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PubMed2003

The role of excitotoxic injury in post-traumatic syringomyelia.

Fifty percent of patients with neurological deterioration from post-traumatic syringomyelia do not respond to treatment. Treatment failure is due in part to an incomplete understanding of the underlying aetiology. An animal model that mimics the human disease is required to investigate underlying pathophysiology and treatment options. A previous study was designed to mimic trauma-induced effects on the spinal cord that result in syringomyelia, combining an excitotoxic insult with kaolin-induced arachnoiditis. In this excitotoxic model, syringes were produced in 82% of animals. The aims of the current study were to improve the model to produce syringes in all animals treated, to examine the relative influences of excitotoxic injury and neuronal loss on syrinx formation, and to use magnetic resonance imaging (MRI) to examine syringes non-invasively. A temporal and dose profile of intraparenchymal quisqualic acid (QA) and subarachnoid kaolin was performed in Sprague Dawley rats. MRI was used to study four syrinx and six control animals. In one subgroup of animals surviving for 6 weeks, 100% (eight of eight) developed syringes. Syrinx formation and enlargement occurred in a dose and time dependent manner, whilst significant neuronal loss was only dose dependent. Animal syrinx histology closely resembled human post-traumatic syringomyelia. Axial T2-weighted MR images demonstrated syrinx presence. The results suggest that the formation of an initial cyst predisposes to syrinx formation in the presence of subarachnoid adhesions.

Animals↗
PubMed1982

Cardiovascular reflexes in syringomyelia.

Cardiovascular reflexes were assessed by simple noninvasive tests in 18 patients with syringomyelia restricted to the spinal cord (syringomyelia) and 22 patients with syringomyelia and brainstem signs (syringobulbia), and the results compared with a control group. Postural hypotension occurred more frequently in patients with bilateral brainstem signs, palatal weakness or vocal cord palsy. Vagal cardiovascular reflexes were disturbed in patients with clinical evidence of involvement of the Xth cranial nerve, and also in those with other brainstem signs. The integrity of the efferent limb of the reflex arc was demonstrated in three patients by testing the heart rate response to intravenous atropine. It was observed that cardiovascular reflexes may be impaired in patients free of symptoms of autonomic dysfunction. These findings may be relevant to the occurrence of sudden death in such patients and to the possible anatomical localization of the defect.

Adolescent↗
PubMed1991

The contribution of median nerve SEPs in the functional assessment of the cervical spinal cord in syringomyelia. A study of 24 patients.

Since the early study of Halliday and Wakefield (1963) it has generally been agreed that scalp somatosensory evoked potentials (SEPs) are normal in patients with dissociated loss of pain and temperature sensation. Up to now a few patients with abnormal spinal N13 and preserved scalp P14 and N20 have been reported in the literature, but there is no firm evidence, based on group data, that this dissociation can be related to any form of dissociated sensory loss. We studied median nerve SEPs in 24 patients with syringomyelia documented by CT scan or MRI. For the recording of the cervical N13 we used a Cv6 anterior cervical montage, which cancels the potentials generated above the foramen magnum and enhances the amplitude of N13. Scalp far-field and early cortical SEPs were recorded using a noncephalic reference electrode. Since N13 pathological changes are mainly changes in amplitude we measured the N13/P9 amplitude ratio in normal subjects and patients and found that it was a reliable index to quantify the amplitude decrease of N13. Absent or reduced N13 was observed in 40 median nerve SEPs (83%) in conjunction with normal P14 and N20 in 30 SEPs. Thus the dissociated loss of the cervical N13 was identified as the most conspicuous SEP feature in syringomyelia. A significant correlation was found between abnormal N13 and loss of pain and temperature sensations, whereas P14 abnormalities correlated well only with loss of joint and touch sensations. In the median nerve territory, sensation was either normal (6 cases) or lost only for pain and temperature (24 cases) when SEPs showed abnormal N13 and normal P14. Although it does not directly reflect the postsynaptic activity of spinal cells receiving their inputs from A delta and C fibres the N13 potential proved to be a reliable index of spinal cord grey matter dysfunction in syringomyelia.

