001). DSCA-C 2 in CMS group was significantly larger than that in CM ( P=0.003) and NC ( P<0.001) groups, while no significant difference was found between the CM and NC groups ( P=0.216). Moreover, DPJ in CMS group was significantly lower than that in CM group ( P<0.001) and NC group ( P<0.001). There was no significant difference in AB between CM and CMS groups ( P=0.948). DSCO-C 2 was significantly positively correlated with DSCA-C 2 in CMS group ( r=0.906, P<0.001), while AB, D-syrinx, DSCO-syrinx, DSCA-syrinx and DPJ were not significantly correlated with DSCA-C 2. There were significant correlations observed between DPJ and other radiographic parameters in the CMS group (all P>0.05). Significant positive correlation between DSCO-C 2 and DPJ was observed in CM group ( r=0.703, P=0.005). There was no significant correlation between DSCO-C 2 and DSCA-C 2 and DPJ in NC group (all P>0.05). Conclusion:CMI adolescents have significant atrophic change of cervical spinal cord and midbrain-pontine junction compared with healthy adolescents, regardless of the existence of syrinx. Moreover, syrinx in CMI patients indicated more obvious atrophic change of midbrain-pontine junction and dilated spinal canal compared with isolated CMI patients.

Siringomielia asociada a malformación de Chiari tipo I. Informe de caso / Syringomyelia associated with Chiari type I malformation. Case report

RESUMEN La siringomielia es una cavidad quística de la medula espinal, y se considera un padecimiento progresivo y degenerativo. Existen múltiples y variadas maneras de clasificarla, basadas principalmente en su origen, o en los mecanismos conocidos de producción. Se presenta el caso de un paciente de 27 años, con cuadro de deficiencia motriz de cuatro meses de evolución, que comenzó en miembros superiores y continuó con torpeza a la marcha. Al examen físico presentó marcha paretoespástica, cuadriparesia con predominio de debilidad en miembros superiores con respecto a los inferiores, además de espasticidad crural y braquial izquierda, con hipotonía braquial derecha, asociado con atrofia distal de ambos miembros superiores e hipoestesia térmica y dolorosa suspendida del miembro superior derecho. La resonancia magnética nuclear mostró cavidad siringomiélica amplia en columna cervical, con descenso de las amígdalas cerebelosas, compatible con malformación de Chiari tipo I. Aunque la asociación de ambas entidades es común, el déficit motriz progresivo en un paciente joven es motivo de ingreso o consulta poco frecuente en el Servicio de Neurología. En estos casos, el tratamiento descompresivo puede disminuir el tamaño de la cavidad siringomiélica.

ABSTRACT Syringomyelia is a cystic cavity of the spinal cord, and is considered a progressive and degenerative condition. There are multiple and varied ways to classify it, based mainly on its origin, or on known production mechanisms. A 27-year-old patient with a four-month history of motor impairment, which began in the upper limbs and continued with clumsy gait, was presented. On physical examination, he presented paretospastic gait, quadriparesis with a predominance of weakness in the upper limbs with respect to the lower ones, as well as crural and left brachial spasticity, with right brachial hypotonia, associated with distal atrophy of both upper limbs and suspended thermal and painful hypoesthesia of the limb upper right. Nuclear magnetic resonance imaging showed a wide syringomyelic cavity in the cervical spine, with descent of the cerebellar tonsils, compatible with type I Chiari malformation. Although the association of both entities is common, progressive motor deficit in a young patient is a reason for admission or consultation rare in the Neurology Service. In these cases, decompressive treatment can decrease the size of the syringomyelic cavity.

Polyarticular charcot: A rare case report and a literature review in Indian context

Charcot or neuropathic arthropathies are a progressive form of destructive, erosive and generally painless arthropathies.Prevalence of neuropathic joints has decreased globally with reduction in the cases of leprosy and syphilis. However,syringomyelia and diabetes mellitus have emerged as the major causes for upper limb and lower limb Charcot jointsrespectively. Literature evidence shows lack of India data pertaining to these arthropathies. The present study describesa case of polyarticular Charcot in a patient with syrinx and Chiari malformation. The patient history revealed a provisionaldiagnosis of rheumatoid arthritis and Koch’s elbow, and was treated with anti-tubercular treatment (ATT) and diseasemodifying anti-rheumatic drugs (DMARDS). Nervous system examination would have easily led to the diagnosis ofsyringomyelia. The present study also provides a review of Indian literature on neuropathic joints from 2001 to 2019.Diabetes mellitus, syringomyelia, leprosy and syphilis are major etiologies for Charcot joints.

