1/86. The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention.OBJECTIVE: To determine how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. methods: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. RESULTS: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. CONCLUSION: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/86. arnold-chiari malformation with syringomyelia in an elderly woman.PRESENTATION: A 76-year-old woman, complaining of leg pain and unsteady gait for 3 years, presented with a spastic paraparetic gait, severe spasticity and touch, thermal and pain sensory loss limited to arms, lower thorax and upper abdomen. brain and spinal cord magnetic resonance imaging showed a large loculated syrinx. Cerebellar tonsillar herniation into the foramen magnum was also seen (arnold-chiari malformation, type I). OUTCOME: The patient had successful cervico-spinal surgical decompression which resulted in marked reduction in hypertonia and weakness, normal gait and normal joint movement at 6 months. CONCLUSION: This unusual, late clinical presentation of a congenital disease underlines the importance of a comprehensive diagnostic work-up in the elderly patients with complex neurological signs.- - - - - - - - - - ranking = 0.5keywords = upper (Clic here for more details about this article) |
3/86. Acute brainstem symptoms associated with cervical syringomyelia.syringomyelia classically presents with slowly progressing dissociated sensory and upper and lower motor deficits. Atypical and acute manifestations have rarely been described. We report here on 3 patients with syringomyelia, who had acute and atypical brainstem symptoms with regard to the underlying disease. These symptoms occurred after acute elevation of the intrathoracic and intra-abdominal pressure, respectively, and remitted subsequently. Vertebrobasilar ischemia was initially suspected.- - - - - - - - - - ranking = 0.5keywords = upper (Clic here for more details about this article) |
4/86. Isolated Horner's syndrome and syringomyelia.Although syringomyelia has been associated with Horner's syndrome, it is typically associated with other neurological findings such as upper limb weakness or numbness. A patient is described who had an isolated Horner's syndrome as the only manifestation of syringomyelia. A 76 year old woman was discovered to have right upper lid ptosis and right pupillary miosis. Neurological examination was unremarkable, and pharmacological testing was consistent with localisation of the lesion to a first or second order sympathetic neuron. neuroimaging disclosed a Chiari I malformation with a syrinx extending to the C2 to C4 level. An isolated Horner's syndrome may be the presenting manifestation of syringomyelia.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
5/86. Resolution of tonsillar herniation and syringomyelia after supratentorial tumor resection: case report and review of the literature.OBJECTIVE AND IMPORTANCE: The pathophysiological features of syringomyelia are not yet entirely understood. We present a case of a supratentorial mass causing tonsillar herniation and syringomyelia. CLINICAL PRESENTATION: A 51-year-old woman underwent magnetic resonance imaging for evaluation of progressive headaches. A large parieto-occipital mass was revealed. Herniation of the cerebellar tonsils and a cervical syrinx were also noted. INTERVENTION: A craniotomy was performed without incident. After tumor resection, the tonsils ascended and the syrinx resolved in a 1-year period. CONCLUSION: This case highlights the importance of tonsillar herniation in the pathogenesis of syringomyelia. "Acquired" Chiari malformations and syringomyelia attributable to supratentorial masses may be treated by mass resection alone, without the need for foramen magnum decompression.- - - - - - - - - - ranking = 2.5156341780891keywords = headache (Clic here for more details about this article) |
6/86. Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report.The authors report the use of neuroendoscopic third ventriculostomy to treat successfully both hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. A 27-year-old woman presented with dizziness, headache, and nausea. Magnetic resonance (MR) imaging demonstrated dilation of all ventricles, downward displacement of the third ventricular floor, obliteration of the retrocerebellar cerebrospinal fluid (CSF) space, funnellike enlargement of the entrance of the central canal in the fourth ventricle, and syringomyelia involving mainly the cervical spinal cord. Cine-MR imaging indicated patency of the aqueduct and an absent CSF flow signal in the area of the cistema magna, which indicated obstruction of the outlets of the fourth ventricle. Although results of radioisotope cisternography indicated failure of CSF absorption, neuroendoscopic third ventriculostomy completely resolved all symptoms as well as the ventricular and spinal cord abnormalities evident on MR images. Neuroendoscopic third ventriculostomy is an important option for treating hydrocephalus in patients with fourth ventricle outlet obstruction.- - - - - - - - - - ranking = 2.5156341780891keywords = headache (Clic here for more details about this article) |
