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[Syringomyelia, its pathogenesis and surgical treatment based on 4 cases' experience].

Four cases of syringomyelia, each were considered to have different pathogenesis of syrinx and presented different clinical and radiological pictures, are reported. Case I was associated with Chiari I malformation and the syrinx communicated with the fourth ventricle through the central canal, case 2 was associated with Chiari II malformation and the syrinx was not communicated with the fourth ventricle, case 3 was thought to be traumatic and case 4 to be arachnoiditis due to unknown etiology. Metrizamide CT myelography was most valuable diagnostic technique to disclose the syringomyelic cavity and its extension. The cases except case 1 showed central opacification without via fourth ventricle, suggesting transneural migration CSF as shown by Aubin et al. Surgical treatment, therefore, was different in each case to obtain normal CSF dynamics. Case 1 was treated by suboccipital craniectomy, muscle plugging to the obex and syringo-subarachnoid shunt. In case 2 syringo-cisternal shunt was done in addition to suboccipital craniectomy. In case 3 syringocisternal shunt was done after laminectomy. In case 4 syringo-peritoneal shunt was performed. All but case 4 were obtained favorable result and case 4 was unfavorable except the disappearance of girdle sensation. For traumatic or inflammatory syringomyelia with tight adhesion between pia and arachnoid membrane in subarachnoid space, syringocisternal shunt was good way to obtain normal CSF dynamics and was expected to relieve the neurological deterioration.

Adolescent↗

Acquired scoliosis associated with hydromyelia and syringomyelia in two dogs.

Progressive scoliosis resulting from hydromyelia and syringomyelia was found in 2 dogs. In one dog, hydromyelia was associated with pachymeningeal fibrosis, with adhesions in the cervical portion of the spinal cord. In the other case, a cause was not established. Neither dog had congenital CNS malformations. The clinicopathologic findings in the 2 dogs are described, and the etiology and pathogenesis of hydromyelia and syringomyelia are discussed.

Animals↗

Cleidocranial dysostosis and syringomyelia. Review of the literature and case report.

A 34-year-old woman with congenital cleidocranial dysostosis had scoliosis diagnosed at age 13. The scoliosis was treated during adolescence with spinal orthoses but continued to progress after skeletal maturation. She was diagnosed as having syringomyelia at age 34. The possibility of syringomyelia should be considered in patients with cleidocranial dysostosis, especially those with neurologic symptoms, scoliosis not responding to conservative therapy, or progressive scoliosis after skeletal maturation. Although only two previous patients have been reported in the literature, the condition undiagnosed could be a more common problem than generally supposed.

Adult↗

[Short latency somatosensory evoked potentials in patients with syringomyelia].

Short latency somatosensory evoked potentials (SSEPs) following median nerve and posterior tibial nerve stimulation were studied in six patients with syringomyelia. Three patients had Chiari malformations, two patients experienced fracture of the spine and one patient had a cauda equina ependymoma. SSEPs following median nerve stimulation were abnormal in all patients, of which five patients showed abnormal SSEPs only in the unilateral stimulation on the side of sensory deficits. SSEPs obtained from three out of eight upper extremities which showed no disturbance of deep sensation, were abnormal, so SSEPs were able to detect subclinical abnormality indicating dorsal column dysfunction. Abnormal patterns of SSEPs were classified in three types as follows; Type 1: disappearance of P13, N16 and N18 (3 cases), Type 2: the prolonged interpeak latency P11-P13 (2 cases), and Type 3: abnormal N16 and N18 with preserving P13 (1 case with Chiari malformation). P9 and P11 were present without prolonged latencies in all cases. SSEPs following posterior tibial nerve stimulation were abnormal in two of the three tested patients. Those two patients had disturbance of deep sensations in the lower extremities. All patients underwent surgical treatment, syringo-peritoneal shunt in four patients, foramen magnum decompression with syringo-subarachnoid shunt in one patient, and total removal of an ependymoma of the cauda equina with syringotomy in one patient. Postoperative neurological improvement were found in three patients, of which two cases also showed improvement in SSEPs. On the contrary SSEPs were unchanged in two patients with posttraumatic syringomyelia, whose postoperative neurological condition was also unchanged.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Dynamics of changes in vibration sensitivity in syringomyelia].

