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The two types of upper limb somatosensory evoked potential abnormality observed in nine patients with syringomyelia were reduced amplitude or absent cervical potentials and an abnormal central conduction time. Although this pattern of abnormalities resembles that observed in other intrinsic spinal cord lesions, it differs from peripheral nerve diseases and cervical radiculopathy in which the central conduction time is normal.
A case of syringomyelia involving the entire spinal cord secondary to a spinal intramedullary tumour is described. Cerebrospinal fluid cytology and microscopic evaluation of gross necropsy specimens revealed a primary large cell lymphoma of the central nervous system. In addition there was massive leptomenigeal lymphomatosis involving the cortex, brainstem, and cerebellum. No solitary intracranial mass lesion was found.
Clinical, respiratory, and polysomnographic findings in three patients with syringomyelia and syringobulbia who developed severe respiratory complications are described. Neurological examination showed evidence of IXth and Xth cranial nerve involvement with dysphagia and dysphonia, but there were no complaints of serious sleep difficulties. Two patients died during sleep and the other was resuscitated during a nap. All patients showed moderate restrictive ventilatory defects with reduced maximal buccal pressures and one also showed a low ventilatory response to CO2 rebreathing. Protracted central, obstructive, and mixed apnoeas and hypopnoeas were commonly observed during sleep. There were no changes in heart rate during these events. A combination of respiratory and cardiovascular mechanisms might have been responsible for the severe complications described.
A case of post-traumatic syringomyelia developing two years after spinal cord injury is presented. The patient was treated with the placement of a thecoperitoneal shunt incorporating a low pressure valve. Response was excellent with restoration of neurological function and almost complete collapse of the cavity at one year follow up. The rationale of this form of treatment is discussed in the light of recent evidence concerning the pathogenesis of the condition.
OBJECTIVES: To assess the incidence of post-traumatic syringomyelia (PTS), to correlate the presence of PTS with its most common signs and symptoms, and to compare results from the Swiss Paraplegic Centre with those reported in the medical literature. METHODS: A total of 449 recent traumatic paraplegic and tetraplegic patients admitted to the Swiss Paraplegic Centre in Zurich between 1 January 1987 and 31 December 1993 were prospectively analysed. Yearly clinical tests with conventional radiographs and additional T1 and T2 weighted images were performed as soon as PTS was diagnosed. RESULTS: Of these 449 patients 20 patients displayed symptoms of PTS (4.45%). Ten non-operated patients remained clinically stable (average time: 37 months). Ten worsened--three refused operation, seven were operated on. Mean worsening time was 97 months. Deterioration was closely related to the enlargement of the cyst whereas in operated patients neurological improvement or stabilisation correlated with collapse of the cyst. CONCLUSIONS: Delay between appearance of the first symptoms of PTS and deterioration making surgery necessary may be long (mean five years in the seven operated patients) underlining the need for regular tests. "Slosh" and "suck" mechanisms could explain cyst enlargement as surgical realignment of the spine resulted in a complete cyst collapse in two of the operated patients (normalisation of CSF flow? ). Cord compression, tense syrinx at the fracture site, and kyphosis seemed to be closely linked to the enlargement of the cyst with subsequent further neurological deterioration.
A neurogenic bladder is seldom described as the first manifestation of syringomyelia. A patient is reported with an extensive syrinx along the entire spinal cord and a Chiari type I malformation, who experienced dysaesthesia and weakness during shooting practice and presented with urinary retention.
OBJECTIVES: Despite many hypotheses, the pathophysiology of syringomyelia is still not well understood. In this report, the authors propose a hypothesis based on analysis of cerebrospinal fluid dynamics in the spine. METHODS: An electric circuit model of the CSF dynamics of the spine was constructed based on a technique of computational fluid mechanics. With this model, the authors calculated how a pulsatile CSF wave coming from the cranial side is propagated along the spinal cord. RESULTS: Reducing the temporary fluid storage capacity of the cisterna magna dramatically increased the pressure wave propagated along the central canal. The peak of this pressure wave resided in the mid-portion of the spinal cord. CONCLUSIONS: The following hypotheses are proposed. The cisterna magna functions as a shock absorber against the pulsatile CSF waves coming from the cranial side. The loss of shock absorbing capacity of the cisterna magna and subsequent increase of central canal wall pressure leads to syrinx formation in patients with Chiari I malformation.
Overt psychological, neurological and haematological abnormalities were present in a 35 year old Jamaican female diagnosed as having idiopathic periodic hypothermia. Although symptoms and signs were ameliorated by warming, no long term satisfactory therapeutic measure was identified. Despite extensive clinical and subsequently histological investigation no cause for the hypothermia was found although she had clinically unsuspected cervical syringomyelia.
We present a case of obstructive sleep apnoea in association with syringomyelia. We describe the successful treatment of the respiratory obstruction by continuous positive airway pressure and then by surgical means. This rare combination of conditions and the management is reviewed.
Magnetic resonance (MR) imaging and computed tomography (CT) were used to study severe syringomyelia, in which the syrinx was so eccentric relative to the center of the spinal cord that at initial examination it appeared to be an exophytic extramedullary mass, in five of six patients with type 2 Arnold-Chiari malformation and one patient with an intramedullary tumor. Sagittal and axial images were routinely obtained at 1.5 T; CT was performed after intrathecal injection of contrast material. On sagittal MR images, the exophytic component of the syrinx typically displaced the spinal cord peripherally and mimicked an extramedullary intradural lesion such as an arachnoid cyst or meningioma. On axial MR images, the gradual appearance and disappearance of this component could be traced as it compressed the spinal cord, which had a signet-ring appearance at the equator of the syrinx. It is concluded that both MR imaging and postmyelographic CT can reveal the characteristics of this lesion on axial images, but MR imaging is superior because it enables direct sagittal imaging.
