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PubMed1977

[Pathogenesis of syringomyelia].

The absence of an ependimic layer in cases of syringomyelia described in literature and in the author's own studies, the dislocation of cavities on the background of formted gray matter, the structure of the edge of the cavity, as well as sclerosis, a gyalinosis of vessels and fibrosis of the meninx--all these phenomena confirm the opinion of syringomyelitic cavities as an acquired defect and not dysraphia. A comparative study of the morphogenesis of medullar cavities in inflammatory, vascular, traumatical processes and in syringomyelia permits to mark their histological similarity and postnecrotic genesis. The glyosis in syringomyelitic cavities has a different character. The fibrillar glyosis along the edge of the cavities appears due to reparative changes and plays a delineating role. Irritative glyosis appears more frequently in angiomatosis, accompanied by chronic microhemorrhages. Expressed glyosis with a formation of cellular rosettes is to a certain extent a tumor (astrocytomaa, epeudimoma), while a syringomyelitic cavity--a histiolytic cyst of a tumor.

Adolescent↗
PubMed1995

[Neuropathology of syringomyelia].

Eighteen autopsy cases of syringomyelia were studied neuropathologically. In 5 cases associated with Chiari type I malformation, the syrinx was irregular in shape and communicated with the subarachnoid space at the entry zone of the posterior nerve roots. The central canal above the level of the syrinx was patent in 2 but closed in 3 out of 5 cases. In cases associated with Chiari type I malformation, communication between the syrinx and the subarachnoid space was considered to play an important role in the pathogenesis of syringomyelia. In 6 cases associated with Chiari type II malformation, the syrinx central canal, and the central canal was patent from the 4th ventricle to the syrinx in all cases. In these cases, direct continuity between the 4th ventricle and the syrinx was essential for the development of the syrinx. In cases associated with posterior fossa or spinal canal tumors, the local circulatory disturbance and/or edema due to the tumors were thought to cause the syrinx.

Adolescent↗
PubMed1996

[Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology].

INTRODUCTION: The major theories of the pathogenesis of idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and the Arnold-Chiari malformation (ARCH) are reviewed. A new theory involving a common pathology for SMI, ESCID and ARCH is proposed. Its confirmation depends on the presence of an abnormally low position of the conus medullaris (CM) in patients with SMI. Material and method. Of 292 patients with syringomyelia (SM), 231 patients with SMI were selected, and 55 of these were chosen in whom the level of the conus medullaris (NCM) could be determined, together with figures for SMI, ESCID and ARCH by magnetic resonance (RM). The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined. RESULTS: 96.54% of patients with SMI showed some descent of the cerebellar tonsils. 72.97% of the SMI patients also had ESCID: 74.07% of the patients in whom the conus medullaris was seen had scoliosis and a low position of the conus medullaris. 6% of the control group had a conus medullaris at the level of the body of L1, whilst 84.21% of the patients with SMI had a partial or complete image of the conus medullaris at this level. In these patients, an unusually low position of the conus medullaris was confirmed. CONCLUSIONS: The unusually low position of the conus medullaris in SMI and its close relationship to ARCH and ESCID make it likely that the same pathogenic mechanism is shared by them all. Abnormal asynchromy of growth of the notochord and the spinal cord gives rise to a distinct disorder which shows as different syndromes.

Adult↗
PubMed1977

Post-traumatic syringomyelia. Review of the literature and two new autopsy cases.

Two cases of post-traumatic syringomyelia studied macro- and microscopically are reported and compared with three previous autopsy cases described in the literature reviewed. The histological findings suggest that the pathogenesis may be explained by a pressure gradient in a small primary cavity. Although syringomyelia is a rare complication of traumatic spinal cord lesions, it is important to draw attention to the condition because it may be improved by surgical intervention.

Adolescent↗
PubMed1996

[Platybasia, basilar groove, odontoid process and kinking of the brainstem: a common etiology with idiopathic syringomyelia, scoliosis and Chiari malformations].

INTRODUCTION: A common aetiopathogenic theory for basilar groove (IMB), platybasia (PTB), odontoid retrocession (RTO), kinking of the brainstem (KTC) applied to idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and Arnold-Chiari malformation (ARCH) is presented. Confirmation is based on an abnormally low position of the conus medullaris (CMB) in the patients with SMI. MATERIALS AND METHOD: 292 patients with syringomyelia (SM), 231 with SMI were selected. Of these, 55 were chosen who had SMI and in whom the level of the conus medullaris (NCM) could be determined, together with the figures for SMI, IMB, PTB, RTO, KTC, ESCID and ARCH on cervical and lumbar MR. The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined. RESULTS: 32 patients had an increased basal angle (58.18%). There was an IMB in a quarter of the patients (25.45%). RTO was observed in half of the patients (47.27%). Just over one third presented a KTC. 6% of the control group had CM at the level of the body of L1, whilst 84.21% of the patients with SMI presented a partial or complete CM image at this level. CONCLUSIONS: CMB in SMIU and its close relationship with IMB, PTB, RTO AND KTC and also with ESCID and ARCH make it likely that they share the same aetiopathogenic mechanism: an abnormal lack of synchronization of the growth of the neuro-axis and the neural canal (AACNN), causing a specific disorder which is seen as different syndromes.

