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PubMed1999

Occipital fibrous dysplasia tonsillar herniation and cervical syringomyelia.

A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foraman magnum has been implicated in the development and subsequent progression of syringomyelia. This report highlights the gradual development of a small posterior fossa due to fibrous dysplasia of the occipital bone with consequent tonsillar herniation resulting in syringomyelia.

Adult↗
PubMed1994

Syringomyelia: clinical observations and experimental studies.

Although cavitary lesions of the spinal cord have been recognized for centuries, only recently have effective, noninvasive imaging techniques allowed antemortem diagnosis of this clinical syndrome. Methods of treatment have not been consistently successful in alleviating or reversing the clinical symptoms caused by these cystic lesions. Incomplete understanding of the underlying pathologic basis for the syringes has impeded the development of effective methods of treatment. This review documents historical considerations regarding clinical observations and experimental studies of this entity and the animal models that have been reported for each of the major types of syringomyelia. Recent studies have suggested that development of a relevant animal model of posttraumatic syringomyelia is imminent. Successful development of an experimental model will not only permit definition of the pathogenesis of cyst formation but also provide methods for testing of therapeutic interventions.

Animals↗
PubMed1998

Extradural anaesthesia for caesarean section in a patient with syringomyelia and Chiari type I anomaly.

We describe elective Caesarean section performed under extradural anaesthesia in a parturient with symptomatic syringomyelia and coexisting Chiari type I anomaly. Syringomyelia is reviewed and the anaesthetic implications of the condition discussed. Anaesthesia should be directed primarily at avoidance of increased intracranial pressure, which can cause sudden deterioration in these patients.

Adult↗
PubMed1985

Posttraumatic cervical syringomyelia. Incidence, clinical presentation, electrophysiological studies, syrinx protein and results of conservative and operative treatment.

In eleven years, 30 (3.2%) of 951 patients with spinal cord injury developed cervical syringomyelia. This condition was found in 22 (4.5%) of 488 posttraumatic tetraplegic and 8 (1.7%) of 463 posttraumatic paraplegic patients; the incidence was about 8 per cent in patients with complete tetraplegia. This study demonstrated the rarer clinical manifestations of syringomyelia, namely autonomic dysfunction, alterations in the sensory level with postural changes, the early occurrence of tendon areflexia and painless motor deterioration. Prolonged F wave latencies were present in all patients with a demonstrable syrinx and a higher protein content was found in the syrinx than in the cisternal fluid. Some of the symptoms and signs in a proportion of the patients treated conservatively remained stable without operative treatment over a number of years. Most of the patients in whom operation was performed for progressive motor weakness or severe pain had good postoperative results although a few developed late sensory or motor changes. There was no benefit in operating on a patient with a small syrinx.

Adult↗
PubMed1991

Pain-related somatosensory evoked potentials in syringomyelia.

Pain-related somatosensory evoked potentials following CO2 laser stimulation (pain SEPs) and conventional electrically-stimulated SEPs (electric SEPs) were examined in 8 patients with syringomyelia who showed various forms of dissociated sensory loss. Unlike clinical examination using a pin or needle, pain SEP is considered to be an objective and quantitative test to investigate functions of peripheral and central sensory pathways responsible for pain-temperature sensation (A delta fibres and the spinothalamic tract). Pain SEPs were abnormal in all patients. The results were generally compatible with the degree of a clinical impairment of pain-temperature sensation. Subclinical abnormality was detected in 3 patients. Electric median nerve SEPs using the scalp reference (Fz) were normal in 6 out of 8 patients. However, anterior and posterior cervical responses using a noncephalic reference were absent or small in 7 patients. Electric SEPs following tibial nerve stimulation were normal in 7 patients. These findings suggest that the function of the ascending fibres through the dorsal columns is intact in most patients, whereas the dorsal horn, where a fixed cervical potential is generated, is impaired. Pain SEPs combined with electric SEPs therefore appear to be extremely useful for investigating physiological function in the sensory pathways in patients who show 'dissociated sensory loss' such as in syringomyelia.

Adult↗
PubMed2003

A novel nonsense mutation in the ABC1 gene causes a severe syringomyelia-like phenotype of Tangier disease.