Adult↗
PubMed2006

Mechanisms of central neuropathic pain: a combined psychophysical and fMRI study in syringomyelia.

The pathophysiology of central pain syndromes is still poorly understood and their treatment remains a major challenge. It has long been suggested that lesions of the spinothalamic pathways are necessary for developing these pain syndromes. The recently proposed thermosensory disinhibition theory suggests that reduction of the inhibition of thermal sensory afferents that affect nociceptive systems may play a major pathophysiological role. Syringomyelia, which is frequently associated with central neuropathic pain, is characterized by a selective or preferential lesion of the spinothalamic tract resulting in thermosensory deficits of various extents and magnitudes. Thus, syringomyelia represents a unique 'pathological model' particularly suited to investigating the relationship between spinothalamic tract dysfunction, thermosensory deficits and pain. Here, we systematically compared the sensory loss (thermal and mechanical), using quantitative sensory testing, between 46 consecutive syringomyelia patients with or without neuropathic pain. We then further investigated the mechanisms of evoked pains in these patients, using functional MRI (fMRI) in a subgroup of patients with cold or brush-evoked allodynia, compared with patients without pain and healthy volunteers. We found no significant difference in the magnitude or extent of sensory deficits between patients with or without neuropathic pain, suggesting that lesions of the spinothalamic pathways are not sufficient for developing central pain. However, a different pattern of sensory deficits was observed between patients with spontaneous pain only (n = 11) and patients with both spontaneous pain and allodynia (n = 20), suggesting that the mechanisms of central pain are not univocal. In patients with spontaneous pain only, the thermal sensory loss was significantly more asymmetrical and there was a direct relationship between the extent of thermosensory deficits (i.e. deafferentation) and the intensity of burning pain. In contrast, patients with allodynia had reduced thermal deficits, in terms of both magnitude and extent. In addition, the sensory deficits were different between patients with cold or tactile allodynia, suggesting distinct pathophysiological mechanisms related to the sub-modalities of allodynia. Our fMRI study further confirmed this, showing that different sub-types of allodynia were associated with distinct patterns of brain activity, which do not necessarily correspond to the 'pain matrix' involved in acute physiological pain. The prefrontal cortex was the only area consistently activated by pathological evoked pains, suggesting that alteration of high-level pain modulatory mechanisms might play a major role in allodynia due to central lesion.

Adult↗
PubMed1991

Syringomyelia as a postoperative sequela of the resection of a chordoma of the clivus: case report.

Syringomyelia has been described frequently in association with various abnormalities of the skull base but rarely with tumors of the skull base. Syringomyelia as a postoperative sequela of surgery of the skull base has not been reported. The authors describe a case of cervical syringomyelia developing after the partial resection of a chordoma and combined conventional and proton beam irradiation. The possible mechanisms causing this abnormality are discussed.

Adult↗
PubMed1999

Terminal syringomyelia in association with the tethered cord syndrome.

OBJECTIVE: With the increasing use of magnetic resonance imaging, terminal syringomyelia (segmental cystic dilation of the caudal one-third of the spinal cord) in association with the tethered cord syndrome has become an appreciable finding. This study attempted to define the clinical significance of this associated pathological condition by describing its clinical and radiological characteristics and its contribution to the clinical status of patients with tethered spinal cords. METHODS: Of 132 consecutive patients with tethered cord syndrome who presented to our department between 1990 and 1997, 32 patients with terminal syringomyelia were enrolled in this study. Clinical findings were correlated with syrinx morphological features, as defined using magnetic resonance imaging. Surgical treatment used two basic approaches, i.e., simple untethering or untethering with concurrent syrinx drainage. RESULTS: Analysis of the neurological deficits established a contribution of segmental symptoms, which were correlated with the extension and dilation of the syrinx cavity. Magnetic resonance imaging scans revealed the frequency of sacral tethering (40.6%), the intramedullary paracentral position of the syrinx (75%), and disturbances in regional cerebrospinal fluid flow (42%). The clinical outcomes seemed to be correlated with syrinx shrinkage; all patients who experienced collapse of the cavity achieved better symptom resolution. CONCLUSION: Radiologically significant terminal syringomyelia affects the clinical presentation of tethered cord syndrome, by increasing or inducing neurological deficits. Better clinical outcomes after syrinx decompression emphasize the importance of the recognition and treatment of this pathological condition.

