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[The differential diagnosis of syringomyelia from pseudosyringomyelitic ulcero-mutilating acropathy].

The authors describe some differential diagnostic criteria of atypical syringomyelia and pseudosyringomyelitic acropathy. It was found that trophic disorders in the skin, metatarsal bones were in atypical syringomyelia usually associated with sensibility disorders of the dissociated type in the zone of corresponding segments. In pseudosyringomyelitic acropathy trophic and skin changes precede sensibility disorders and do not always correspond to innervation zones of the lower extremities. Dysraphic state is of importance for the diagnosis of syringomyelia.

Adult↗

Abnormal spread of junctional acetylcholine receptor of paraspinal muscles in scoliosis associated with syringomyelia.

To test the denervation of paraspinal muscles and further investigate the pathogenesis of scoliosis associated with syringomyelia via detecting the spread of acetylcholine receptor (AChR) beyond the confines of the functional neuromuscular junction. Patients were divided into three groups: Group A consisted of 25 patients with scoliosis associated with syringomyelia, Group B included 16 adolescents with idiopathic scoliosis, and Group C comprised 10 cases without scoliosis. Bilateral biopsy of paraspinal muscles was performed during scheduled spinal surgery. Histological evaluation used a double-stain immunofluorescence technique for AchR and acetylcholinesterase. Histological analysis showed that 14 of 25 patients in Group A scored positive for the presence of AchR outside of the neuromuscular junction. There was no significant difference of the positive rate between patients with different degrees of cerebellar tonsillar descent, between patients with distended and non-distended syrinx, with syringx length 10 vertebral bodies, and with Cobb angle 45 degrees (p>0.05). The denervation of paraspinal muscles is present in some patients with scoliosis associated with syringomyelia, suggesting that scoliosis may be caused by a strength imbalance of paraspinal muscles in these patients.

Adolescent↗

Gamma subunits expression of junctional acetylcholine receptor of paraspinal muscles in scoliosis associated with syringomyelia.

UNLABELLED: To investigate the denervation of paravertebral muscles in scoliosis associated with syringomyelia via detecting the subunit expression of junctional acetylcholine receptors. METHODS: All the cases were divided into three groups: Group A consisted of 20 patients with scoliosis associated with syringomyelia, Group B included 10 adolescents with idiopathic scoliosis, and Group C was 10 cases without scoliosis. Bilateral biopsy of the paraspinal muscles was performed during scheduled spinal surgery. The mRNA expression of junctional acetylcholine receptor (AchR) subunits were detected by RT-PCR method. RESULTS: 13 Patient (65%) in Group A had a positive expression of the mRNA for the gamma subunit. The positive rate of gamma subunit expression was 40% on the convex side of the curve, and 25% on the convex side. The gamma subunit expression was absent from all samples in Group B. In Group C, only one patient had the gamma subunit expression on the left side. CONCLUSION: The gamma subunit expression of the junctional acetylcholine receptor is changed in scoliosis associated with syringomyelia, suggesting the presence of denervation in paravertebral muscles. The denervation of paravertebral muscles may play an role in the pathogenesis of scoliosis.

Adolescent↗

[A quantitative analysis of cerebrospinal fluid flow in posttraumatic syringomyelia].

Cerebrospinal fluid (CSF) flow within the spinal canal and syrinx in posttraumatic syringomyelia were studied by cardiac-gated phase images of magnetic resonance imaging in 12 normal volunteers and 8 patients with syringomyelia. The cardiac-gated phase method was simple and useful for detection of CSF flow; 1) phase modulation was in direct proportion to flow velocity, 2) phase modulation was not affected by the T1 or T2 relaxation time. In normal volunteers, CSF flows caudally during systole and cranially during diastole. The maximum caudal CSF flow velocity at C2 level was from 0.45 cm/sec to 1.71 cm/sec, average; 1.27 cm/sec. All of symptomatic posttraumatic syringomyelia patients had the flow in the syrinx.

Adolescent↗

Arnold-Chiari type 1 malformation and syringomyelia.

A 12-year-old boy had a long history of headache since the age of 4 years. Numbness and weakness of the upper extremities and chest tightness, especially in the morning, were noted since 2 years ago. The headache became more severe and frequent and attacked even in sleep since one year ago. Arnold-Chiari type 1 malformation, cervical syringomyelia and mild hydrocephalus were demonstrated by MRI scan of the central nervous system. Headache and other symptoms disappeared immediately after surgical intervention. The formation of syringomyelia may be multifactorial, but Arnold-Chiari type 1 malformation and uneventful birth history play an important role in the underlying cause of this case. We presented this youngest case who had Arnold-Chiari type 1 malformation and onset of headache from the age of 4 years, discussed the pathogenesis of syringomyelia and reviewed the literature.

Arnold-Chiari Malformation↗

[Diagnostic value of magnetic resonance imaging for syringomyelia].