The state of vibrational sensibility was studied in 35 patients with syringomyelia, with the aid of a special device, which evoked in the tuning fork a vibration of the same amplitude (128) in repeated studies. The indices of vibrational sensibility in patients with syringomyelia were compared to normal figures. As a norm the author employed the medium indices of vibrational sensibility in seconds in 50 normals. According to the vibrational sensibility all the patients were divided into 4 groups. The absence of vibrational perception, its drop below the norm and appearance of hyperpallesthesia corresponded to the most severe forms of the disease. In a drop of temporal indices, but not lower than the norm, the side, where the pathological process was more marked under the treatment influence, showed a restituted vibrational feeling. Disordered vibrational sensibility was found not only to the level of affected segments, but beyond them, which supports the conductive character of these disturbances.

Adult↗

Delayed CT metrizamide enhancement of syringomyelia secondary to tumor.

Six cases of syringomyelia associated with intramedullary tumor were examined by metrizamide myelography and delayed computed tomography. All cases showed opacification of syringomyelic cavities 7-24 hr after myelography. This finding suggests that metrizamide can pass through the cord substance from the subarachnoid space into a syrinx cavity in noncommunicating types of syringomyelia. It supports the theory of transneural passage of fluid as part of the origin of such cavities. These cases also support the thesis that, in patients with cord cavities, tumor can be excluded only if the cavity is demonstrated within all areas of cord enlargement.

Adult↗

Nuclear magnetic resonance imaging of syringomyelia.

Five patients with syringomyelia were examined with a 3.5 kG nuclear magnetic resonance (NMR) imager. Syrinx cavities were visualized in all five cases, and image quality compared favorably with metrizamide computed tomography (CT). Axial images were optimal for identifying syrinx cavities, and sagittal views were useful in providing an overview of cord morphology and in examining the craniocervical junction. Inversion-recovery images were less valuable than the spin-echo sequences. By varying spin-echo imaging parameters, tissue relaxation times could be determined and the fluid nature of the syrinx cavities confirmed. This limited study suggests that NMR may challenge the current role of CT in the diagnostic workup of syringomyelia.

Diagnosis, Differential↗

Preoperative demonstration of postinflammatory syringomyelia.

Syringomyelia secondary to arachnoiditis is a rarely reported entity, which has not been described in the radiographic literature. A series of eight patients who had either postinfectious, posthemorrhagic, iatrogenic, or idiopathic arachnoiditis was examined recently with metrizamide computed tomography (CT). These patients presented with progressive neurologic deficits that could not be attributed to the level of arachnoid changes on myelography. On immediate and/or delayed CT scans, metrizamide accumulation within the central part of the cord was identified, both at and above the level of the arachnoiditis. In some patients, the cord diameter was normal. Syrinx cavities were demonstrated and shunted at surgery with subsequent partial relief or arrest of the symptoms. The mechanism of development of postinflammatory syringomyelia is discussed.

Adult↗

[Dorsal interapophyseal arthropathy and spinal cord compression in syringomyelia].

A dorsal spinal cord compression by exuberant arthropathy and osteophytes of the articular processes occurred in a 61 years old patient with syringomyelia. The clinical symptoms were related to postero lateral cord involvement and rapidly appeared after syringo-peritoneal shunting. C.T. scan precisely displayed the pathological process and the cord compression. Surgery enabled a definite amelioration of secondary sensory deficits. It was to our knowledge the first description of such an involvement of articular processes in syringomyelia.

Adult↗

[Cystography of the spinal cord for delineating tactics for surgical treatment of syringomyelia].

Radiocontrast study of cystic cavities of the spinal cord was conducted in 23 patients with the clinical picture of syringomyelia and syringobulbia. Diffuse cystic cavities were demonstrated in the cervical and thoracic segments of the spinal cord in 22 patients. In 9 patients, the spinal cysts communicated with the cavity of the fourth ventricle. In 2 cases, the cysts extended to the region of the spinal cord terminal filament. The information obtained allowed a differential approach to the choice of the method and tactics of surgical treatment of syringohydromyelia. Macrosurgical operations on the craniovertebral level with tamponade of the communication between the spinal cord cyst and the cavity of the fourth ventricle were carried out in patients with the communicating form of syringomyelia. Operations for dividing the terminal filament of the spinal cord or myelotomy with drainage of the cyst at the cervical or thoracic level were performed in other cases.