Adolescent↗
PubMed2001

Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION: A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE: Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION: We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.

Adult↗
PubMed2001

Incidentally identified syringomyelia associated with Chiari I malformations: is early interventional surgery necessary?

OBJECTIVE: The purpose of this study was to analyze clinical data and magnetic resonance imaging (MRI) findings for patients with asymptomatic, incidentally identified syringomyelia associated with Chiari I malformations who were monitored for more than 10 years, and to clarify the natural history of these lesions. METHODS: The clinical records of nine patients who had not been surgically treated and were regularly subjected to neurological and MRI examinations were analyzed. In MRI studies, the axial diameter of the syrinx at the widest level, the longitudinal extent of the syrinx, and the extent of tonsillar herniation into the spinal canal were analyzed. As a control, MRI findings for 11 patients with symptomatic syringomyelia associated with Chiari I malformations who had been surgically treated were also analyzed, and these MRI parameters were statistically compared between the asymptomatic and symptomatic groups. RESULTS: One patient underwent surgery, because of neurological changes, 7 years after the first visit. None of the remaining patients demonstrated any neurological change during the follow-up period (11.2+/-0.7 yr), and all of them have been faring well without surgery. No statistically significant differences in MRI findings between the asymptomatic and symptomatic groups were observed. CONCLUSION: The long-term clinical courses of patients with asymptomatic, incidentally identified syringomyelia associated with Chiari I malformations were observed to be benign. MRI parameters did not provide predictable values to recommend interventional surgery. Unless changes in neurological or MRI findings are detected, early interventional surgery is not necessary.

Adolescent↗
PubMed2002

Dandy-Walker complex and syringomyelia in an adult: case report and discussion.

OBJECTIVE AND IMPORTANCE: Syringomyelia in association with the Dandy-Walker complex is rare and only 17 cases have been reported, mostly as autopsy findings or incidental reports in series for other conditions. We present a further case report and discuss the possible pathogenetic mechanisms of syrinx formation in the presence of the Dandy-Walker complex. We emphasize the role of foramen magnum obstruction and alteration in cerebrospinal fluid flow dynamics in syrinx formation. The therapeutic importance of disimpaction of the foramen magnum is stressed. CLINICAL PRESENTATION: A 39-year-old man presented with headache. As a child, he had had cystoperitoneal and ventriculoperitoneal shunts inserted for hydrocephalus in association with the Dandy-Walker complex. On examination, the patient had limited upgaze but was otherwise normal. Magnetic resonance imaging revealed a large cervicothoracic syrinx and a Dandy-Walker cyst obstructing the foramen magnum. INTERVENTION: The cystoperitoneal shunt was revised. The patient's headaches resolved, and follow-up imaging demonstrated resolution of the syrinx and disimpaction of the foramen magnum. CONCLUSION: Disimpaction of the foramen magnum can resolve syringomyelia in patients with the Dandy-Walker complex. This can be achieved by a shunt procedure or by formal foramen magnum decompression. The rarity of the association between the Dandy-Walker complex and syringomyelia prevents firm conclusions from being made regarding the best management strategy for this condition.

Adult↗
PubMed1986

Posttraumatic syringomyelia: the British Columbia experience.

