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The Western Journal of Medicine Jun 1988Posttraumatic syringomyelia is becoming increasingly recognized as a sequel to major and minor spinal cord injury, paralleling the development and widespread...
Posttraumatic syringomyelia is becoming increasingly recognized as a sequel to major and minor spinal cord injury, paralleling the development and widespread availability of magnetic resonance imaging as a diagnostic modality for evaluating possible spinal pathologic lesions. Delayed, subacute, or progressive neurologic deterioration in victims of traumatic spinal injury with "fixed deficits" should raise the suspicion of posttraumatic syringomyelia. Alternatively, it may present as sensory or motor complaints occurring on a delayed basis after minor spinal trauma causing no initial neurologic impairment. At our institution, we have treated six of eight patients with this condition by shunting fluid from the intramedullary cyst to the peritoneal cavity by means of a simple valveless shunt, resulting in sustained neurologic improvement in five patients.
Topics: Adolescent; Adult; Aged; Female; Humans; Male; Middle Aged; Spinal Cord Injuries; Syringomyelia
PubMed: 3176472
DOI: No ID Found -
Revista de Neurologia Jan 2017
Topics: Child; Humans; Pediatrics; Syringomyelia
PubMed: 28075005
DOI: No ID Found -
The Journal of International Medical... Apr 2019Dandy-Walker syndrome associated with syringomyelia is a rare condition, with few reports of adult cases. We describe an adult case of Dandy-Walker syndrome with... (Review)
Review
Dandy-Walker syndrome associated with syringomyelia is a rare condition, with few reports of adult cases. We describe an adult case of Dandy-Walker syndrome with concomitant syringomyelia. A 33-year-old man presented with a 3-month history of walking instability, numbness in the hands, memory deterioration, and urinary incontinence. A physical examination showed a positive Romberg sign. Brain computed tomography and magnetic resonance imaging showed hydrocephalus, a cyst in the posterior fossa, absence of the cerebellar vermis, hypoplasia of the corpus callosum and cerebella, and syringomyelia. All of these symptoms were consistent with the diagnosis of Dandy-Walker syndrome. Surgery involving arachnoid adhesiolysis and endoscopic third ventriculostomy was performed. At the 6-month follow-up, the symptoms were completely relieved. Magnetic resonance imaging showed that syringomyelia was greatly reduced and the hydrocephalus remained unchanged. Dandy-Walker syndrome with concomitant syringomyelia in adults is exceedingly rare. Early diagnosis and appropriate surgical treatment of this condition should be highlighted. Combined arachnoid adhesiolysis and endoscopic third ventriculostomy may be an effective approach.
Topics: Adult; Dandy-Walker Syndrome; Humans; Male; Prognosis; Syringomyelia
PubMed: 30799663
DOI: 10.1177/0300060518808961 -
Fluids and Barriers of the CNS Jul 2020Syringomyelia is a common spinal cord lesion. However, whether CSF blockage is linked to the formation and enlargement of syringomyelia is still controversial. The...
BACKGROUND
Syringomyelia is a common spinal cord lesion. However, whether CSF blockage is linked to the formation and enlargement of syringomyelia is still controversial. The current model of syringomyelia needs modification to more closely mimic the clinical situation.
METHODS
We placed cotton strips under the T13 lamina of 40 8-week-old rats and blocked CSF flow by extradural compression. After 4 and 8 weeks, MRI was performed to evaluate the morphology of syringomyelia and the ratio of spinal cord diameter to syrinx diameter calculated. Locomotor function was evaluated weekly. Spinal cord sections, staining and immunohistochemistry were performed 8 weeks after surgery, the ratio of the central canal to the spinal cord area was calculated, and ependymal cells were counted. In another experiment, we performed decompression surgery for 8 rats with induced syringomyelia at the 8th week after surgery. During the surgery, the cotton strip was completely removed without damaging the dura mater. Then, the rats received MRI imaging during the following weeks and were sacrificed for pathological examination at the end of the experiment.