Adult↗
PubMed1996

Cleidocranial dysplasia and syringomyelia. Case report.

cleidocranial dysplasia (CCD) is a dysmorphic syndrome characterized by delayed ossification of the cranial sutures, clavicular hypoplasia and dental dysplasia. We recently observed a 24-year old male patient with CCD and associated syringomyelia and Chiari I malformation (CMI). Only three cases with such an association had been described. The role of posterior fossa bone dysplasia in the pathogenesis of CMI and syringomyelia is discussed.

Adult↗
PubMed1997

[Syringomyelia appearing in a short term after brain abscess].

We describe a rare case of syringomyelia that developed within only 4 months after the cure of the focal cerebritis and abscess. An 18-year-old man who had suffered from meningitis and brain abscess 1.5 years before, presented with pain and sensory disturbance in his left shoulder. Magnetic resonance image (MRI) showed a syrinx formation extending from the upper cervical to the lower thoracic cord. Investigation by MRI during the course of the brain abscess had the appearance of posterior cerebellar arachnoid cyst 3 months after the onset of brain abscess and syrinx developed within 9 months. After posterior fossa decompression and reopening of the Magendie's foramen, his symptom and MRI abnormality improved after one month. In contrast to reported cases of syringomyelia following cerebritis, syrinx formation in our case appeared in relatively a short time. We speculated that basal arachnoiditis and the formation of posterior cerebellar arachnoid cyst caused syrinx formation in a short term after cerebritis.

Adolescent↗
PubMedDate not supplied

[Magnetic resonance in the neuroradiologic diagnosis of syringomyelia].

Syringomyelia is the chronic progressive disease which etiology has still been discussed. The diagnosis and differential diagnosis of all SM types using magnetic resonance as specific and most sensitive method of examination have been presented in the study for the first time in our country. In two-year period since the introduction of this examination method in our Institute we examined 25 patients with 3 primary congenital and 22 with secondary SM, among whom 15 had post-traumatic and 7 had traumatic forms. All the changes characteristic for syringomyelia in neuroradiologic diagnosis were described in details. Magnetic resonance is primary and superior neuroradiologic method in all spinal processes for the discovery of neurologic, undefined myelopathic changes, such as SM. The methods of micro-surgical operative treatment are not the subject of this study.

Adolescent↗
PubMed1976

Craniofacial dysostosis with syringomyelia and associated anomalies.

A 16-year old boy had craniofacial dysostosis, hydrocephalus, and syringomyelia. Other anomalies included platybasia, a Klippel-Feil anomaly, webbed toes, and a cutaneous hemangioma. Evaluation included cerebral angiography, ventriculography, and myelography.

Abnormalities, Multiple↗
PubMed1987

Syringomyelia: electrophysiologic aspects.

The clinical and EMG findings in 44 patients with syringomyelia who were seen at the Mayo Clinic between 1976 and 1985 are presented. In 10 of the patients, somatosensory evoked potentials (SEPs) of the upper and lower extremities were obtained. All 44 patients had radiographic or surgical evidence of a cervical syrinx. The most common abnormality on nerve conduction studies was a reduced hypothenar compound muscle action potential amplitude (23 patients). Abnormal findings on needle electromyography were present in 33 patients and included sparse fibrillation potentials, reduced motor unit potential (MUP) recruitment, and chronic neurogenic MUP changes in muscles innervated by the C-5, T-1 roots, with the most pronounced changes in small hand muscles. Ulnar and median nerve SEPs were usually normal in the presence of a dissociated sensory loss and were usually abnormal when all sensory modalities were impaired. Abnormalities of tibial nerve SEPs were frequent and were related to impaired proprioceptive sensation in the lower extremities.

Electromyography↗
PubMed1992

AAEM case report #24: electrodiagnosis in posttraumatic syringomyelia.

An adult male with C-7 quadriplegia developed neck pain. Axillary F central latencies were prolonged, and MRI showed a syrinx extending to C-1. After shunting, F latencies normalized. At subsequent follow-up, a rostral syrinx persisted by magnetic resonance imaging (MRI); motor evoked potential (MEP) latencies were prolonged but F latencies were normal. Later, the syrinx was less distended by MRI, MEPs normalized, and strength improved. We discuss the electrophysiologic methods available for diagnosing and monitoring posttraumatic syringomyelia.

Adult↗
PubMed2005

Posttraumatic syringomyelia--a serious complication in tetra- and paraplegic patients.

Post-traumatic syringomyelia (PTS) is relatively rare, but its complications can be serious. In the beginning of the operative treatment (1900-1930), scarring could be reduced to a certain degree. In modern treatment (1980 1990) a shunt implantation showed also little effect in long-term follow-up studies. Influenced by the work of B. Williams, 58 PTS patients underwent surgery to create a pseudomeningomyelocele, an artificial CSF reservoir, performed to normalize the CSF flow. In a 10-year-postoperative follow-up study (minimum observation two years), good results were obtained in more than 70%.