Posttraumatic syringomyelia is an uncommon late complication of spinal cord injury. This study identified nine patients with posttraumatic syringomyelia and examined initial presentation, neurologic status, ability to perform functional activities, and results of treatment. Pain and numbness were the most common presenting symptoms. Motor impairment occurred later but was more disabling. Functional abilities depended mainly on the level of the original spinal cord injury. Three patients were managed conservatively and have had no significant progression of their neurologic deficit. Six patients were managed with syringoperitoneal or syringosubarachnoid shunts. Pain improved most consistently after surgery. Motor power improved less and sensation least. Ability to perform activities of daily living did not significantly change after surgery. Posttraumatic syringomyelia remains a difficult therapeutic problem in the spinal cord-injured population.

Adult↗
PubMed1995

Syringomyelia after removal of benign spinal extramedullary neoplasms.

STUDY DESIGN: This is a case report. OBJECTIVES: Two patients with noncommunicating syringomyelia manifesting symptoms years after the uneventful excision of a benign intradural extramedullary neoplasms are presented. SUMMARY OF BACKGROUND DATA: The association of noncommunicating syringomyelia with benign extramedullary neoplasms as a coexistent or delayed process is a rare event. The pathogenesis of such spinal cord cavitation after tumor removal and the relationship with therapeutic intervention is not well defined. METHODS: The delayed occurrence of neurologic impairment emphasizes the need for considering development of syringomyelia rather than tumor recurrence. RESULTS: The effects of chronic spinal cord compression in the thoracic region may be accelerated by adverse stresses acting on the spinal cord associated with tethering of the spinal cord. CONCLUSION: In defining the pathogenesis of this form of spinal cord cavitation, these cases suggest that therapeutic approaches directed at resolving the process should not only include lyses of adhesions, but shunting procedures, which appear to be most efficacious when these procedures are performed at or cephalad to the level of original tumor removal.

Aged↗
PubMed1995

"Conservative" cranio-cervical decompression in the treatment of syringomyelia-Chiari I complex. A prospective study of 20 adult cases.

STUDY DESIGN: This study was designed to investigate prospectively a series of patients with syringomyelia-Chiari I complex operated on by a minimally invasive procedure. OBJECTIVE: The objective was to determine whether a relatively simple and low-risk osteo-dural decompression would obtain as good clinico-radiological results as those observed after the commonly used, more complex posterior fossa surgery. SUMMARY OF BACKGROUND DATA: The majority of hypotheses concerning the pathogenesis of syringomyelia link it to the existence of some derangement of the cerebro-spinal fluid flow at the cranio-spinal level and, consequently, surgical procedures are directed toward decompressing the cranio-cervical junction; however, there is no clear-cut evidence that simple osteo-dural decompression should be associated with manipulation of nervous tissue for the purpose of restoring to normal an altered cerebro-spinal fluid flow dynamic. METHODS: The procedure consisted of a small suboccipital craniectomy and laminectomy of C1 (when necessary C2 as well), followed by dura mater opening. Exploration and plugging of the obex, resection of arachnoid adherences, or amputation of the amygdala(e) were never performed. Every effort was made to keep the arachnoid intact. The series included 20 patients with progressive disease treated according the above-mentioned criteria. RESULTS: Mortality and morbidity were nil and MRI controls showed shrinkage of the syrinx in all cases. At long-term follow-up (average, 2.4 years), 8 patients were considered improved, 11 stabilized, and 1 patient continued to deteriorate despite the collapse of the cord cavity. CONCLUSION: Considering that the results of this "conservative" cranio-cervical decompression are at least comparable to those obtained with other surgical procedures, this procedure, which was not associated with mortality or morbidity, currently should be considered the first option in the treatment of syringomyelia-Chiari I complex.

Adolescent↗
PubMed1998

Management of posttraumatic syringomyelia using an expansile duraplasty. A case report.