RESULTS
Syringomyelia formed in 82.5% (33/40) of rats at the 8-week follow-up. The Basso, Beattie and Bresnahan (BBB) scores of rats in the experimental group decreased from 21.0±0.0 to 18.0 ±3.9 in the first week after operation but returned to normal in later weeks. The BBB score indicated that the locomotor deficit caused by compression is temporary and can spontaneously recover. MRI showed that the syrinx is located in the center of the spinal cord, which is very similar to the most common syringomyelia in humans. The ratio of the central canal to the spinal cord area reached (2.9 ± 2.0) × 10, while that of the sham group was (5.4 ± 1.5) × 10. The number of ependymal cells lining the central canal was significantly increased (101.9 ± 39.6 vs 54.5 ± 3.4). There was no syrinx or proliferative inflammatory cells in the spinal cord parenchyma. After decompression, the syringomyelia size decreased in 50% (4/8) of the rats and increased in another 50% (4/8).
CONCLUSION
Extradural blockade of CSF flow can induce syringomyelia in rats. Temporary locomotor deficit occurred in some rats. This reproducible rat model of syringomyelia, which mimics syringomyelia in humans, can provide a good model for the study of disease mechanisms and therapies.
Topics: Animals; Disease Models, Animal; Female; Locomotion; Rats, Sprague-Dawley; Spinal Cord; Spinal Cord Compression; Syringomyelia
PubMed: 32736591
DOI: 10.1186/s12987-020-00213-4 -
Ugeskrift For Laeger May 2020Cerebrospinal fluid flow dynamics are not fully understood. However, it is well known, that flow dynamics play a crucial role in development and treatment of a wide... (Review)
Review
Cerebrospinal fluid flow dynamics are not fully understood. However, it is well known, that flow dynamics play a crucial role in development and treatment of a wide variety of diseases in the central nervous system, among them, syringomyelia. Hence, in-depth knowledge of the mechanisms underlying flow dynamics is of great interest to improve diagnostics, prognostics, and to assess treatment efficacy. In this review we argue, that phase-contrast MRI may have these attributes, as it introduces detailed non-invasive qualitative and quantitative information on cerebrospinal fluid flow dynamics.
Topics: Central Nervous System; Humans; Magnetic Resonance Imaging; Syringomyelia
PubMed: 32515330
DOI: No ID Found -
The American Journal of Case Reports Oct 2022BACKGROUND Patients with Arnold-Chiari Malformation I (CM-I) treated with foramen magnum decompression (FMD) can have ongoing neck pain, headaches, and other symptoms...
Neck pain and Headache Complicated by Persistent Syringomyelia After Foramen Magnum Decompression for Chiari I Malformation: Improvement with Multimodal Chiropractic Therapies.
BACKGROUND Patients with Arnold-Chiari Malformation I (CM-I) treated with foramen magnum decompression (FMD) can have ongoing neck pain, headaches, and other symptoms complicated by persistent syringomyelia, yet there is little research regarding treatment of these symptoms. CASE REPORT A 62-year-old woman with a history of residual syringomyelia following FMD and ventriculoperitoneal shunt for CM-I presented to a chiropractor with progressively worsening neck pain, occipital headache, upper extremity numbness and weakness, and gait abnormality, with a World Health Organization Quality of Life score (WHO-QOL) of 52%. Symptoms were improved by FMD 16 years prior, then progressively worsened, and had resisted other forms of treatment, including exercises, acupuncture, and medications. Examination by the chiropractor revealed upper extremity neurologic deficits, including grip strength. The chiropractor ordered whole spine magnetic resonance imaging, which demonstrated a persistent cervico-thoracic syrinx and findings of cervical spondylosis, and treated the patient using a multimodal approach, with gentle cervical spine mobilization, soft tissue manipulation, and core and finger muscle rehabilitative exercises. The patient responded positively, and at the 6-month follow-up her WHO-QOL score was 80%, her grip strength and forward head position had improved, and she was now able to eat using chopsticks. CONCLUSIONS This case highlights a patient with neck pain, headaches, and persistent syringomyelia after FMD for CM-I who improved following multimodal chiropractic and rehabilitative therapies. Given the limited, low-level evidence for these interventions in patients with persistent symptoms and syringomyelia after FMD, these therapies cannot be broadly recommended, yet could be considered on a case-by-case basis.