Spinal cord function was monitored during surgery in 2 cases of syringomyelia. The intraoperative recording of spinal segmental and conduction potentials evoked at cervical levels by the stimulation of median and tibial nerves were essential in the definition of the extent of the lesion and was consistent with postoperative neurological outcome.
The case of a 30-year-old man with posttraumatic syringomyelia involving the cervical and upper thoracic spine is reported. The patient was followed clinically and with magnetic resonance (MR) imaging over a 3-year period. Spontaneous complete remission of the syrinx-related symptoms correlated with a partial collapse of the syrinx, as proved by serial MR imaging.
The dimensions of the syrinx, the remaining spinal cord and the degree of cerebellar herniation were analyzed by one- and two-dimensional MRI studies in 22 patients with syringomyelia. A deep and/or broad cyst tends to be a long one. The dimensions of the syrinx and the spinal cord have an inverse relationship. The degree of cerebellar herniation is not related to the longitudinal or transverse extension of the cyst or the ratio cyst/cord or the remaining spinal cord (neither for diameters nor for cross-sectional areas). The dimensions of cysts are not significantly greater in patients with tonsillar herniation than in patients without. We conclude that cerebellar herniation is not a major prognostic factor and should not be overestimated for the development and progression of the disease.
We describe a 30-year-old woman with syringomyelia, who developed recurrent painless whitlows of the left hand that led to amputation of the terminal phalanx of the 4th finger and considerable shortening of the terminal phalanges of the 1st, 2nd, 3rd and 5th fingers. A roentgenogram of the left hand showed a complete resorption of the terminal phalanges of the 3rd, 4th and 5th fingers and partial resorption of the terminal phalanx of the 2nd finger. Although the defective sensory function undoubtedly contributes to the development of bone resorption by facilitating mechanical repetitive injuries and infections, impaired vasomotor function possibly plays an equally important role.
Under general anaesthesia and muscle relaxation, a thoracic epidural catheter was inserted at the T8-T9 level in a 7-year-old boy scheduled to have a Nissen fundoplication to provide postoperative analgesia. After 4 ml of lignocaine 1.5% was injected through the catheter, hypotension resulted. Fifty-five minutes later 5 ml of bupivacaine 0.25% produced the same effect. In the recovery room a similar injection resulted in lower blood pressure and temporary apnoea. Sensory and motor deficits were noted the next day and four days later magnetic resonance imaging demonstrated spinal cord syringomyelia extending from T5 to T10. Four years later, dysaesthesia from T6 to T10 weakness of the left lower extremity and bladder and bowel dysfunction persist. The risks of inserting thoracic epidural catheters in patients under general anaesthesia and muscle relaxation are discussed, emphasising the possibility of spinal cord injury with disastrous consequences.
Intraoperative electrophysiologic monitoring can diminish the risk of neurologic injury by enabling the detection of injury at a time when it can be reversed or minimized. This report describes a 14-year-old girl with a Chiari's malformation type I and syringomyelia who underwent a suboccipital decompression and dural patch grafting with concurrent somatosensory evoked potentials. When the patient was turned into the prone position and the neck was flexed, the left-sided somatosensory evoked potential deteriorated. After the patient's neck was repositioned, the left median nerve potential improved but did not return to baseline. Postoperatively, the patient had decreased proprioception of her left arm, which completely resolved at 2-week follow-up. This single case report does not establish the need for routine somatosensory evoked potential monitoring. Nevertheless, deterioration of the potential in this case led directly to a change in the surgical positioning, which may have significantly reduced the chances of a permanent neurologic injury.
Syringomyelia was first recognized as a disease process some 400 years ago. The process of cystic dilation of the spinal cord is unpredictable and may result in a delay of many decades before the symptoms and signs of neurologic and orthopedic changes become apparent and commensurate with the observed cystic changes within the spinal cord. The syringomyelic process is usually associated with trauma, tumor, or congenital abnormalities at the craniocervical junction or along the spinal neuraxis. Several theories have been proposed as to processes involved in the development of spinal cyst formation, although none are completely compatible with the observed clinical pathology of syrinx development. Magnetic resonance imaging has markedly improved our ability to study the anatomy and natural history of syrinx formation, but to date, our understanding of the process remains imprecise. In view of the limited understanding of the pathophysiology of this disease process, it is not surprising that a variety of treatment regimens have been proposed and their efficacy remains difficult to fully evaluate.
An occipitoatlantoaxial malformation and ventricular septal defect (VSD) were diagnosed in a 36-hour-old female camel. Physical examination revealed a firm protrusion of the dorsal aspect of the atlas and axis, tilting of the head to the left, and a grade V/VI systolic murmur. Neurological examination revealed proprioceptive deficits and ataxia of all 4 limbs. Radiographic examination and necropsy demonstrated malformation, fusion of the atlas to the occiput and hypoplasia of the dens of the axis, and subluxation of the atlantoaxial joint. Dorsoventral laxity of the atlantoaxial joint was also present, with compression of the cervical spinal cord. A 1.5-cm-diameter VSD was observed also. Histopathologic examination of the cervical spinal cord revealed a cavity extending from the level of the first to fourth cervical segment, dorsal to the central canal, 5 cm long and 1-2 mm in diameter. The cells around the cavity were positive for glial fibrillary acidic protein and sporadically positive for vimentin. This cavitary structure was consistent with syringomyelia, which was lined by glial cells, surrounded by edematous white matter with Wallerian-like degeneration and with neuronal necrosis in the adjacent dorsal horns.