STUDY DESIGN: A patient in whom posttraumatic syringomyelia developed 34 years after an L2 fracture is reported. OBJECTIVES: To review the pathophysiology and current management modalities for posttraumatic syringomyelia. The delayed presentation and management rationale of this case are emphasized. SUMMARY OF BACKGROUND DATA: This case represents the most delayed onset of symptoms from a posttraumatic syrinx reported in the literature. Although lysis of arachnoid adhesions and expansile duraplasty to recreate the subarachnoid space have been described for nonshuntable syrinxes, this form of management was used as the primary management modality in this case. METHODS: A posttraumatic syrinx was managed by lysis of the arachnoid adhesions, fenestration of the cyst, and an expansile duraplasty. RESULTS: After surgery, the patient's symptoms improved, and magnetic resonance imaging showed a decrease in the size of the syrinx. CONCLUSION: Posttraumatic syringomyelia represents one of the few surgically remediable presentations of spinal cord injury. Consequently, it is necessary to continuously develop and monitor new management options for this disease. In the case reported here, the syrinx was treated successfully without the implanting a drainage tube.

Child↗
PubMed2002

Intramedullary tuberculoma with syringomyelia.

Intramedullary tuberculoma with syringomyelia is rare. We treated a woman with back pain and weakness of the left leg that had slowly progressed for more than 30 years. Radiologic evaluation demonstrated a crescent-shaped calcification at the level of the C6 vertebra, and syringomyelia from C7 to T9. Laminectomy and syringosubdural shunt placement were performed, and a tuberculoma was removed. Back pain resolved after the operation, and mobility was facilitated. We recommend surgery for intrathecal tuberculoma with syringomyelia even when the course has been prolonged with no active tuberculous lesion.

Cervical Vertebrae↗
PubMed2003

Altered subarachnoid space compliance and fluid flow in an animal model of posttraumatic syringomyelia.

STUDY DESIGN: A histologic study of cerebrospinal fluid tracers in Sprague-Dawley rats undergoing lumboperitoneal shunt insertion in the excitotoxic animal model of posttraumatic syringomyelia (PTS). OBJECTIVES: To determine the effects of cerebrospinal fluid (CSF) diversion from the subarachnoid space on perivascular flow (PVS) and syrinx formation in posttraumatic syringomyelia. SUMMARY OF BACKGROUND DATA: In an animal model of PTS, fluid enters syringes from the subarachnoid space via perivascular spaces. Preferential PVS flow occurs at the level of the syrinx. It has been suggested that arachnoiditis predisposes to posttraumatic syringomyelia formation by obstructing subarachnoid cerebrospinal fluid flow and enhancing perivascular flow. MATERIALS AND METHODS: Thirty-two male Sprague-Dawley rats were investigated using the CSF tracer horseradish peroxidase (HRP), the excitotoxic model of PTS, and lumboperitoneal shunt insertion. Five experimental groups consisted of normal controls, syrinx only and shunt only controls, and shunt insertion before or after syrinx formation. In all groups except normal controls, CSF flow studies were performed 6 weeks after the final intervention. Grading scales were used to quantify HRP staining. RESULTS: All excitotoxic model animals formed syringes. Perivascular flow was greatest at the level of the syrinx. Cerebral cortex perivascular flow was significantly reduced after shunt insertion in animals with a syrinx (P < 0.05). Shunt insertion did not alter syrinx length or size. There were no significant differences between shunt and syrinx first groups. CONCLUSIONS: Increasing caudal subarachnoid space compliance with a shunt does not affect local CSF flow into the spinal cord and syrinx. These results suggest that localized alterations in compliance, as opposed to obstruction from traumatic arachnoiditis, may act as an important factor in syrinx pathogenesis.

Animals↗
PubMed2005

Atlanto-occipital dislocation with traumatic pseudomeningocele formation and post-traumatic syringomyelia.

