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Experimental communicating syringomyelia in dogs after cisternal kaolin injection. Part 2. Pressure studies.

Pressure measurements in cerebrospinal fluid have been made from the ventricles and the spinal subarachnoid space in dogs made hydrocephalic by intracisternal injections of kaolin. Raised intraventricular pressure has been confirmed in the confirmed in the ventricles. The spinal pressure is usually markedly different from that in the head, confirming that the arachnoiditis produced around the cistern isolates the spinal subarachnoid space from the CSF in the head. Pulsatile pressures have been investigated specifically and the interrelationship between the pulsation and baseline pressures in the development of communicating syringomyelia discussed. There are differences between these experimental animals and the human disease, particularly in the baseline pressures which are usually equal in the head and the spine of humans at rest even in cases with syringomyelia.

Animals↗

Increased growth of myoblasts from hypertrophic muscles in syringomyelia.

Proliferation and differentiation of myoblasts from hypertrophic muscles were studied "in vitro" in two cases of syringomyelia with muscle hypertrophy (MH). Their myoblast growth was compared with that of muscle cells sampled on the contralateral side in the same patients and in control subjects. The effect of a circulating factor was tested using patient sera in place of fetal calf and horse sera. The results showed that MH cells were morphologically abnormal (giant and granular). MH myoblasts proliferated more rapidly than contralateral and normal myoblasts, their fusion was accelerated and the resulting myotubes synthesized higher levels of protein. MH sera increased these effects. Serum factors are therefore likely to be involved in "in vivo" muscle hypertrophy. These findings suggest that the pathogenesis of muscle hypertrophy in syringomyelia involves acquired abnormalities due to molecules released in response to neural lesions.

Adult↗

Successful treatment of adult Arnold-Chiari malformation associated with basilar impression and syringomyelia by the transoral anterior approach.

A case of adult type I Arnold-Chiari malformation associated with basilar impression, syringomyelia, atlantoaxial dislocation, and occipitalization of the atlas is reported. Preoperative magnetic resonance imaging clearly revealed evidence of severe anterior compression of the cervicomedullary junction due to basilar impression and a sharp clivoaxial angle. Therefore, transoral anterior decompression and fusion were performed, resulting in an improvement of the patient's neurologic signs and symptoms. Postoperative magnetic resonance imaging showed an obvious reduction of the tonsillar herniation and syringomyelia, as well as an improvement of the cervicomedullary compression.

Adult↗

Spinal neurenteric cyst in association with syringomyelia: case report.

A case of spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia is presented. Neuroradiological evaluation was performed by means of magnetic resonance imaging of the spine and a computed tomography myelograph. The diagnosis of neurenteric cyst was established by histological and immunohistochemical studies. The pathogenesis of syringomyelia associated with an extramedullary mass lesion is discussed.

Adult↗

Syrinx-fourth ventricle and lateral ventricle-peritoneal shunts for congenital hydrocephalus with syringobulbia and syringomyelia: case report.

A case of congenital hydrocephalus with syringobulbia and syringomyelia was reported. Although his neurological condition had deteriorated because of shunt malfunction, progressing syringobulbia, and syringomyelia, he recovered because of the direct communication from the syrinx in the medulla to the fourth ventricle, the shunt from the syrinx in the cervical spinal cord to the fourth ventricle, and the lateral ventriculoperitoneal shunt.

Adult↗

HLA and syringomyelia.

The HLA status of 53 patients with syringomyelia was assessed. Forty had an associated Chiari anomaly. A significant increase of HLA-A9 was found. Genetic factors may influence the development of syringomyelia.

Budd-Chiari Syndrome↗

Somatosensory evoked potentials and magnetic resonance imaging in syringomyelia.

Somatosensory evoked potentials (SEPs) to median and posterior tibial stimulation were obtained in 22 patients with syringomyelia. All patients had magnetic resonance imaging (MR) which defined the maximum transverse diameter of the syrinx as well as its longitudinal extension. SEP was abnormal in 16 (72%) patients. Median and posterior tibial SEPs were abnormal in 11 and 15 patients respectively. Both tests were abnormal in 10 patients. Ten patients showed absence of one or more central potentials (P/N13, N20, N22) and 7 patients demonstrated increased conduction times (N9-N20, P/N13-N20, N22-P40). The mean maximum transverse diameter of the syrinx was 7.5 mm in patients with normal SEPs and 16.2 mm in patients with abnormal SEPs. Abnormal SEP was observed in all 5 patients with loss of position sense, in 9 of 13 (69%) with loss of superficial pain and temperature, and 1 of 2 patients with motor deficit only. Central SEP abnormalities were observed in 3 of 5 patients with sensory deficits indistinguishable from a peripheral neuropathy and in 2 patients in the asymptomatic extremity. Three of 4 patients with syringomyelia and Chiari malformation had a normal SEP.

Adolescent↗

Subpially recorded cervical spinal cord evoked potentials in syringomyelia.