The use of magnetic resonance phase-contrast cine in Chiari malformation with syringomyelia

@#mesodermal origin and is commonly associated withsyringomyelia. Foramen magnum decompression is thefirst-line of standard treatment in symptomatic patients witha confirmed radiographic diagnosis. Magnetic resonance(MR) cine allows accurate evaluation of cerebrospinal fluid(CSF) physiology at the craniovertebral junction but oftenthis is under-utilised in Malaysia. Methods: In this series, we looked into nine cases of CM withsyringomyelia from clinical and radiological perspectivebefore and after surgery. The radiological parameters wereherniated tonsillar length, syrinx: cord ratio, syrinx lengthand diameter. Flow velocity and morphologic changes inChiari were illustrated. Results: Seven patients showed either reduction in syrinxlength, syrinx: cord ratio or both postoperatively. Clinicalrecovery somewhat varied in motor and sensory symptoms.Four patients gained better functional grade in modifiedRankin scale (MRS) while the rest remained similar. Thestudy highlighted the advantage of CSF flow dynamicsinformation over MR anatomical radiographic improvementin addressing the neurologic and functional recovery. Wealso discussed the practicality of cine sequence inpreoperative patient selection, syrinx analysis andpostoperative flow evaluation in anticipation of clinicaloutcome. Conclusion: Phase-contrast cine MRI is a useful tooldictated by resource availability. We recommend its routineuse in preoperative analysis and subsequent observationalfollow-up after surgery.

Factors predicting the occurrence of syringomyelia in patients with Chiari I malformations

Background: Chiari I malformations are defined as the downward herniation of cerebellar tonsils more than 5 mm through the foramen magnum. Syringomyelia is a common association of Chiari I malformation.Methods: This purpose of this study was to determine the various factors predicting the occurrence of syringomyelia in patients with Chiari I malformations. This retrospective study was conducted in Government medical college, Thrissur, Kerala, India. 27 patients admitted with diagnosis of symptomatic Chiari I malformations during the period of January 2015 to January 2019 were selected for the study.Results: The mean age was 22.8 years and syringomyelia was more common in patients older than 10 years (p value0.005). Females were 17(63%) and males were 10(37%) but there no difference in occurrence of syringomyelia among both sexes(p value is 0.16). Syringomyelia was present in 17(63%) cases. Association between various factors and syringomyelia measured by calculating P value which was 0.005 for age >10 years, 0.16 for sex, 0.093 for duration of symptoms >12 months, 0.05 for tonsillar descend >10mm and 0.097 for hypertension. The p value for the association of duration of symptoms >12months and extend of tonsillar descend is 0.001 Mean duration of symptoms (onset of first symptom to the time of presentation) was 16.3 months and syringomyelia was more common in patients with duration of symptoms more than 12 months but this difference was statistically not significant (p value 0.093). Mean tonsillar descend from the level of foramen magnum was 11.3 mms and syringomyelia was more common in patients with tonsillar herniation more than 10mm (p value 0.05). There is a statistically significant relationship between duration of symptoms more than 12 months and tonsillar descend more than 10mm(p value 0.001).There is no significant association between hypertension and occurrence of syringomyelia in patients with Chiari I malformation.Conclusions: Incidence of Chiari I Malformations is more among adults and it is slightly higher in females. Syringomyelia is a common association of Chiari I Malformations. The occurrence of syringomyelia in patients with Chiari I Malformations associated with increasing age of patients and extend of tonsillar herniation. There is no statistically significant association between syringomyelia and duration of symptoms or hypertension.

Arachnoiditis ossificans with syringomyelia: a unique case of myelopathy

Arachnoiditis ossificans (AO) is a rare disorder characterized by calcification of arachnoid membranes first described by Kaufman and Dunsmore in 1971. It is a very rare cause of spinal canal stenosis leading to neurological compromise presenting with progressive lower extremity myelopathy. It has been described to be a sequela of various conditions previous intradural surgery, myelograms, vascular malformations and adhesive arachnoiditis. Associated conditions may include syringomyelia. The imaging findings on MRI may be confusion. Preferred diagnostic method is non contrast computed tomography (CT). Surgical intervention is still controversial and can include decompression. The authors report the case of 48 years female presenting with gradually progressing paraparesis. Magnetic resonance imaging of the spine revealed a spinal cord syrinx but with an extramedullary intradural hypo-intensity. A computed tomography scan clearly demonstrated the abnormality and its extent. We present a unique case of syringomyelia resulting from spinal arachnoiditis ossificans and review the relevant literature. This case reports a unique presentation of arachnoiditis ossificans with syringomyelia in which etiology is not clear. We also highlight the difficulty is diagnosis on MRI and need of non-contrast CT.