Topics: Female; Humans; Middle Aged; Syringomyelia; Arnold-Chiari Malformation; Foramen Magnum; Quality of Life; Neck Pain; Chiropractic; Decompression, Surgical; Magnetic Resonance Imaging; Headache; Treatment Outcome
PubMed: 36315459
DOI: 10.12659/AJCR.937826 -
Journal of the Neurological Sciences Feb 2012Syringomyelia is a rare disease characterized by abnormal fluid-filled cavities within the spinal cord, and is associated with Chiari malformations, arachnoiditis, or... (Comparative Study)
Comparative Study Randomized Controlled Trial
BACKGROUND
Syringomyelia is a rare disease characterized by abnormal fluid-filled cavities within the spinal cord, and is associated with Chiari malformations, arachnoiditis, or spinal cord tumors. The widespread availability of magnetic resonance imaging (MRI) in Japan has allowed for easy identification of syrinxes. The aim of this study was to survey the clinicoepidemiological characteristics of syringomyelia in Japan.
METHODS
A 2-stage postal survey was conducted in late 2009. The first survey aimed to estimate the number of patients with syringomyelia, and the second survey aimed to elucidate clinicoepidemiological characteristics. Diagnosis of syringomyelia was based on the findings of MRI or computed tomographic myelography.
RESULTS
In the first survey, we received 2133 responses from 2937 randomly selected departments and collected data of 1215 syringomyelia patients (543 men and 672 women). The total response rate for the first survey was 73%. The estimated prevalence of ambulatory syringomyelia patients in Japan was 1.94 per 100000. In the second survey, the proportion of asymptomatic syringomyelia patients was 22.7%. Chiari type I malformations and idiopathic syringomyelia were the first and second most common etiologies.
CONCLUSIONS
Our nationwide survey indicated that widespread MRI availability has contributed to the diagnosis of both asymptomatic and idiopathic cases.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Female; Health Surveys; Humans; Infant; Japan; Magnetic Resonance Imaging; Male; Middle Aged; Syringomyelia; Young Adult
PubMed: 21943925
DOI: 10.1016/j.jns.2011.08.045 -
Revista de Neurologia Nov 2016Patients with multisutural or single craniosynostosis, often suffer from Chiari malformation and syringomyelia. The surgical management of syringomyelia in these... (Review)
Review
INTRODUCTION
Patients with multisutural or single craniosynostosis, often suffer from Chiari malformation and syringomyelia. The surgical management of syringomyelia in these patients is controversial.
CASE REPORT
A 3-year-old girl was referred with complex craniosynostosis that had not been corrected surgically. She was asymptomatic despite the cranial MRI showed a Chiari malformation and one year later she developed a cervico-dorso-lumbar syringomyelia. She underwent a decompressive suboccipital craniectomy but subsequently suffered a worsening of syringomyelia. The intracranial pressure monitoring was pathological so it was decided to perform a decompressive bilateral fronto-parieto-temporal craniotomy and remodeling of the cranial vault, achieving a significant reduction of syringomyelia.
CONCLUSIONS
After reviewing the literature, it is noted that there is currently no consensus on the treatment of syringomyelia in patients with craniosynostosis and Chiari malformation. Some authors recommend the simultaneous surgical suboccipital and cranial vault decompression, others only decompression of the cranial vault and other enlargement of the posterior fossa with distractors. In cases where the suboccipital decompression was performed first, the syringomyelia was not improved or stabilized. We conclude that the most effective treatment for patients with syringomyelia and craniosynostosis is decompressive remodeling of the cranial vault, as the main cause of syringomyelia is the raised intracranial pressure and lack of skull compliance.