Intraoperative spinal cord evoked potentials (SCEPs) to median nerve stimulation were detected subpially from the dorsal surface of the cervical spinal cord in 5 patients with cervical syringomyelia and were compared to normal SCEPs obtained from the unaffected side in 6 patients during intraoperative monitoring of dorsal root entry zone lesion. Normal SCEP began with a positive deflection P9 and a complex N11/N13 with several low amplitude short potentials superimposed on the N11/N13. The complex was followed by a second negative potential N2 and a late prolonged positivity, P. In the 4 patients in whom median nerve somatosensory evoked potentials (SEPs) were present preoperatively, SCEP consisted of the N11 potential and the following low amplitude short (LAS) potentials, while the N13 wave was missing. In the fifth patient, in whom the preoperative median nerve SEP was missing, SCEPs were of much lower amplitude and shorter duration than normal. The potentials N2 and P were not recorded in any of our 5 patients. Changes in N13 wave, N2 and P potentials noted in syringomyelia were presumed to be the result of destruction of the spinal cord dorsal horn neurons caused by spinal cord central cavitation.

Adult↗

The relation between functional deficits, motor and sensory conduction times and MRI findings in syringomyelia.

Opinions about the relation between the dimensions of the syrinx and the severity and distribution of symptoms in patients with syringomyelia are controversial. Therefore, this study investigates the relation of clinical symptoms, a disability score, quantified (1- and 2-dimensional) radiological findings (magnetic resonance imaging, MRI) and electrophysiological data (somatosensory and motor evoked potentials) in 22 patients with syringomyelia. There was a close relation between clinical symptoms and electrophysiological data. By both electrophysiological methods subclinical deficits could be detected. Furthermore, the results disclosed that the clinical symptoms, the degree of disability and the duration of the disease are not related to the dimensions of the syrinx or the electrophysiological results obtained by investigating the long ascending and descending spinal tracts of the lower limbs. Our findings suggest that, besides the syrinx, other factors not directly visible in the MRI are responsible for the development and progress of clinical symptoms.

Adolescent↗

The pathogenesis of syringomyelia in spinal cord ependymoma.

A spinal cord ependymoma with syringomyelia is presented. The pathogenesis of syrinx formation, associated with intramedullary tumors is not fully understood. In order to examine the mechanism of formation of the tumor-associated syrinx, syrinx fluid was obtained during surgery and concentrations of proteins were measured in the syrinx fluid, the cerebrospinal fluid (CSF) and blood serum. Protein analysis of fluid specimens showed the fluid in tumor-associated syrinx to be an exudate. This strongly indicates that, in this case, intramedullary tumor-associated syringomyelia is based on disruption of the blood-brain barrier.

Blood Proteins↗

Endoscopic third ventriculostomy in the management of Chiari I malformation and syringomyelia associated with hydrocephalus.

BACKGROUND: Chiari malformation with syringomyelia is conventionally managed with foramen magnum decompression and duroplasty. In presence of hydrocephalus, a ventriculoperitoneal shunt insertion has been the initial preferred mode of treatment. METHODS: Two patients of Chiari I malformation with syringomyelia who had associated hydrocephalus were initially treated with endoscopic third ventriculostomy (ETV). RESULTS: At follow up, both the patients symptomatically improved. Repeat MRI studies revealed collapse of the syrinx cavity and reduction of the Chiari malformation. CONCLUSIONS: Chiari malformation and syrinx associated with hydrocephalus may be effectively managed with endoscopic third ventriculostomy.

Adult↗

Anaesthesia for caesarean section in a patient with syringomyelia and Arnold-Chiari type I malformation.

A 37-year-old primiparous woman with syringomyelia and Arnold-Chiari type I malformation was scheduled to undergo elective caesarean section for a fetus in the breech presentation. Caesarean section was performed under general anaesthesia without complications; all we observed was an exaggerated response to atracurium. The patient was discharged home 7 days after the operation without neurological deterioration. General anaesthesia was chosen in this patient to avoid any spinal manipulation that could increase intracranial pressure or reduce intraspinal pressure and cause deterioration of neurological symptoms. Syringomyelia is a progressive myelopathy characterised by cystic degeneration within the spinal cord, which causes severe neurological deficits. The anaesthetic management is discussed.

Adult↗

Conservative treatment of a patient with syringomyelia using chiropractic biophysics protocols.

OBJECTIVE: To present a case of a 41-year-old man with syringomyelia and intractable pain and the subsequent reduction of symptoms. CLINICAL FEATURES: This patient acquired a traumatically induced syrinx in his upper cervical spinal cord after he fell approximately 9 feet and landed on his head, upper back, and neck 9 years before presenting for care. He was diagnosed with a spinal cord cyst (syrinx), located at approximately C2 through C4 after magnetic resonance imaging. In 1995, the patient underwent occipitoatlantal decompression surgery, which improved his symptoms for a short time. INTERVENTION AND OUTCOMES: The patient was treated using Clinical Biomechanics of Posture protocol. The patient was seen 26 times over the course of 3 weeks. His scale for pain severity decreased 50% and other subjective complaints decreased. His posture improved based upon pretreatment and posttreatment lateral cervical radiographs, showing a change from a 10 degrees lordosis with midcervical kyphosis to a 30 degrees lordosis. One-year follow-up examination showed stable improvement in the cervical lordosis and pain intensity. CONCLUSION: This case represents a change in subjective and objective measurements after conservative chiropractic care. This case provides an example that structural rehabilitation may have a positive effect on symptoms of a patient with syringomyelia.