Presyrinx Associated with Post-Traumatic Hydrocephalus Successfully Treated by Ventriculoperitoneal Shunt / 대한신경손상학회지

Presyrinx consists of reversible spinal cord swelling without frank cavitation, as observed on T2 weighted magnetic resonance imaging (MRI). The condition may evolve into syringomyelia, but timely surgical interventions have achieved meaningful results. Here, we report the case of a 27-year-old woman who presented with headache, dizziness, and diplopia 2 months after suffering a mild head trauma. On MRI, hydrocephalus, downward herniation of the cerebellar tonsil, and a diffuse high signal change in the cervical spinal cord were detected. After insertion of a ventriculoperitoneal shunt, her neurological symptoms resolved, and she has had no signs of presyrinx recurrence for >4 years.

Comparison of the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari I malformation associated with syringomyelia and atlantoaxial instability / 中国基层医药

Objective@#To compare the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari I malformation associated with syringomyelia and atlantoaxial instability.@*Methods@#From January 2016 to October 2017, 36 cases of Chiari I malformation associated with syringomyelia and atlantoaxial instability in Linfen People's Hospital were selected in the research.The patients were divided into two groups according to random number table method, with 18 cases in each group.The observation group was given decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment.The control group was given traditional decompression of posterior fossa + posterior cervical fusion and internal fixation treatment.The efficacy, complications occurred within 1 year after operation, JOA score and spinal cord cavity size at different time points before and after operation of the two groups were compared.@*Results@#The total effective rate of the observation group [94.44%(17/18)] was slightly higher than that of the control group [88.89%(16/18)] (χ2=1.957, P>0.05). The total effective rate of the observation group at 1 year after operation was 88.89%(16/18), which was significantly higher than that of the control group [72.22%(13/18)] (χ2=3.498, P<0.05). The incidence rate of complications of the observation group at 1 year after operation was 11.11%(2/18), which was significantly lower than that of the control group [38.89%(7/18)](χ2=11.685, P<0.05). The JOA scores at discharge[(15.97±1.25)point] and 1 year after operation[(15.53±1.19)point] of the observation group were significantly higher than those of the control group[(14.21±1.18)point, (14.06±1.15)point](t=3.815, 3.706, all P<0.05). The spinal cord cavity size at discharge [(2.13±0.64)mm] and 1 year after operation[(2.28±0.69)mm] of the observation group were significantly higher than those of the control group [(3.41±0.76)mm, (3.45±0.78)mm](t=5.743, 5.511, all P<0.05).@*Conclusion@#The long-term efficacy of decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment on Chiari I malformation associated with syringomyelia and atlantoaxial instability is remarkable, the recovery of spinal cord function and syringomyelia is well and the postoperative complications incidence is relatively low, which is worthy of popularization and application.

Investigation of the Neuropathic Pain Caused by Syringomyelia Associated with Chiari I Malformation

STUDY DESIGN: Retrospective cohort study. PURPOSE: To investigate the correlation between the syrinx morphology and neuropathic pain caused by syringomyelia associated with Chiari I malformation. OVERVIEW OF LITERATURE: Neuropathic pain caused by syringomyelia is refractory and markedly impairs the patient. METHODS: We examined 24 patients with neuropathic pain caused by syringomyelia associated with Chiari I malformation. We statistically analyzed the illness duration and age at surgery between patients with and without neuropathic pain. Additionally, we classified the morphology of the syringes into deviated (D), enlarged (E), central (C), and bulkhead (B) types using T2-weighted axial imaging. Moreover, we investigated the correlation between syrinx morphology and neuropathic pain. A Mann–Whitney U-test was performed to compare between the presence or absence of neuropathic pain and the presence or absence of type D syringes. RESULTS: The median age at surgery was 27.5 years, and the median illness duration was 24 months. Among the 24 patients, 11 had preoperative neuropathic pain, one of which was free of neuropathic pain during the final follow-up period. Among patients with neuropathic pain, the syringes’ preoperative morphology was type D in nine patients and types E and C in one patient each. No patient exhibited type B morphology. Among patients without neuropathic pain, the preoperative morphology of the syringes was type D in three patients, type E in seven patients, and types C and B in two patients each. For types D and E, a correlation between neuropathic pain and syrinx morphology was observed. Moreover, type D was associated with significant neuropathic pain in both preoperative and postoperative states. CONCLUSIONS: This study showed a correlation between the morphological features of the syringes and the occurrence of neuropathic pain in patients with syringomyelia associated with Chiari I malformation.

