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PubMed1976

""Non-communicating'' syringomyelia: a non-existent entity.

Encroachment by man's overlarge forebrain is responsible for the anatomic substrate of syringomyelia, i.e. a hindbrain hernia developing in fetal life with persisting hydromyelia. Communication between the syrinx and 4th ventricle is readily disclosed at operation, but because of postmortem shrinkage, almost never at autopsy. Syringomyelia developing in 16 of 864 post-traumatic paraplegics has been classified non-communicating. However, the syrinx, its fluid, and the ultimate clinical picture are the same as in the non-traumatic, and surgical exposure has disclosed the communication. The symptoms develop because the traumatic subarachnoid block exaggerates the causative intracranial fluid pulse waves by eliminating the dampling effect of the yielding dural sac below. A non-traumatic spinal block also may result in syringomyelia.

Cerebrospinal Fluid↗
PubMed1998

Features of spontaneous improvement in syringomyelia with low-situated cerebellar tonsils.

We analysed six patients who showed spontaneous improvement among the 19 nonsurgically treated cases of syringomyelia with low-situated cerebellar tonsils who visited our outpatient clinic. MR images both before and after improvement were available for two of these patients; CT-myelography before improvement and MR images after improvement were used for two patients; and in the remaining two patients, MR images after improvement only were used. Consequently, we were able to abstract several features from detailed and precise neurological and radiological evaluations of these patients. These features consisted of mild (rather than severe) disability, as well as four radiological features--a rounded shape of the lower edge of the cerebellar tonsils, a low tightness of the foramen magnum, an elevation of the tonsils during the course of the disease. Results of our radiological analyses were consistent with the theories proposed for the pathogenesis of syringomyelia in the light of the CSF flow around the foramen magnum. We propose that the features we have isolated are useful in determining treatment policies and prognoses of patients with syringomyelia accompanied by low-situated cerebellar tonsils.

Adult↗
PubMed1998

Features of spontaneous improvement in syringomyelia with low-situated cerebellar tonsils.

We analysed four patients who showed spontaneous improvement among the 19 nonsurgically treated cases of syringomyelia with low-situated cerebellar tonsils who visited our outpatient clinic. MR images both before and after improvement were available for two of these patients; CT-myelography before improvement and MR images after improvement were used for two patients. Consequently, we were able to abstract several features from detailed and precise neurological and radiological evaluations of these patients. These features consisted of mild (rather than severe) disability, as well as three radiological features--a rounded shape of the lower edge of the cerebellar tonsils, a low tightness of the foramen magnum, an elevation of the tonsils during the course of the disease. Results of our radiological analyses were consistent with the theories proposed for the pathogenesis of syringomyelia in the light of the CSF flow around the foramen magnum. We propose that the features we have isolated are useful in determining treatment policies and prognoses of patients with syringomyelia, accompanied by low-situated cerebellar tonsils.

Adult↗
PubMed1999

The "presyrinx" state: a reversible myelopathic condition that may precede syringomyelia.

BACKGROUND AND PURPOSE: Alteration of CSF flow has been proposed to be an important mechanism leading to the development of syringomyelia. We hypothesize that a "presyrinx" condition attributable to a potentially reversible alteration in normal CSF flow exists and that its appearance may be caused by variations in the competence of the central canal of the spinal cord. METHODS: Five patients with clinical evidence of myelopathy, no history of spinal cord trauma, enlargement of the cervical spinal cord with T1 and T2 prolongation but no cavitation, evidence of altered or obstructed CSF flow, and no evidence of intramedullary tumor or a spinal vascular event underwent MR imaging before and after intervention that alleviated obstruction to CSF flow. RESULTS: Preoperatively, all patients had enlarged spinal cords and parenchymal T1 and T2 prolongation without cavitation. Results of MR examinations after intervention showed resolution of cord enlargement and normalization or improvement of cord signal abnormalities. In one patient with severe arachnoid adhesions who initially improved after decompression, late evolution into syringomyelia occurred in association with continued CSF obstruction. CONCLUSION: Nontraumatic obstruction of the CSF pathways in the spine may result in spinal cord parenchymal T2 prolongation that is reversible after restoration of patency of CSF pathways. We refer to this MR appearance as the "presyrinx" state and stress the importance of timely intervention to limit progression to syringomyelia.