Cerebrospinal Fluid Shunts↗
PubMed1988

Treatment of syringomyelia by syringo-peritoneal shunt.

Syringomyelic cavitation of various origins (excluding tumour) has been treated by syringo-peritoneal shunting (SPS) in 10 patients. The main clinical and radiological symptoms are reviewed and postoperative results evaluated; among the symptoms, pain is the most likely to improve after surgery. But the global outcome in the majority of cases remains unchanged, some patients showing neurological deterioration. Although MRI is of fundamental importance in diagnosis, the significance of the postoperative appearances on MRI has still to be elucidated. Other studies are certainly necessary to define the exact place of SPS in the treatment of syringomyelia.

Adult↗
PubMed1995

Experimental model of chronic tonsillar herniation associated with early stage syringomyelia.

This report describes an experimental model of chronic tonsillar herniation and its effects on the spinal cord. In ten rats, a small piece of chemically induced mammary cancer was transplanted to the supraoccipital bone. In all cases, the transplanted cancers grew into the posterior fossa, destroying the supraoccipital bone and compressing the cerebellum extradurally. In six of the ten rats, tonsillar herniation was observed at 8-14 weeks after transplantation. Transdural infiltration of the tumor cells was not apparent in any animal. In those rats with tonsillar herniation (n = 6), the spinal cord from the C5 to the T8 segments showed enlargement of the central canal without exception. Histological examination revealed the following changes: stretching and thinning of the ependymal cells; swelling of the astrocytic processes; and extra-cellular edema, predominantly in the dorsal gray matter, but also in the ventral inner portion of the dorsal column. In the control group (n = 4) and those rats without tonsillar herniation (n = 4), such histological changes of the spinal cord were not observed. Although the lesions can not be regarded as representing mature syringomyelia, they most likely constitute an earlier evolutionary stage.

Animals↗
PubMed1994

Operative treatment and prognosis of syringomyelia.

Twenty-two patients suffering from syringomyelia were treated operatively. Different shunt procedures were performed. Most often syringo-subarachnoid shunt (seven cases) and syringopleural shunt (eight cases) were used. Operative findings and complications were discussed. Postoperative improvement was observed in five patients, twelve were stable-unchanged, four showed further deterioration and one died. Operative treatment should be performed before gross neurological deficit is established.

Adolescent↗
PubMed1980

Dumb-bell sarcoma of the foramen jugulare with syringomyelia. A radio-induced tumour?

The clinicopathological findings of a 50-year-old man, who developed cervicothoracic syringomyelia at the age of 25 are presented. He was given radiation therapy at the age of 33. At the age of 57 he developed a foramen jugulare syndrome on the left, caused by a low grade leiomyosarcoma. Etiologically, the most attractive hypothesis appears to be that the tumour was induced by radiation therapy administered 24 years previously.

Adult↗
PubMed1985

Severe polyneuropathy in Tangier disease mimicking syringomyelia or leprosy. Clinical, biochemical, electrophysiological, and morphological evaluation, including electron microscopy of nerve, muscle, and skin biopsies.

Polyneuropathy in Tangier disease can be divided into three clinical types. The most severe form (type III) with a syringomyelia-like syndrome has been described in three cases only. Here, a fourth case of this type is presented. Because of unusual trophic disturbances even leprosy was suspected. Electrodiagnostic findings, including evoked cerebral potentials in this case, were suggestive of a generalized neuropathy with some degree of primary or secondary demyelination and implied possible impairment of central structures. Sural nerve biopsy, including electron microscopy and quantitative analysis, revealed a predominant reduction of smaller myelinated and unmyelinated fibres. The main morphological feature was the abundance of abnormal non-membrane-bound vacuoles in Schwann cells, mostly of the unmyelinated type, and in some endoneurial fibroblasts, macrophages and perineurial cells. There was no inverse relationship between lipid vacuoles and axons in Schwann cell complexes as suspected by others. An excess of endoneurial collagen as well as an increased fascicular area were obvious. In five skin biopsy specimens of different regions typical vacuoles were noted in Schwann cells, histiocytes, nevus cells, and rarely in perineurial cells.

Adult↗
PubMed1991

The natural history and results of surgery in 50 cases of syringomyelia.

In recent years, new neuroimaging techniques have revived interest in syringomyelia with respect to indications and results of surgery. Fifty patients, 36 of whom underwent surgery, have been reviewed. All patients but 3 underwent a new clinical assessment and 33 of them were also neurophysiologically investigated. In approximately one-third of the non-surgically treated patients the clinical course was benign. In 26 of the surgically treated patients an improvement was noted at the short-term assessment both for spasticity and pain, but in most of them it was not maintained in the medium term. Therefore, an accurate selection of the patients to be treated surgically is strongly recommended, particularly when the natural history of the disease is considered. Decompression of the posterior fossa seems to give the best results, yet no curative surgical treatment has been devised to date.

Adolescent↗