Topics: Arnold-Chiari Malformation; Child, Preschool; Craniosynostoses; Decompression, Surgical; Female; Humans; Magnetic Resonance Imaging; Neurosurgical Procedures; Skull; Syringomyelia
PubMed: 27819403
DOI: No ID Found -
Scientific Reports Mar 2021The microstructure of the spinal cord in syringomyelia has not been well studied. The aim of this study was to evaluate the microstructure of the cervical cord in...
The microstructure of the spinal cord in syringomyelia has not been well studied. The aim of this study was to evaluate the microstructure of the cervical cord in patients with syringomyelia using diffusion tensor imaging (DTI) and to investigate the association between DTI parameters and the size of the syrinx cavity. Thirty patients with syringomyelia and 11 age-matched controls were included in this study. DTI and T1/T2-weighted MRI were used to estimate spinal microstructure. The patients were divided into a clinical symptom group (group A) and a non-clinical symptom group (group B) according to ASIA assessments. The fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values (mm/s) were measured and compared between patients and controls. Correlation between FA/ADC and the size of the syrinx cavity was examined with a bivariate analysis. FA values were lower (P < 0.000) and ADC values were higher (P < 0.000) compared to the controls at the level of all syrinxes examined in patients with syringomyelia; both FA values and ADC values reached normal values either above or below the syrinx levels (all P > 0.05). FA values and ADC values at all cervical levels were not significantly different either in controls or outside of the syrinx (all P > 0.05). FA values of group A was significantly lower than those of group B (P < 0.000). There was a negative association between FA values and the size of syrinx cavity, and a positive association between ADC values and the size of syrinx cavity (FA: P < 0.05, ADC: P < 0.05). The microstructure of the cervical spinal cord is different across all patients with syringomyelia. DTI is a promising tool for estimating quantitative pathological characteristics that are not visible with general MRI.
Topics: Adolescent; Adult; Anisotropy; Cervical Cord; Cervical Vertebrae; Child; Diffusion Magnetic Resonance Imaging; Diffusion Tensor Imaging; Female; Humans; Male; Neck; Spinal Cord; Syringomyelia; Young Adult
PubMed: 33664296
DOI: 10.1038/s41598-021-84164-2 -
Journal of Neurotrauma Aug 2013Aquaporin-4 (AQP4) is an astroglial water channel protein that plays an important role in the transmembrane movement of water within the central nervous system. AQP4 has...
Aquaporin-4 (AQP4) is an astroglial water channel protein that plays an important role in the transmembrane movement of water within the central nervous system. AQP4 has been implicated in numerous pathological conditions involving abnormal fluid accumulation, including spinal cord edema following traumatic injury. AQP4 has not been studied in post-traumatic syringomyelia, a condition that cannot be completely explained by current theories of cerebrospinal fluid dynamics. Alterations of AQP4 expression or function may contribute to the fluid imbalance leading to syrinx formation or enlargement. The aim of this study was to examine AQP4 expression levels and distribution in an animal model of post-traumatic syringomyelia. Immunofluorescence and western blotting were used to assess AQP4 and glial fibrillary acidic protein (GFAP) expression in an excitotoxic amino acid/arachnoiditis model of post-traumatic syringomyelia in Sprague-Dawley rats. At all time-points, GFAP-positive astrocytes were observed in tissue surrounding syrinx cavities, although western blot analysis demonstrated an overall decrease in GFAP expression, except at the latest stage investigated. AQP4 expression was significantly higher at the level of syrinx at three and six weeks following the initial syrinx induction surgery. Significant increases in AQP4 expression also were observed in the upper cervical cord, rostral to the syrinx except in the acute stage of the condition at the three-day time-point. Immunostaining showed that AQP4 was expressed around all syrinx cavities, most notably adjacent to a mature syrinx (six- and 12-week time-point). This suggests a relationship between AQP4 and fluid accumulation in post-traumatic syringomyelia. However, whether this is a causal relationship or occurs in response to an increase in fluid needs to be established.
Topics: Animals; Aquaporin 4; Gene Expression Regulation; Male; Rats; Rats, Sprague-Dawley; Spinal Cord Injuries; Syringomyelia
PubMed: 23441695
DOI: 10.1089/neu.2012.2614