Tangier disease is a rare autosomal recessive disorder caused by mutations in the recently identified ATP-binding cassette transporter 1 gene (ABC1). A typical clinical manifestation of Tangier disease is peripheral neuropathy. Former studies differentiated between two manifestations: the more frequent mono- or polyneuropathic form and a syringomyelia-like type. It is unknown whether specific mutations in the ABC1 gene or a particular genetic background are responsible for either of these forms. A family is presented comprising a case with a severe syringomyelia-like phenotype of Tangier disease and absence of cardiovascular disease. Sequencing analysis of the ABC1 gene was performed. A new homozygous C-->T transition in exon 18 was found in the index patient. This mutation results in a stop codon at position 909 (R909X) leading to premature termination of translation. Her clinically asymptomatic daughters, her sister and one of her nieces were heterozygous. Sural nerve biopsies were studied in the index patient at the age of 45 and 54 years; both revealed a severe neuropathy, characterized by a subtotal and finally complete loss of nerve fibres. The entire loss of Schwann cells resulted in an extraordinary form of endoneurial sclerosis. Only rare capillaries, lipid-laden macrophages and fibroblasts had survived in the endoneurium. This case appears to be unique in respect to the underlying novel mutation in the ABC1 gene and its association with complete endoneurial sclerosis of all fascicles in the sural nerve and absence of cardiovascular disease.

ATP Binding Cassette Transporter 1↗
PubMed1997

Secondary syringomyelia due to intramedullary spinal cord metastasis. Case report and review of literature.

Intramedullary spinal cord metastasis is relatively rare. We describe a patient having intramedullary spinal cord metastasis associated with syringomyelia, confirmed by magnetic resonance imaging, in a patient who had poorly differentiated carcinoma of the lung. The patient responded to treatment with steroids and radiotherapy, with complete resolution of neurologic symptoms and syringomyelia.

Adult↗
PubMed1990

Motor-evoked potentials reflect spinal cord function in post-traumatic syringomyelia.

The purpose of this study was to examine electrophysiologic abnormalities, including motor-evoked potentials, in a patient with post-traumatic syringomyelia before and after syringopleural shunting. A patient with C5 quadriplegia presented with pain, ascending sensory loss, and new weakness in the left upper extremity two yr after spinal cord injury. MRI revealed a syrinx extending from C2 to T12. We measured central motor conduction times (CMCTs) to the biceps, median F-wave latencies, needle electromyography and motor nerve conduction studies. Six days before surgery, CMCTs were 9.0 ms on the left and 7.8 ms on the right (normal less than 8.0), median F-waves were absent on the left and needle EMG revealed evidence of denervation in the left biceps. Fifteen days after syringopleural shunting at the T7 level, CMCTs had dropped to 6.9 ms on the left and 4.6 ms on the right; the left median F-wave reappeared with a normal latency. Repeat MRI revealed the syrinx to be smaller in diameter. These results suggest that CMCTs measured from magnetic stimulation of the motor cortex may be useful in the diagnosis of post-traumatic syringomyelia, as well as for following such patients postoperatively.

Adult↗
PubMed1980

High resolution computed tomography in cervical syringomyelia.

Thirty-two cases of documented cervical syringomyelia, 13 of which were already surgically proven, were explored by high resolution computed tomography (CT). A central cavitation was demonstrated in all cases. High resolution CT appears to be more reliable than myelography and should be the examination of choice in cervical syringomyelia.

Cervical Vertebrae↗
PubMed1987

Abnormalities in spinal neurons and dorsal root ganglion cells in Tangier disease presenting with a syringomyelia-like syndrome.

A woman with homozygous Tangier disease had progressive syringomyelia-like neuropathy. She died with cardiac failure at age 61. A sural nerve biopsy taken at age 60 had shown lipid storage in Schwann and interstitial cells, and a pronounced loss of unmyelinated fibers. The neurons of the L5 spinal ganglion and, to a lesser extent, all neurons of the sacral spinal cord, contained large lipid inclusions which in electron micrographs differed from those in Schwann and satellite cells. There was no storage material in glial cells. The neuronal inclusions were membrane-bound and consisted of electron-dense and electron-lucent components. There was evidence of neuronal death in the spinal ganglion, and a diameter histogram showed that small cytons had preferentially been lost. The inclusions probably were secondary lysosomes or residual bodies, and resembled giant lipofuscin granules. Nevertheless, they were uncolored and displayed weak autofluorescence as compared to the aging pigment in control ganglia. It is tentatively suggested that the syringomyelia-like neuropathy in Tangier disease represents a lysosomal storage disorder preferentially affecting small dorsal root ganglion cells.