7/86. Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression.Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum. CMI has been shown to be closely associated with the development of syringomyelia (SM). OBJECTS: Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development of SM. However, the exact mechanism of the evolution of SM is still subject to controversy. We report here the case of a 12-year-old girl admitted to hospital with headache, vomiting, ataxia, and moderate pyramidal signs. methods: Radiological evaluation revealed the presence of CMI, accompanied by a small SM. The patient underwent posterior fossa decompression and improved significantly. She was re-admitted 6 months later with clinical evidence of progressive spinal cord dysfunction. MR revealed gross expansion of the syrinx. CONCLUSIONS: This case raises questions regarding the pathophysiology of CMI and its association with SM. The case indicates the need for neurological and radiological follow-up for patients undergoing posterior fossa decompression due to CMI, even for those without an initial syrinx. This is the first report known to us of expansion of a syrinx following decompression of an associated CMI.- - - - - - - - - - ranking = 2.5156341780891keywords = headache (Clic here for more details about this article) |
8/86. Cutaneous and mixed nerve silent periods in syringomyelia.OBJECTIVES AND methods: We studied cutaneous and mixed nerve silent periods (CSP, MNSP) in 4 patients with cervical syringomyelia documented by magnetic resonance imaging who on clinical examination presented with unilateral hypalgesia and hypothermesthesia. In addition, we recorded upper and lower extremity somatosensory and motor evoked potentials (SEP, MEP), and cortical silent periods. RESULTS: In all patients, CSP and the later portion of MNSP were absent or shortened on their affected side, while both were normal on their unaffected side. In all patients, SEP latencies were normal following both median and tibial nerve stimulation. In two patients, the amplitude N13 (median nerve SEP), and in one patient each the amplitudes N20 (median nerve SEP) and P37 (tibial nerve SEP) were reduced. Central motor conduction time was prolonged to abductor digiti minimi muscle in one patient on the affected side, but was normal to tibialis anterior muscle in all patients. Cortical silent periods where present bilaterally in spite of unilateral complete absence of CSP and MNSP in two subjects tested. Loss of CSP and MNSP were a sensitive parameter of spinal cord dysfunction in syringomyelia. The cervical median nerve SEP response N13 reflected gray matter involvement, while corticospinal tract dysfunction was less frequently observed. CONCLUSION: Our data suggest that CSP and later portion of MNSP are generated at the spinal level by the same small myelinated A-delta fibers, and that their central network is distinct from large diameter fiber afferents and efferents.- - - - - - - - - - ranking = 0.5keywords = upper (Clic here for more details about this article) |
9/86. 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.- - - - - - - - - - ranking = 0.5keywords = upper (Clic here for more details about this article) |
10/86. cesarean section in a patient with syringomyelia.PURPOSE: To describe the anesthetic management of cesarean section in a patient with syringomyelia. CLINICAL FEATURES: A 27-yr-old pregnant woman with syringomyelia was scheduled to undergo elective cesarean section. At the age of 25 yr, she had begun to experience headaches, and at the age of 26 yr, a diagnosis of syringomyelia of the upper spinal cord was made on the basis of magnetic resonance imaging findings. No symptoms other than headache were noted preoperatively. General anesthesia was used for the cesarean section. After the administration of 1 mg vecuronium as a priming dose, 5 mg vecuronium were injected. At the onset of clinical muscle weakness, 225 mg thiamylal were promptly administered as the induction agent and the patient was intubated (timing principle with priming method) and pressure on the cricoid cartilage applied to prevent regurgitation of stomach contents. anesthesia was maintained with oxygen, nitrous oxide and isoflurane at a low concentration. Mild hyperventilation was used throughout the procedure. anesthesia and surgery proceeded without any problem, response to vecuronium was clinically normal and recovery was uneventful. Neurological status remained normal. CONCLUSION: We report the safe use of general anesthesia for cesarean section in a patient with syringomyelia. Precautions were taken to avoid increases in intracranial pressure and our patient experienced no untoward neurologic event.- - - - - - - - - - ranking = 5.5312683561781keywords = headache, upper (Clic here for more details about this article) |
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