Adult↗

[Role of the muscle factor in the pathogenesis of painful forms of syringomyelia].

On the basis of examining the disease histories of patients with syringomyelia it was found that in 68.7% of the cases the disease began from algic manifestations. Under observation there were 48 patients with syringomyelia who complained of pains in the neck and the upper extremities. A connection between the algic syndrome and the pathology of the vertebral column cervical division was noted. It was local myopathosis which was found to be the principle factor causing the algic manifestations. It was discovered even in patients who had no cervical osteochondrosis. When the deep was discovered even in patients who had no cervical osteochondrosis. When the deep sensitivity was affected the myopathoses could not be determined. The myopathoses were characterized electromyographically by the presence of constant bioelectrical activity registered by needle electrodes. In the spectrum of motor unit activity shortened and prolonged potentials were revealed simultaneously. The regional circulatory changes in the upper extremities were found to be connected to a considerable extent with the intensity of the algic manifestations in local myopathoses. Segmental message is an effective means for eliminating the local myopathoses.

Arm↗

[Terminal ventriculostomy in the treatment of syringomyelia].

Terminal ventriculostomy is one of the methods used in surgical treatment of syringomyelia. The procedure is well tolerated and may be used in cases in which atlanto-occipital decompression is too serious an operation. The author used the method in treatment of 10 patients with different stages of syringomyelia. In 5 cases the time of follow-up was 12--20 months, in 2 of them the result was very good with very great improvement, in 3 improvement was slight but the progression of the disease was halted. In 5 cases with shorter follow-up improvement was observed in 3 and no improvement immediately after the operation in 2 cases. Further follow-up of these patients will show whether the results of the operation will be permanent.

Adult↗

[Histopathological analysis of herniated cerebellar tonsils resected from the patients with Chiari type I malformation with syringomyelia].

Cerebellar tonsillectomy is a controversial treatment for Chiari type I malformation combined with syringomyelia. To demonstrate the validity of this procedure, we evaluated the postoperative clinical course, the histopathological finding in the resected cerebellar tonsils and the MR images of four patients. The patients were all women, ranging in age from 27 to 58 years old (mean: 43 years). The postoperative follows-up period lasted from 28 to 51 months (average: 38 mos), and the neurological symptoms and signs improved in all patients. Histopathological examination of the resected tonsils revealed a loss of Purkinje cells and granule cells. Vacuolated degeneration and chromatolysis were also seen, and modified Bielschowsky stain revealed axonal degeneration. These findings appeared to be irreversible. Postoperative T1-weighted MR images of the cervical and thoracic spine demonstrated a decrease in the size of the syrinx and the disappearance of evidence of tonsillar herniation in all patients. Postoperative phase-contrast MR images showed good CSF pulsation in the subarachnoid space at the craniocervical junction in all three patients examined. It was possible to avoid surgical complications by using careful microsurgical techniques. Based on these results, we concluded that cerebellar tonsillectomy is an adequate surgical strategies for treating Chiari type I malformation associated with syringomyelia.

Adult↗

[Chiromegaly in syringomyelia--radiologic studies of hand bones].

Chiromegaly, localized hypertrophy of a hand, is known to be a rare but pathognomonic trophic disturbance in syringomyelia. We recently reported that the sympathetic hyperfunction was associated with chiromegaly. This time we carried out radiological studies of hand bones in 4 patients with syringomyelic chiromegaly. In simple roentogenography, enlargement and enhanced shadows of hand bones were recognized in the chiromegalic side. Radiographic measurements showed metacarpophalangeal length and width were increased by 1.7% and 8.8% respectively compared to the normal side, and metacarpal index was decreased significantly in the chiromegalic side. In X-ray photodensitometry (MD/MS method) of the second metacarpal bones, bone density was increased by 10.6% compared to the normal side. These results suggested that bone hypertrophy was associated with chiromegaly in patient with syringomyelia. Trophic disturbance may exert some influence on the bone tissue.

