Cases reported "Syringomyelia"

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1/64. multiple sclerosis and non-communicating syringomyelia: a casual association or linked diseases?

    Non-communicating syringomyelia (NCS) can be associated with different disease processes such as arachnoidytis, trauma or tumor. Approximately 12 cases, documented radiographically, of the association of non-communicating syringomyelia and multiple sclerosis (MS) have been described but their relationship remains obscure. In 3 patients with laboratory supported MS spinal magnetic resonance imaging (MRI) revealed a central cystic cavity. In 2 patients lesions on the spinal cord above the cavity were demonstrated. At 3-year follow-up in 1 patient, no change in the cavity was detected. Although 3 cases are insufficient for providing a definitive conclusion on the relationship between these two diseases, we suggest that demyelinating lesions have to be regarded as possible causes of spinal, asymptomatic cavities.
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2/64. Post-traumatic syringomyelia following complete neurological recovery.

    OBJECTIVE: To describe the later neurologic deterioration secondary to the appearance of a post-traumatic syringomyelic cavity, in a patient who, in the initial phase, had an incomplete spinal cord lesion (asia C), which improved to asia E. methods: A 52-year-old male patient who, at the age of 19 (1965), suffered a spinal cord injury. He presented with a fracture of the sixth and seventh cervical neurological segment at the time of the lesion, evolving to asia E. Nine years after the traumatism, he began to feel pain accompanied by a sensory and motor deficit. RESULTS: With the aid of myelography and MRI, the existence of a syringomyelic cavity was detected, which extended from the fourth to the seventh cervical segments. The patient was operated on, on various occasions, placing a syringo-subarachnoid shunt. The neurological status of the patient continued to deteriorate and, at present, he has a complete lesion below the fourth neurological cervical segment with a partially preserved sensitive area up to T1. CONCLUSION: The development of the syringomyelic cavity could be one of the causes of later neurologic deterioration in patients with traumatic spinal cord injury with neurological recovery 'ad integrum' in the initial phase of spinal cord injury.
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3/64. Cerebral dissection from syringomyelia demonstrated using cine magnetic resonance imaging. Case report.

    A 16-year-old boy presented at the authors' emergency department with a sudden deterioration of respiration. He had been paraparetic for 3 years and had become quadriplegic 2 days previously. Magnetic resonance images revealed a Chiari I malformation and a hydromyelic cavity extending from C-1 to T-11. Rostrally, a small cylindrically shaped lesion extended from the cervicomedullary junction to the left semioval center. The patient made a dramatic neurological recovery following suboccipital craniectomy and upper cervical laminectomies with augmentation duraplasties followed by placement of a syringoperitoneal shunt.
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4/64. multiple sclerosis with secondary syringomyelia. An autopsy report.

    We report an elderly woman with multiple sclerosis who showed an extensive cavity formation in the midthoracic cord in addition to multiple abnormal intensity signals in the central nervous system on magnetic resonance imaging (MRI). The cavity decreased in size in response to corticosteroid therapy with an improvement in neurological symptoms. The autopsy demonstrated a slit-like cavity lined with no ependymal cells on the luminal surface in the lower cervical to midthoracic cord, with circumferentially distributed demyelinative lesions, leading to the pathological diagnosis of secondary syringomyelia. In this patient a limited necrosis formed in the spinal cord might have developed into a cavity formation with edematous fluid leading to subsequent episodes of neurological exacerbation.
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5/64. decompression of the spinal subarachnoid space as a solution for syringomyelia without Chiari malformation.

    STUDY DESIGN: review and analysis of seven cases of syringomyelia treated surgically. OBJECTIVE: To demonstrate the beneficial role of decompressive surgery for the altered cerebrospinal fluid (CSF) flow dynamics in syringomyelia not associated with Chiari I malformation. A comparison between the pre- and post-operative syrinx size and CSF flow in the subarachnoid space was made using cine-mode magnetic resonance imaging (cine-MRI) and then correlated with clinical improvement. SETTING: University Hospital, Seoul, korea. methods: Conventional spinal MRI and cine-MRI were performed in the region of CSF flow obstruction preoperatively in seven patients with syringomyelia not associated with Chiari I malformation. The group consisted of one case of syrinx with post-traumatic compression fracture, one case of post-traumatic arachnoiditis, two cases of holocord syrinx associated with hydrocephalus without Chiari malformation, one case of syrinx with post-traumatic pseudomeningeal cyst, one case of post-laminectomy kyphosis-associated syringomyelia and one case of post-tuberculous arachnoiditis syringomyelia. Based on the preoperative cine-MRI, the types of surgery appropriate to correct the CSF flow obstruction were chosen: decompressive laminectomy-adhesiolysis and augmentation duraplasty in arachnoiditis cases, ventriculoperitoneal shunt for hydrocephalus, cyst extirpation in pseudomeningeal cyst and both anterior and posterior decompression-fusion in the case of post-laminectomy kyphosis. A syrinx-draining shunt operation was performed in three cases; where the syringomyelia was associated with post-traumatic compression fracture refractory to a previous decompression, where hydrocephalus was present in which the decompression by ventriculoperitoneal shunt was insufficient and where post-traumatic arachnoiditis was present in which the decompression was impossible due to diffuse adhesion. Change in syrinx size was evaluated with post-operative MRI in all seven cases and restoration of flow dynamics was evaluated with cine-MRI in three of the cases, two patients with clinical improvement and one patient with no change of clinical status, respectively. RESULTS: Four out of seven patients showed symptomatic improvement after each decompressive operation. In the remaining three cases, reconstruction of the spinal subarachnoid space was not possible due to diffuse adhesion or was not the main problem as in the patient with syrinx associated with hydrocephalus who had to undergo a shunt operation. One of these three patients showed clinical improvement after undergoing syringosubarachnoid shunt. A decrease of syrinx size was observed in only two out of the five patients who showed clinical improvement after treatment. Of these five patients, two patients underwent post-operative cine-MRI and the restoration of normal CSF flow dynamics was noted in both patients. Of the remaining two patients, one underwent post-operative cine-MRI and there was no change in the CSF flow dynamics evident. CONCLUSION: These results suggest that the restoration of CSF flow dynamics between the syrinx and the subarachnoid space by decompressive operation is more effective than simple drainage of the syrinx cavity itself in the treatment of syringomyelia without Chiari malformation.
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6/64. syringomyelia without hindbrain herniation: tight cisterna magna. Report of four cases and a review of the literature.