Adult↗
PubMed2005

Resolution of tonsillar herniation and cervical syringomyelia following resection of a large petrous meningioma: case report and review of literature.

We describe a case of a large, petrous meningioma associated with tonsillar herniation and cervical syringomyelia. The patient, a 53-year-old woman, had a 6-month history of a dull, aching pain in the occipital region associated with numbness in the right C2 dermatome and left gaze evoked nystagmus. Magnetic resonance imaging (MRI) revealed a large tumor in the right posterior fossa associated with moderate supratentorial hydrocephalus. Secondary tonsillar herniation and cervical syringomyelia extending from C2 to C6 were also identified. The tumor, later confirmed to be a meningioma originating from the petrous region, was resected completely via a retrosigmoid approach. Postoperative MRI demonstrated total resolution of the tonsillar herniation and cervical syringomyelia. The radiological features, potential pathophysiological mechanisms, and treatment strategies are discussed in relation to the recent literature.

Case Reports↗
PubMedDate not supplied

CSF flow measurement in syringomyelia.

BACKGROUND AND PURPOSE: CSF circulation has been reported to represent a major factor in the pathophysiology of syringomyelia. Our purpose was to determine the CSF flow patterns in spinal cord cysts and in the subararachnoid space in patients with syringomyelia associated with Chiari I malformation and to evaluate the modifications of the flow resulting from surgery. METHODS: Eighteen patients with syringomyelia were examined with a 3D Fourier encoding velocity imaging technique. A prospectively gated 2D axial sequence with velocity encoding in the craniocaudal direction in the cervical region was set at a velocity of +/- 10 cm/s. Velocity measurements were performed in the larger portion of the cysts and, at the same cervical level, in the pericystic subarachnoid spaces. All patients underwent a surgical procedure involving dural opening followed by duroplasty. Pre- and postoperative velocity measurements of all patients were taken, with a mean follow-up of 10.2 months. We compared the velocity measurements with the morphology of the cysts and with the clinical data. Spinal subarachnoid spaces of 19 healthy subjects were also studied using the same technique. RESULTS: A pulsatile flow was observed in syrinx cavities and in the pericystic subarachnoid spaces (PCSS). Preoperative maximum systolic cyst velocities were higher than were diastolic velocities. A systolic velocity peak was well defined in all cases, first in the cyst and then in the PCSS. Higher systolic and diastolic cyst velocities are observed in large cysts and in patients with a poor clinical status. After surgery, a decrease in cyst volume (evaluated on the basis of the extension of the cyst and the compression of the PCSS) was observed in 13 patients. In the postoperative course, we noticed a decrease of systolic and diastolic cyst velocities and a parallel increase of systolic PCSS velocities. Diastolic cyst velocities correlated with the preoperative clinical status of the patients and, after surgery, in patients with a satisfactory foraminal enlargement evaluated on the basis of the visibility of the cisterna magna. CONCLUSION: CSF flow measurement constitutes a direct evaluation for the follow-up of patients with syringomyelic cysts. Diastolic and systolic cyst velocities can assist in the evaluation of the efficacy of surgery.

Adult↗
PubMed1999

Syringomyelia and complex regional pain syndrome as complications of multiple sclerosis.

OBJECTIVE: To describe a patient from Southeast Asia with the optic-spinal phenotype of multiple sclerosis who developed syringomyelia and resultant complex regional pain syndrome (formerly named reflex sympathetic dystrophy). DESIGN: Case report. SETTING: Department of neurology at a tertiary care hospital in the Republic of Singapore. PATIENT: A 53-year-old Chinese woman with a history of optic neuritis developed an episode of left hemiparesis leading to a diagnosis of multiple sclerosis. Serial neuroimaging studies revealed an active demyelinating plaque in the cervical area that later progressed into a syrinx. Over a period of 1 year she also developed signs of sympathetic dysfunction including Horner syndrome of the left eye and complex regional pain syndrome in the left hand. CONCLUSIONS: A case of the optic-spinal phenotype of multiple sclerosis that is commonly observed in Southeast Asia is described. This characteristically tissue-destructive form of multiple sclerosis resulted in syringomyelia complicated by a complex regional pain syndrome. Possible pathogenic mechanisms for these associations are discussed.