Ten cases of syringomyelia confirmed by magnetic resonance imaging (MRI) were presented. The cavities in the spinal cord were of low signal on T1 weighted image (T1WI) and high signal on T2 weighted image (T2WI). The segments of the spinal cord with involved lesion resembled "bamboo joints" or were of "beaded" shape on sagittal section image and of "necklace" shape on cross section image. Size of the lesions in the spinal cord shown by MRI was larger than that judged by clinical symptoms. MRI can clearly show the position, size and shape of the cavities and other abnormal conditions. Results of this study show that MRI is the most useful diagnostic tool for syringomyelia. It should be emphasized that there was no dissociated impairment of pain and temperature and touch sensation in 40% of the patients and chronic spontaneous segmental pain was one of the important clinical characteristics of syringomyelia.

Adult↗

[Clinically manifest or silent primary and secondary syringomyelia. The magnetic resonance findings].

The MR scans were retrospectively reviewed of 40 patients who had been diagnosed as having syringomyelia. Our results demonstrate that syringomyelia can be found in asymptomatic patients as well as in many with atypical symptomatology. Our study stresses MR efficacy in investigating this pathologic condition. As a matter of fact, MR allowed us to visualize the cavity, its extension, the eventual association with Arnold Chiari type-I malformation and/or syringobulbia. In all cases cord enlargement and the presence of septations could also be demonstrated. In most cases the possible pathogenesis of syringomyelia could also be supposed.

Adolescent↗

[Diagnostic value of delayed enhancement CT scan for syringomyelia].

The article reported nine cases with syringomyelia confirmed by delayed enhancement CT scan. It was emphasized that the delayed enhancement CT scan was helpful not only to as certain position, shape, and size of lesions, but also to be fairly significant to the diagnosis of syringomyelia without typical manifestation, especially in differential diagnosis. It was suggested that delayed enhancement CT scan should be taken as a routine examination method for syringomyelia in hospitals having CT scan.

Adolescent↗

[An extramedullary spinal cord tumor associated with syringomyelia: a case report].

A 53-year-old woman presenting weakness in the left hand for ten years was diagnosed seven years ago as having juvenile muscular atrophy of the unilateral upper extremity. Afterwards, her left forearm and hand gradually became weaker. A neurologist pointed out syringomyelia in the film of MR imaging. However, the cause of syringomyelia was not demonstrated until gadolinium-DTPA-enhanced MR imaging disclosed a spinal tumor. Through an operation it was found that it was an intradural extramedullary tumor at C7 level. Histological diagnosis of the tumor was meningioma. Only 12 reports of syringomyelia associated with extramedullary intraspinal tumors were found. Among them each tumor in three cases was diagnosed by delayed metrizamide computed tomography, only one case by MR imaging. Though in our case myelography, postmyelographic CT, intravenous enhanced CT and MR imaging were performed, gadolinium-DTPA-enhanced MR imaging was eventually most useful in diagnosing this tumor. The pathophysiologic mechanism by which the syrinx fills, and the differentiation in MR imaging between syrinx and tumor cyst were reviewed.

Female↗

Syringomyelia associated with intradural extramedullary masses of the spinal canal.

Three cases of syringomyelia associated with intradural extramedullary tumors of the spinal canal are reported to demonstrate their radiographic feature, to postulate a mechanism for their formation, and to describe the clinical deterioration occurring in two patients immediately after metrizamide myelography. It is believed that the mechanism by which these syrinx cavities formed was similar to the mechanism of syrinx formation within chronically injured spinal cords. The cystic cavities may extend for considerable distances away from the extramedullary mass and may be present above and/or below the mass. The development of a syringomyelia in the face of an intradural extramedullary tumor may not be rare, and it is suspected that with the more frequent use of delayed metrizamide CT and now magnetic resonance imaging, syringomyelia will be found as an occasional consequence of this type of mass.

Adult↗

MR of postoperative syringomyelia.

Twenty-seven MR scans of 20 patients surgically treated for syringomyelia were reviewed. Thirteen patients had syringomyelia associated with the Chiari I malformation, four cases were posttraumatic, and three were idiopathic. The operations performed included syringosubarachnoid and syringoperitoneal shunts, myelotomies, and foramen magnum decompressions. Three of the foramen magnum decompressions had associated posterior fossa duroplasties, two had fourth-ventricle-to-subarachnoid shunts, and two had plugging at the obex. On 20 scans of patients in whom the syrinx cavity had been shunted, the shunt catheter was seen in 15 (75%). When adequately treated by shunting, syringes are completely collapsed and show no flow void. Nine patients were treated by foramen magnum decompression; all were well seen by MR. Three of these patients had a poor clinical result; these were the only patients in whom CSF was not seen between the foramen magnum and the neural structures of the posterior fossa on any images. The proposed mechanisms of syrinx formation and extension are discussed and related to the surgical procedures used to treat syringomyelia.

Adult↗

Syringomyelia in association with posterior fossa cysts.

This paper presents three patients with a triad of syringomyelia, midline posterior fossa cysts, and hydrocephalus. In the first patient, the clinical presentation was related to spinal cord cavitation, and the cranial anomalies were unexpected. In cases 2 and 3, the brain anomalies dominated the clinical picture, and syringomyelia was unexpected. These cases show that an examination of the whole neuraxis is as important in patients with midline posterior fossa cysts as it is in patients with developmental syringomyelia or Chiari I malformation.