STUDY DESIGN: A case report of traumatic atlanto-occipital dislocation complicated by the development of anterior and posterolateral pseudomeningoceles and the late development of syringohydromyelia is presented. OBJECTIVE: To describe a unique post-traumatic and postsurgical course following atlanto-occipital dislocation. SUMMARY OF BACKGROUND DATA: Syringomyelia is a significant potential long-term complication in patients recovering from traumatic atlanto-occipital dislocation. Cord enlargement and increased T2 signal can be a marker of abnormal cerebrospinal fluid flow dynamics. This "presyrinx state" can be seen before clinical evidence of neurologic compromise. Pseudomeningocele formation after atlanto-occipital dislocation is rare, with only 3 reported cases. To our knowledge, all reported cases describe retropharyngeal pseudomeningoceles, and posterolateral pseudomeningocele as seen in this case has not previously been described. METHODS: A single case is reported with an emphasis on the imaging findings related to the patient's subsequent neurologic deterioration. RESULTS: Following a pedestrian-motor vehicle collision, the patient received initial evaluation and treatment at a local foreign medical facility, where his cervical spine was cleared. Several days following stabilizing treatment and surgery, the patient was transferred to a foreign-based United States military medical facility and ultimately to our institution, where magnetic resonance imaging demonstrated occipitocervical dissociation. The patient was taken to the operating room for surgical stabilization. Four months after his index operation, the patient underwent halo removal. Follow-up magnetic resonance imaging revealed thickening of the cervical spinal cord in conjunction with diffuse high cord T2 signal and a small low cervical segment of syringomyelia. The patient was observed with follow-up magnetic resonance imaging obtained after 1 month. At this time, the low cervical syrinx had enlarged slightly, a small thoracic syrinx was observed, and cine imaging of cerebrospinal fluid flow demonstrated obstruction at the level of the foramen magnum. The patient was taken to the operating room fordecompression of the foramen magnum and posterior fossa and duraplasty. One month later, the patient's clinical condition began to deteriorate, and repeat imaging showed continued enlargement of the patient's syrinx and hydrocephalus. He was admitted for an urgent shunt procedure but unfortunately sustained cardiorespiratory arrest while on the ward awaiting surgery. CONCLUSIONS: Atlanto-occipital dislocation is rarely survivable, and delayed diagnosis can negatively affect long-term clinical outcome. This case illustrates how, despite early signs of improvement, post-traumatic syringomyelia may occur months or even years after spinal trauma and should always be considered in patients who experience late neurologic deterioration after atlanto-occipital dislocation.

Accidents, Traffic↗
PubMed2000

Syringomyelia: an expanding problem.

Syringomyelia, a central cavitation of the spinal cord, was once thought to be a rare disorder. Associated with congenital malformations as well as trauma and tumors, a syringomyelia can cause a variety of sensory complaints, including parasthesias, changes in temperature recognition, and pain. Other symptoms may range from gastrointestinal problems to shoulder arthropathy. Left unchecked, a syringomyelia can expand and lead to permanent disability. The nurse practitioner should be suspicious of the presence of a syrinx in patients who have otherwise unexplained sensory deficits that follow cervical or thoracic dermatomes.

Adult↗
PubMed2004

Effects of surgery on the sensory deficits of syringomyelia and predictors of outcome: a long term prospective study.

OBJECTIVE: To quantify the effects of surgery on the thermal deficits of syringomyelia and assess the predictors for such effects. METHODS: The subjects were 16 consecutive patients (12 men, 4 women; mean (SD) duration of sensory symptoms, 5.1 (4.5) years) presenting with the typical symptoms of syringomyelia related to Chiari I malformation or trauma, and requiring surgical treatment. They were evaluated before surgery, then at six months and two years. Sensory evaluation included determination of the extent of thermal deficits and quantitative assessment of thermal, mechanical, vibration detection, and pain thresholds. Neuropathic pain intensity was evaluated on visual analogue scales. Magnetic resonance imaging was done before and after surgery to measure syrinx dimensions. RESULTS: The magnitude and extent of thermal deficits improved in a subgroup of patients and this was best predicted by the duration of sensory symptoms: patients operated on less than two years after the onset of their symptoms tended to improve, while those operated on later were stabilised or deteriorated slightly. The effect of surgery on thermal deficits was correlated with the duration of sensory symptoms. Surgery also affected vibration deficits in patients with the Chiari malformation, neuropathic pain on effort, and syrinx dimensions. CONCLUSIONS: The duration of sensory deficits is the best predictive factor of the efficacy of surgery for the thermal symptoms of syringomyelia. Early surgery is required if these deficits are to be minimised.

Adult↗