Cervical Vertebrae↗
PubMed2000

Periodic limb movements in syringomyelia and syringobulbia.

Twenty-six patients with syringomyelia were studied with polysomnography to determine the frequency of periodic limb movements (PLM) and its relationship to the presence of a Chiari anomaly, the severity of corticospinal tract involvement, and localization of the syrinx. Sixteen patients showed PLM in stages I and II of non-REM sleep and three PLM also while awake. There were no statistically significant differences in overall disability, corticospinal signs, presence of an associated Chiari anomaly, and disease duration between patients with and without PLM, although there was a trend for patients with PLM to have more severe disease. There was preservation of the lumbosacral enlargement of the spinal cord by the syrinx in all patients with PLM. The latency delay between lower and upper limb muscles was suggestive of conduction along propriospinal pathways. Syringomyelia may lead to an abnormal state of spinal hyperexcitability favoring the appearance of PLM. Detailed magnetic resonance image studies of patients with different localizations of the syrinx cavities may help to determine which tracts are involved in the production of PLM.

Adolescent↗
PubMed1992

SEPs and CNS magnetic stimulation in syringomyelia.

Somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) to transcranial and spinal stimulation from upper and lower limb muscles were elicited in 13 patients with syringomyelia. Seven had an associated Chiari type I anomaly. Diagnosis was confirmed by MRI. In 5 cases, SEPs and MEPs were performed before and after surgical treatment. Prolonged central motor conduction times or absent motor responses in upper or lower limbs were found in most patients. The greatest number of abnormalities was disclosed by measurement of CMCT followed by SEPs after tibial nerve stimulation. Two of 5 cases undergoing surgery improved clinically and showed reduction in CMCT after surgical treatment. Our study shows that MEPs were useful in the evaluation of neurophysiological status in syringomyelia patients, helping to estimate anterolateral spinal cord function.

Adult↗
PubMed1993

Transcranial cortical stimulation in syringomyelia: correlation with disability?

Central motor conduction times were studied using transcranial magnetic stimulation in 17 patients with syringomyelia. Central motor conduction time (lower-limb pathway) was prolonged or responses were absent in 44% of stimulations. When results were compared with clinical findings and magnetic resonance tomography, only a weak correlation was found. Transcranial magnetic stimulation is thus of limited value for diagnosing and monitoring the course of syringomyelia.

Action Potentials↗
PubMed1992

Neuroendoscopic technique for the operative treatment of septated syringomyelia.

The management of septated, chambered syringomyelia has until now been problematic because the usual operative methods cannot secure drainage of all chambers of the cavity. The development of a flexible neuroendoscope affords the possibility to perforate the septa under visual control which subdivide the cavity. We present our experiences with 11 cases of septated syringomyelia, which have been operated upon using a flexible neuroendoscope.

Endoscopes↗
PubMed1988

Pathological findings in suboccipital decompression in 63 patients with syringomyelia.

Out of 97 patients with syringomyelia treated surgically, in 63 of them suboccipital decompression was performed. Various kinds of congenital or early acquired abnormalities were found to be the possible cause of the disease. Simple Arnold-Chiari malformation was found in 29 patients. In next 16 cases the Arnold-Chiari malformation was accompanied by partial occlusion of the foramen of Magendie with tiny, translucid membrane, that covered lower 1/2 or 3/4 of the foramen. In another 9 cases of Arnold-Chiari malformation, foramen of Magendie was totally occluded with thick, grayish membrane. In 7 cases lowered cerebellar tonsils were firmly attached to the medulla and joined by adhesions between themselves. In 1 case total atresia of foramen of Magendie was present only. In 1 case no pathological changes were found. The above mentioned findings point out, that in practically all the cases of syringomyelia various kinds of gross pathology, that leads to difficulties in normal outflow of the CSF from the IVth ventricle to cisterns can be found. From that point of view the suboccipital decompression seems to be the most reasonable surgical procedure.

Cerebral Ventricles↗
PubMed1988

Late results of the surgical treatment of syringomyelia.