Adolescent↗

[Thermographic findings of syringomyelia].

Thermographic findings in 10 cases of syringomyelia were reported. 7 cases were associated with Arnold-Chiari malformation and 3 cases with spinal adhesive arachnoiditis. The cases included 3 men and 7 women, aged 7 to 56 years (mean age 27.7 years). Comparing the right side of the body with the left side, in 9 cases, thermography demonstrated asymmetric skin temperature of the trunk or extremities. MRI revealed laterality of the syrinx at the level of cervical or lumbo-sacral cord in 9 cases. The side with the lower temperature corresponded with the side of lateralized syrinx in 8 cases. Neurologically, 9 cases had laterality of sensory disturbance and 6 cases had asymmetric motor weakness. The side with the lower temperature also corresponded with the laterality of sensory disturbance in 8 cases, and the side of the motor weakness in 4 cases. Considering these findings, many of the patients with syringomyelia are thought to have asymmetric skin temperature. Intramedullary involvement of sympathetic pathway by the syrinx may cause such a change. As thermography is non-invasive and easy to carry out, it should be a useful method for estimating or even detecting cases of syringomyelia, especially when applied to out-patients.

Adolescent↗

[X-ray diagnosis of syringomyelia].

The results of contrast X-ray examination of 34 patients with syringomyelia are discussed. Air encephalomyelography was performed in all patients and ventriculography and positive myelocisternography of the craniovertebral junction were conducted when indicated. The Arnold-Chiari syndrome was detected in most cases. Cystography of myodilomas was conducted in operated on patients with the communicating form of syringomyelia. The results allow a proper approach to the tactics of X-ray examination of patients with syringomyelia, which is necessary for the choice of an adequate method of surgical management.

Adolescent↗

Cervical spondylosis and syringomyelia: suboptimal results, incomplete treatment, and the role of intraoperative ultrasound.

The pathophysiology of cervical spondylotic myelopathy and syringomyelia is incompletely understood. Only 50-60% of the former group of patients and only 30-40% of the latter group of patients show long-term improvement. One possible cause for this might be continued anterior compression of the spinal cord in the former and incomplete drainage of the fluid-filled cavities in the latter. Intraoperative ultrasound imaging can be done in the operating room (4-6, 16-18) and can identify whether an adequate decompression has been done in patients with cervical spondylotic myelopathy and whether there has been complete drainage in shunting of patients with syringomyelia. Intraoperative ultrasound imaging aids the neurosurgeon in checking to see if he did what he set out to do. It is useful in operative procedures for cervical spondylotic myelopathy and syringomyelia.

Cervical Vertebrae↗

[Diagnosis of syringomyelia by using the method of nuclear magnetic resonance (NMR)].

On the basis of own observation from many years and a literature survey the authors discuss the diagnostic difficulties in syringomyelia in the light of the images obtained by conventional radiological techniques. The introduction of CT extended greatly the effectiveness of syringomyelia diagnosis. However, the best results in the diagnosis of the pathological lesions in syringomyelia are obtained using the technique of nuclear magnetic resonance.

Adult↗

Syringomyelia in scoliotic patients.

Two patients with a congenital or juvenile scoliosis and syringomyelia are described. The first subjective neurological symptoms appeared when the patients were teenagers, but syringomyelia was not diagnosed until the ages of 22 and 34, after the rapid progression of the symptoms. For successful surgical treatment of scoliosis or syringomyelia, a thorough radiological examination is emphasized in scoliotic patients with minimal neurological symptoms.

Adult↗

[Pathogenesis and the treatment of secondary syringomyelia].

Six cases with secondary syringomyelia were evaluated clinically and the pathogenesis was discussed. Three cases had the tumors; an ependymoma arising from the conus medullaris and the filum terminale, a foramen magnum meningioma extending to C2 and a thoracic astrocytoma. Two cases had past history of spinal cord injury with L1 and L2 fracture-dislocation, respectively. One case showed hydromyelic symptoms associated with isolated fourth ventricle after post-meningitic hydrocephalus. Clinical symptoms and signs were complex and various in each case due to the association of the original disease and the syrinx. Syringomyelic symptoms were dominant in three cases of which the syrinx extended from the conus to the cervical cord. Initial symptoms of two cases with post-traumatic syringomyelia were tingling pains which began near the site of injury and extended rostrally. Metrizamide myelography revealed complete or incomplete block at the location of the tumors or the injuries. Delayed CT demonstrated the syrinx in all cases. The syrinx was always present near the sites of primary lesions. The communication between the syrinx and the fourth ventricle was suspected in three cases, and the communication of the syrinx and the spinal subarachnoid space was suspected in two cases. All cases underwent the surgical treatments. Total removal of the tumors were completed in two cases and relieved the majority of symptoms. On the other hand, a case with a thoracic astrocytoma underwent biopsy of the tumor and irradiation, followed by poor outcome. Syringo-peritoneal shunts were performed in two cases with post-traumatic syringomyelia and relieved pain, but neurological signs were unchanged.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