Comparison of the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability / 中国基层医药

Objective To compare the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability.Methods From January 2016 to October 2017,36 cases of Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability in Linfen People's Hospital were selected in the research.The patients were divided into two groups according to random number table method,with 18 cases in each group.The observation group was given decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment.The control group was given traditional decompression of posterior fossa + posterior cervical fusion and internal fixation treatment.The efficacy,complications occurred within 1 year after operation,JOA score and spinal cord cavity size at different time points before and after operation of the two groups were compared.Results The total effective rate of the observation group [94.44％ (17/18)] was slightly higher than that of the control group [88.89％ (16/18)] (x2 =1.957,P ＞ 0.05).The total effective rate of the observation group at 1 year after operation was 88.89％ (16/18),which was significantly higher than that of the control group [72.22％ (13/18)] (x2 =3.498,P ＜ 0.05).The incidence rate of complications of the observation group at 1 year after operation was 11.11％ (2/18),which was significantly lower than that of the control group [38.89％ (7/18)] (x2 =11.685,P ＜ 0.05).The JOA scores at discharge[(15.97 ± 1.25) point] and 1 year after operation[(15.53 ± 1.19) point] of the observation group were significantly higher than those of the control group [(14.21 ± 1.18) point,(14.06 ±1.15) point] (t =3.815,3.706,all P ＜ 0.05).The spinal cord cavity size at discharge [(2.13 ± 0.64) mm] and 1 year after operation [(2.28 ± 0.69) mm] of the observation group were significantly higher than those of the control group [(3.41 ±0.76)mm,(3.45 ±0.78)mm] (t =5.743,5.511,all P＜0.05).Conclusion The long-term efficacy of decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment on Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability is remarkable,the recovery of spinal cord function and syringomyelia is well and the postoperative complications incidence is relatively low,which is worthy of popularization and application.

Arnold Chiari tipo I y siringomielia en el adulto / Arnold Chiari type I and siringomyelia in the adult: report of a case

La malformación de Arnold Chiari es una anomalía congénita caracterizadapor el defecto de la base del cráneo y la herniación de parte del tronco y cerebelo a través del agujero magno, y puede estar asociado a siringomielia o hidrocefalia, la prevalencia de la enfermedad oscila entre el 4.3 x 1/100000 habitantes, afecta a ambos sexos, con ligero predominio en las mujeres, se trata de una paciente de 58 años de edad con antecedentes patológicos de relevancia, psoriasis del cuero cabelludo hace 8 años, gastritis y alergia a la aspirina y sus derivados, que fue hospitalizado porcefalea, debilidad muscular progresiva que inicia en miembro inferior izquierdo luego a miembros superiores bilateralmente, problemas del equilibrio, dolor en el cuello los brazos y la espalda, los estudios diagnósticos por imagen de resonancia magnética (RMN) de cerebro y columna cervico-dorsal ha demostrado una herniación de más 5mm por debajo del foramen magno. La siringomielia con malformación de Arnold Chiari tipo I es rara en adultos.

The Arnold Chiari malformation is a congenital anomaly characterized by the defect of the base of the skull and the herniation of part of the trunk and cerebellum through the foramen magnum, and may be associated with syringomyelia or hydrocephalus, the prevalence of the disease ranges from 4.3 x 1/100000 inhabitants, affects both sexes, with a slight predominance in women, this is a 58-year-old patient with relevant pathological history, psoriasis of the scalp 8 years ago, gastritis and allergy to aspirin and its derivatives, who was hospitalized for progressive muscle weakness headache that starts in the left lower limb after upper limbs bilaterally, balance problems, pain in the neck, arms and back, diagnostic studies by magnetic resonance imaging (MRI) of the brain and cervico-dorsal spine has shown a herniation of more than 5mm below the foramen magnum. Syringomyelia with Arnold Chiari type I malformation is rare in adults.