    Idiopathic syringomyelia, which is not associated with any definite pathogenic lesions, has been treated mainly by shunting of the syrinx and rarely by craniocervical decompression. The authors report four cases of syringomyelia thought to be idiopathic syringomyelia but treated by craniocervical decompression with favorable results. syringomyelia was present without hindbrain herniation. In such cases, the subarachnoid space anterior to the brainstem at the level of the foramen magnum is usually open but the cisterna magna is impacted by the tonsils, a condition the authors term "tight cisterna magna." All patients underwent foramen magnum decompression and C-1 laminectomy, and the outer layer of the dura was peeled off. Further intradural exploration was performed when outflow of cerebrospinal fluid (CSF) from the fourth ventricle was deemed to be insufficient. Postoperatively, improvement in symptoms and a reduction in syrinx size were demonstrated in three patients, and a reduction in ventricle size was shown in two. syringomyelia associated with tight cisterna magna should not be classified as idiopathic syringomyelia; rather, it belongs to the category of organic syringomyelia such as Chiari malformation. A possible pathogenesis of cavitation is obstruction of the CSF outflow from the foramen of Magendie, and the cavity may be a communicating dilation of the central canal. Ventricular dilation may depend on the extent to which CSF drainage is impaired from the foramina of Luschka. These cavities may respond to craniocervical decompression if it results in sufficient CSF outflow from the foramen of Magendie, even in cases with concomitant hydrocephalus.
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7/64. Surgical management of spinal cord hemangioblastomas in patients with von hippel-lindau disease.

    OBJECT: Von Hippel-Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients. methods: Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20-58 years) at the National Institutes of health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered. CONCLUSIONS: Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.
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8/64. Spontaneous resolution of syringomyelia: report of two cases and review of the literature.

    OBJECTIVE AND IMPORTANCE: The natural history of syringomyelia is highly unpredictable, and some patients experience improvement or stabilization without surgery. However, the mechanisms of the formation and spontaneous resolution of syringomyelia remain controversial. This report concerns two patients with syringomyelia who demonstrated spontaneous reductions in syrinx size, accompanied by symptomatic improvement. CLINICAL PRESENTATION: One patient was a 10-year-old girl with syringomyelia associated with a tight cisterna magna and basilar impression, who demonstrated a spontaneous decrease in syrinx size, accompanied by symptomatic improvement, in 22 months. The other patient was a 39-year-old man with syringomyelia associated with a Chiari I malformation, who demonstrated a spontaneous reduction in syrinx size and neurological improvement, accompanied by elevation of the cerebellar tonsils, 6 months after diagnosis. INTERVENTION: The patients were monitored. CONCLUSION: The mechanisms of spontaneous resolution of syringomyelia, as well as the factors leading to the cerebrospinal fluid flow disturbances that cause syringomyelia, may vary. Resolution of foramen magnum lesion-related syringomyelia may be the result of spontaneous correction of the abnormal cerebrospinal fluid flow, as observed in our cases, or of cavity fluid drainage into the spinal arachnoid space because of spinal cord fissuring.
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9/64. syringomyelia in twin brothers discordant for Chiari I malformation: case report.

    Familial syringomyelia outside of trauma, tumor, or infection has been reported. Cases are presented that highlight the possible connection between familial syringomyelia and the Chiari 0 malformation. We report on 11-year-old twin brothers both with syringomyelia. magnetic resonance imaging further revealed that one brother had Chiari I malformation and the other had Chiari 0 malformation. Both underwent posterior fossa decompression with radiologic improvement of their syringes. These case reports lend credence to earlier reports of improvement in syringomyelia following posterior fossa decompression in the absence of Chiari I malformation, the so-called Chiari 0 malformation. In addition, these case reports should influence the manner in which familial syringomyelia without tonsillar ectopia is defined and addressed, that is, posterior fossa decompression versus shunting of the syrinx, thereby addressing the potential cause of the syringomyelia and not only the enlarged cavity itself.
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10/64. Intraoperative endomyelography during syrinx drainage: technical note.

    There is no currently available noninvasive technique to ascertain with certainty the continuity of the cavity of a septated syrinx. A technique is described that allows the confirmation of the continuity before the surgical drainage of a syrinx. This technique may prevent the failure of the drainage because of septations and double cavities. Intraoperative endomyelography is simple to perform and requires no special equipment. pressure measurements made before the injection of the contrast agent may provide insights into the pathogenesis of the syrinx and may be of prognostic and therapeutic significance.
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