Absorptiometry, Photon↗

Syringomyelia and scoliosis in children.

We reviewed 28 patients < 18 years of age with scoliosis and syringomyelia. Children with scoliosis and syringomyelia had an equal incidence of left- and right-sided curves with a normal sagittal alignment. Most were first seen at Risser 0 with significant curves, and curve progression occurred in half of the patients. Bracing was not effective in preventing curve progression. Neurologic signs, present in most children, stabilized after syrinx drainage. Neither the sex or age of the child, nor the type of curve, nor the drainage of the syrinx was predictive of curve progression.

Adolescent↗

[CSF dynamics in the patients with syringomyelia associated with Chiari's malformation--quantitative analysis on cine MRI].

In a series of 12 patients with syringomyelia associated with Chiari's malformation, the authors quantitatively analyzed cerebrospinal fluid (CSF) dynamics in the subarachnoid space of the craniospinal junction, using cine MRI combined with pre-saturation method. In most of subjects, cine MRI revealed (1) decreased or increased maximum velocity of CSF in the caudal direction and (2) disturbed CSF motion in the caudal direction (delayed % cardiac cycle) in the craniospinal junction, strongly suggesting disturbed CSF dynamics in the craniospinal junction because of the tonsilar herniation. Of 12 subjects, 8 patients underwent foramen magnum decompression and 4 underwent syringo-subarachnoid shunt (SS shunt). In the patients who showed marked collapse of syrinx after foramen magnum decompression, follow-up cine MRI revealed the normalization of % cardiac cycle, representing postoperative improvement of CSF dynamics in the craniospinal junction. On the other hand, % cardiac cycle did not improve significantly in the patients who did not show marked collapse of the syrinx or suffered from meningitis after surgery. Significant changes were not observed in the patients who underwent SS shunt. In summary, these results suggested that cine MRI combined with pre-saturation method could detect the pathophysiological changes and evaluate the efficacy of the surgery, especially foramen magnum decompression, in the patients with syringomyelia associated with Chiari's malformation.

Adolescent↗

Foramen magnum decompression with removal of the outer layer of the dura as treatment for syringomyelia occurring with Chiari I malformation.

The surgical results of foramen magnum decompression in seven patients with syringomyelia associated with Chiari I malformations are reported. The patients were two men and five women, ranging in age from 23 to 54 years (mean, 30 yr). A bony foramen magnum decompression combined with the removal of the outer layer of the dura mater was performed in seven patients. In an average postoperative follow-up period of 2 years (range, 1-3 yr), neurological symptoms and signs improved in six of seven patients and were unchanged in one patient. Postoperative magnetic resonance imaging scans taken a few weeks after surgery showed a decrease in the size of the syrinx or a disappearance of the syrinx in five of seven patients; a few months were required for a similar reduction in the remaining two patients. In all seven patients, the decompression of the foramen magnum proved to be sufficient, as disclosed by postoperative magnetic resonance imaging. Foramen magnum decompression by our surgical technique is advantageous because all the procedures are extradural and there are, therefore, fewer postoperative complications than the foramen magnum decompression techniques previously reported. Foramen magnum decompression by the authors' surgical technique is effective as the initial surgical treatment for syringomyelia associated with Chiari I malformations.

Adult↗

[Syringomyelia due to arachnoiditis. Clinical-radiological description of 5 patients].

Five patients (mean age 44 years--range 21-67 years) with arachnoiditis of different origin who posteriorly developed syringomyelia are presented. The etiopathogenic mechanism of the arachnoiditis was post injury in one case, 2 patients had history of meningitis (tuberculous and pneumococcic) and in the other two no related factor was found. The period of latency among the causes which originated arachnoiditis and the diagnosis of syringomyelia oscillated between 10 months and 16 years. The localization of the cavity was dorsal in 3 cases, cervical in another and in the last it was extended along the whole spine. No patient demonstrated the Arnold-Chiari malformation nor basilar impression. Analyzing the clinical history, radiologic studies and surgical findings the most probable etiopathogenic mechanism involved in each case is discussed.

Adult↗