Pain treatment and recovery of functionality in a former athlete diagnosed with myofascial pain syndrome in the course of syringomyelia. Case report / Tratamento da dor e recuperação da funcionalidade em ex-atleta diagnosticada com síndrome dolorosa miofascial no curso de siringomielia. Relato de caso

ABSTRACT BACKGROUND AND OBJECTIVES: There are few studies that address non-surgical treatment in cases of syringomyelia, which reduces the possibilities of treatment for the patient. The objective of this study was to analyze the efficacy of the physiotherapeutic treatment for the symptoms of the pathology. CASE REPORT: Idiopathic syringomyelia is a condition in which a cystic-shaped cavity appears within the spinal cord. After the diagnosis of syringomyelia in C3-C6, 3 years ago, the patient, a former volleyball athlete, remained stable with no anesthetic dissociation, muscle atrophy or limb paresthesia. However, she began to have constant back and neck pain to the extent of limiting her functioning in jogging, volleyball, and difficulties of movement in daily routine. The physiotherapeutic treatment in 6 sessions, worked in the muscle and fascial release with dry needling and manual myofascial release associated with specific vertebral adjustments with chiropractic techniques, and after the cessation of the pain, specific muscle strengthening exercises. CONCLUSION: Physiotherapy showed to be an effective treatment for patient with syringomyelia that presented symptoms to myofascial pain syndrome.

RESUMO JUSTIFICATIVA E OBJETIVOS: Existem poucos estudos que abordam o tratamento não cirúrgico nos casos de siringomielia, fato que diminui as possibilidades de tratamento para o paciente. O objetivo deste estudo foi analisar a eficácia do tratamento fisioterapêutico para os sintomas da doença. RELATO DO CASO: A siringomielia idiopática é uma doença na qual uma cavidade em forma de cisto aparece dentro da medula espinhal. Após o diagnóstico de siringomielia em C3-C6, 3 anos atrás, a paciente, ex-atleta de voleibol, se manteve com o quadro estável e sem dissociação anestésica, atrofia muscular ou parestesia de membros, porém começou a ter dores lombares e cervicais constantes a ponto de ter limitação da função em corrida, voleibol e dificuldades de movimentação no dia a dia. Durante o tratamento fisioterapêutico em 6 sessões foi abordada a liberação muscular e fascial a partir do agulhamento à seco e liberação miofascial manual associado a ajustes vertebrais específicos com técnicas quiropráticas, e após cessamento das dores fortalecimento muscular específico. CONCLUSÃO: A Fisioterapia demonstrou ser um tratamento eficaz em uma paciente com siringomielia que apresentava sintomas relacionados à síndrome dolorosa miofascial.

Un enfoque clínico e histórico de la Malformación de Chiari Tipo I en adultos / A clinical and historic approach Type I Chiari malformation in adults

RESUMEN Se revisó la literatura existente sobre la Malformación de Chiari Tipo I en el adulto, haciendo énfasis en su diagnóstico imagenológico y tratamiento médico-quirúrgico. La malformación de Chiari es una enfermedad poco frecuente. Representa entre el 1 y el 4 % de todas las patologías neuroquirúrgicas. El diagnóstico se realiza meses y hasta años después de comenzada la sintomatología y se confirma en el 100 % de los casos con resonancia magnética de cráneo. El tratamiento es quirúrgico en los pacientes sintomáticos, siendo controversial en aquellos oligosintomáticos o con diagnóstico casual. La cirugía siempre debe realizarse con el apoyo de monitoreo neurofisiológico, que puede ser determinante en la técnica quirúrgica a emplear. La mortalidad asociada a la cirugía es muy baja, oscilando entre el 0 y 0,5 % según la mayoría de las series. Lo más importante es la selección de los pacientes para el tratamiento quirúrgico. No debe asumirse una actitud expectante en espera de un deterioro neurológico que justifique la cirugía, cuando ésta se hace a tiempo los resultados son mejores y con un mínimo de complicaciones (AU).

ABSTRACT The existent literature on Type I Chiari Malformation in adults was reviewed, making emphasis in its imaging diagnosis and medico-surgical treatment. Chiari malformation is a few frequent disease. It represents between 1 % and 4 % of all the neurosurgery pathologies. The diagnosis is made up months and even years after the beginning of the symptoms and it is confirmed by cranial magnetic resonance in the 100 % of the cases. The treatment is surgical in symptomatic patients, and it is controversial in the oligosymptomatic ones and in those with casual diagnosis. The surgery should be performed with the support of neurophysiological monitoring that might be determinant in the surgical technique to use. The mortality associated to the surgery is low, ranging from 0 and 0.5 % in most of the series. The most important thing is the choice of the patients for the surgical treatment. The neurological deterioration should not be expected to justify the surgery; when the surgery is carried out on time the results are better and with the minimum of complications (AU).