Diagnosis, Differential↗
PubMed1991

Percutaneous drainage and serial magnetic resonance imaging in the diagnosis of symptomatic posttraumatic syringomyelia: case report and review of the literature.

A patient with high cervical tetraplegia with new-onset headaches and posttraumatic syringomyelia is presented. Percutaneous drainage of the syrinx resulted in a resolution of the headaches and collapse of the syrinx on follow-up magnetic resonance imaging (MRI). The return of the symptoms correlated with the re-expansion of the syrinx on MRI. The patient underwent syringopleural shunting with persistent resolution of the symptoms and collapse of the syrinx on MRI. The value of percutaneous drainage and serial MRI to determine the clinical significance of posttraumatic syringomyelia is discussed.

Adult↗
PubMed1997

Syringomyelia treated with a nonvalved syringoperitoneal shunt: a follow-up study.

OBJECTIVE: Eighteen patients with syringomyelia received nonvalved syringoperitoneal shunts during the years 1987 through 1996. In 15 cases, the fistulae were multicompartmental, either separated by segments of normal cord or septated. METHOD: Even in the multicystic cases, only one syringeal catheter was introduced, usually into the caudalmost cavity. Access to the fistula was obtained via a midline myelotomy, which was performed in an area at which the spinal cord overlying the fistula was at its thinnest. RESULTS: The progressive clinical course of syringomyelia was arrested in all patients. Surgery resulted in improvement for 11 patients. Five patients remained unchanged without further progression. Two patients became worse as the result of new deficits caused by surgery. In four patients, the myelotomy lead to new but discrete sensory loss of minor importance. Postoperative magnetic resonance images showed a rapid and persistent collapse of all fistulae in all patients. CONCLUSIONS: We conclude that syringoperitoneal shunting is favorable in patients with large fistulae. In patients with Chiari malformations, the procedure may be a second alternative to foramen magnum decompression.

Adolescent↗
PubMed1993

Scoliosis associated with syringomyelia.

From January 1986 to December 1991, 1,059 patients with scoliosis of 20 degrees or more were reviewed. By magnetic resonance imaging, 43 patients were found to have syringomyelia. Thirty-eight patients had Chiari type 1 malformation. Twenty-two of the patients were younger than 10 years. Scoliosis was the only presenting symptom in 32 patients. All patients had some neurologic abnormalities. Fifteen patients had left thoracic curves. The incidence of scoliosis with syringomyelia in our outpatient clinic was 4.0%; 18.4% in boys, and 2.6% in girls. Magnetic resonance imaging should be performed in patients who have abnormal neurologic findings.

Adolescent↗
PubMed1996

Intraoperative improvement of somatosensory evoked potentials and local spinal cord blood flow in patients with syringomyelia.