Accidental Falls↗

The role of altered impedance in the pathophysiology of normal pressure hydrocephalus, Alzheimer's disease and syringomyelia.

Normal pressure hydrocephalus, Alzheimer's disease and syringomyelia appear to be completely unrelated diseases, however, they share a reduction in subarachnoid space compliance as part of their pathophysiology. This paper discusses the physiology of pulsatile fluid flow and its relationship to compliance/impedance. Unlike continuous or non-pulsatile flow where the vessel resistance and pressure gradient are the major determinants of the volume of fluid flowing, when the fluid flow in a vessel pulsates then the vessel compliance/impedance becomes important. A reduction in compliance in the craniospinal cavity in each of the three diseases discussed, leads to a limitation of the outflow vessel compliance. Therefore, there is an increase in outflow vessel impedance. The venous blood, CSF and interstitial brain/spinal cord fluid all have significantly pulsatile flow and an increase in the impedance of the fluid outflow in each disease would limit the volume of these fluids as they attempt to cross the subarachnoid space. It is hypothesised that a reduction in the efficiency of the outflow of venous blood, CSF and interstitial brain/spinal cord fluid would lead to the accumulation of CSF in NPH, cord fluid in syringomyelia and delay the excretion of beta amyloid via the interstitial drainage pathways in AD.

Alzheimer Disease↗

Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report.

BACKGROUND: Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia. CASE DESCRIPTION: The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved. CONCLUSION: We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication.

Arnold-Chiari Malformation↗

Cervical vertebrae measurements in syringomyelia.

Lateral X-rays of the cervical spine taken under standard conditions in 69 cases of syringomyelia have been measured to assess the relative sizes of the canals and bodies. One hundred and five cases without cervical spine abnormality provided controls. The expected expansion has been observed in males from C5 to C7 and in females from C3 to C7. The oddity that the expansive process seemed to come higher in females than in males has been noted by other workers. In both sexes it has been found that diminution of vertebral body size is a more consistent finding than enlargement of the canals and correlation coefficients have confirmed that enlargement of the canal appears to take place at least in part at the expense of the body. Possible factors in the enlargement are discussed. Oblique measurements taken in 57 syrinx cases and 89 controls showed the expected change caused by the view of the lower cervical vertebrae being closer to AP and pictures of the upper vertebrae being progressively closer to lateral views. There was a tendency for the bigger oblique diameters to occur in these cases with the biggest sagittal diameters and an insignificant tendency for the biggest canals to have a diminished difference between the two oblique measurements. There was no evidence that oblique measurements could be used to detect slight degrees of enlargement in cases of syringomyelia.

Adult↗

Micturitional disturbance in syringomyelia.

Micturitional histories and urodynamic studies were performed in 14 patients with syringomyelia. Eleven patients were revealed to have urinary symptoms including difficulty of voiding in 8, urinary retention in 3, nocturnal and diurnal urinary frequency in 3, urinary incontinence in 2, and sense of urgency and enuresis in one. These urinary symptoms appeared after 5.3 years (ranging from 2 months to 13 years) from the occurrence of the neurological symptoms. Urodynamic studies revealed detrusor hyperreflexia in 7, detrusor areflexia in 4, detrusor-sphincter dyssynergia in 4 and uninhibited sphincter relaxation in 2 patients. Analysis of the motor unit potentials of the external sphincter revealed 5 of 6 patients had high amplitude or polyphasic neurogenic changes. Supranuclear as well as nuclear types of parasympathetic and somatic nerve dysfunctions seemed to be responsible for micturitional disturbance in our patients with syringomyelia. During the follow-up period of 2 to 63 months, urinary symptoms gradually improved in 4 of 6 patients after syringosubarachnoid shunts and in 3 of 4 after alpha-adrenoreceptor blocking agents.

Adult↗

Syringomyelia in myotonic dystrophy due to spinal hemangioblastoma.

BACKGROUND: Syringomyelia is an uncommon, poorly understood finding in patients with myotonic dystrophy. METHODS: We describe a patient with myotonic dystrophy and neck pain in whom an extensive neuroradiologic diagnostic work-up was carried out. RESULTS: Magnetic resonance imaging revealed a large intramedullary cavity extending from the bulbo-medullary junction to the conus medullaris. After intravenous Gadolinium-DTPA administration, an enhanced nodule was seen at T6. Spinal arteriography showed a single hypervascular nodule and slow flow perimedullary draining veins consistent with hemangioblastoma. After removal of the nodule, a partial collapse of the intramedullary cyst was observed. CONCLUSIONS: Intramedullary tumors can underlie syringomyelia in patients with myotonic dystrophy and have to be actively investigated with modern neuroradiologic investigations.

Adolescent↗