97 patients with syringomyelia were treated surgically during the last 12 years. Only those patients in which the disease showed rapid progression in the last 1/2-1 year were operated. Of these 97 patients 54 were followed for 5 years or longer. 28 patients were treated by suboccipital decompression, 17 by terminal ventriculostomy, 4 by myelotomy and syringo-subarachnoid shunt; 5 had a combination of two procedures. Very good results were obtained in 12 patients (22%), satisfactory-in 31 (58%) and poor results-in 11 (20%). Very good results were more often achieved after suboccipital decompression, but each method had as well excellent as unsatisfactory results, and the number of the patients is too small as to allow statistically significant conclusions. It is almost impossible to predict the result of the surgical treatment in syringomyelia, but good results were obtained most often in young patients with relatively short duration of the disease. However, it is not the rule. Satisfactory results can even be obtained in older patients with long lasting disease and poor neurological status. The best method of surgical treatment is still under discussion.

Aging↗
PubMed1992

Traumatic cervical syringomyelia related to birth injury.

A rare case of cervical syringomyelia related to breech delivery is reported. The initial diagnosis was bilateral brachial plexus palsy due to birth injury, which was revealed by magnetic resonance imaging (MRI) to be traumatic syringomyelia. The usefulness of MRI in the early diagnosis of cervical cord birth injury, especially in differentiating between brachial plexus palsy due to birth injury and spinal cord trauma due to birth injury in infancy, is emphasized.

Birth Injuries↗
PubMed1975

[Syringomyelia and normal pressure hydrocephalus (author's transl)].

A case of syringomyelia with coexisting hydrocephalus is reported and a pathogenic relationship between both conditions is discussed. Gardner proposed that anomalies at the exit of the fourth ventricle produce a communicating syringomyelia. Hakim and Adams maintain that normal pressure hydrocephalus can aggravate this condition by a hydraulic pressure effect which can be stopped by the insertion of a ventriculoatrial shunt with improvement in the clinical picture. This has been our experience in the case reported.

Adult↗
PubMed1990

Syringomyelia and syringobulbia following tuberculous meningitis.

Tuberculous meningitis may rarely be followed by the development of a syrinx even after apparently successful chemotherapy. There are only six previously reported cases of this unusual complication; these are reviewed. A case is described of syringomyelia which developed 1 year after TBM. One year later magnetic resonance imaging demonstrated not only the spontaneous resolution of the syringomyelia but also the appearance of a new cavity within the brain stem. This surprising sequence of events illustrates the need for extensive studies of the natural history of syrinxes of differing aetiologies.

Adult↗
PubMed1994

Increased pulsatile movement of the hindbrain in syringomyelia associated with the Chiari malformation: cine-MRI with presaturation bolus tracking.

Cine-MRI with presaturation bolus tracking was used in patients with syringomyelia associated with a Chiari malformation to study pulsatile movement of the hindbrain, cervical spinal cord, cerebrospinal fluid and the fluid within the syrinx. Nine patients had 13 examinations, 6 preoperative, 3 after syringosubarachnoid shunting and 4 after posterior fossa decompression. Five controls were also examined. Dynamic display of the acquired images demonstrated downward displacement of the presaturation bolus on the cerebellar tonsils and medulla oblongata (or upper cervical cord) at the C1 level in all preoperative examinations and in two patients after syringosubarachnoid shunting but with residual foramen magnum obstruction. Downward displacement of the bolus on the cervical spinal cord was also demonstrated in 7 examinations, but not observed in the controls. Thus, the hind-brain-spinal cord axis showed larger pulsatile movements in patients with foramen magnum obstruction. Based on these observations and a review of the literature, a new theory on the mode of extension of syringomyelia, emphasising the role of increased pulsatile movement of the hind-brain-spinal cord axis is proposed: that the pulsatile movements, together with a one-way valve mechanism in the syrinx cavity act as a "vacuum-pump" to enlarge the syrinx.

Adolescent↗
PubMed1993

Lipomyeloschisis associated with thoracic syringomyelia and Chiari I malformation.

Co-existence of Chiari I malformation and myelomeningocele is uncommon. Syringomyelia, when associated with a Chiari I malformation, classically involves the cervical spinal cord. Intramedullary extension of lipoma is unusual in lipomyeloschisis. A patient with lumbar lipomyelomeningocele with tethered cord, lower thoracic syringomyelia and Chiari I malformation, shown by MRI is reported.

Arnold-Chiari Malformation↗