This report is the first to correlate data concerning intraoperative somatosensory evoked potentials (SSEPs) and local spinal cord blood flow (ISCBF) in patients with syringomyelia. In a consecutive study, bilateral median nerve SSEPs were recorded intraoperatively in 13 patients undergoing a syrinx shunt to the posterior fossa cisterns (syringocisternostomy). ISCBF was measured in five of these patients using laser doppler flowmetry (LDF) calibrated in arbitrary units (AU). SSEP recordings obtained 30 min after syrinx decompression demonstrated a slight but consistent reduction of N20 latencies (mean change: 0.53 ms right, p < 0.003; 0.58 ms left, p < 0.001) concurrent with a similar but less consistent increase of N20 amplitudes (0.16 mV right, p = 0.256; 0.29 mV left, p = 0.03). Prior to shunting, LDF recordings from the spinal cord overlying syrinxes revealed very low ISCBF values in five of five patients (mean LDF, 13.2 AU +/- 15.3 SD). Immediately after shunting, there was a dramatic rise of ISCBF (mean LDF, 241.2 AU +/- 106.3 SD) associated with visualized hyperemia of the spinal cord and pial vessels. The ISCBF fell to intermediate levels after 2 min (157.2 AU +/- 33.0 SD) and remained at these levels during the interval of recording (5 min). Hyperventilation testing in two patients prior to shunting revealed no change in ISCBF consistent with a loss of CO2 vascular reactivity and a paradoxical increase of ISCBF in one patient 5 min after shunting. Each patient in this study experienced neurological improvement in the immediate postoperative period associated with collapse or disappearance of the syrinx on magnetic resonance imaging scans. Because syrinx shunting results in an acute decompression of the distended spinal cord, it is possible that the rapid improvement of SSEPs reflects a relief of mechanical factors such as stretching and compression of nervous tissue. However, the LDF findings in this study suggest that distended spinal cord cavities are also capable of producing regional ischemia. A significant reduction of ISCBF is a possible contributing cause of neurological injury and SSEP abnormalities. Intraoperative improvement of SSEPs and ISCBF were found to correlate well with neurological recovery following syringocisternostomy. Our results indicate that SSEP monitoring can provide useful information during surgical procedures for syringomyelia and that further experience with LDF monitoring may provide insights into the pathophysiology of this condition.

Adolescent↗
PubMed2002

Outcomes in surgical treatment of "idiopathic-like" scoliosis associated with syringomyelia.

Patients with "idiopathic-like" spinal deformities associated with syringomyelia were retrospectively reviewed. Ten patients had surgical stabilization of their curvatures with at least a 2-year follow-up, and an additional five patients were evaluated for deformity pattern with <2 years of follow-up. Paralytic curve patterns, scoliosis associated with spina bifida, congenital scoliosis, or other associated syndromes were discarded. All 10 patients with surgery who were followed for an average of 46 months lost 10 degrees correction above, through, or below the instrumented segments. A total of 50% lost correction through the instrumented segments. Anterior fusion stabilized the instrumented portion of the spine better than posterior instrumentation alone. Eighty percent of the 15 patients had thoracic kyphosis >40 degrees. Only one patient was lordoscoliotic. Syringomyelia deformities tend to be kyphoscoliotic in 80% of cases and behave more like paralytic curvatures postoperatively. MRI is recommended for apparent idiopathic scoliotic curvatures that are kyphoscoliotic and not lordoscoliotic.

Adolescent↗
PubMedDate not supplied

Management of scoliosis due to syringomyelia in childhood and adolescence.

To determine the effect of syringomyelia treatment (drainage) on the efficacy of orthotic management and safety of operative management of an associated scoliosis, the records of four boys with scoliosis due to syringomyelia were reviewed. The average follow-up was 8 years. On presentation, all had an abnormal neurologic examination. Each syrinx was treated by laminectomy and insertion of a syringosubarachnoid shunt. After drainage, three of four improved neurologically. Two older patients underwent uneventful posterior spinal fusion with Harrington distraction instrumentation after their drainage procedures. One boy developed kyphosis at the site of his laminectomy. Two young patients, whose curves were initially well controlled with bracing, developed continued curve progression over time. Drainage of the syrinx delayed but did not prevent curve progression in immature patients, but did allow use of distraction instrumentation without complication for operative management of the associated scoliosis.

Adolescent↗
PubMed1986

Noonan's syndrome with syringomyelia.

A case of Noonan's syndrome with cervical syringomyelia is presented here representing the second reported instance of such association. A CT of metrizamide myelography revealed a high density in the middle subintradural space in the cervical region below C2. Sensory dissociation was minimal and localized in the left C4. It could not be determined whether this association between syringomyelia and Noonan's syndrome is related or represents a chance.

Adolescent↗
PubMed1983

Computed tomography in the diagnosis of syringomyelia.

We describe the usefulness of computed tomography (CT) in the diagnosis of syringomyelia in 12 patients for whom the diagnosis had remained unconfirmed with other diagnostic methods. In 7 of these patients, CT myelography revealed an intramedullary cavitation filled with contrast medium. In 3 patients, the cavitation was not filled, and positive contrast medium was not used in another 2. In these 5 cases, however, CT visualized low-density areas within the cord. CT would seem to be the most preferable method for confirming the clinical diagnosis of syringomyelia and is particularly useful in atypical cases.

Adult↗