Syringohydromyelia associated to therapeutic procedures for severe forms of neurocysticercoses: case report.
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Syringohydromyelia is defined as a longitudinal dilatation of the central canal of the spinal cord with accumulated cerebrospinal fluid. This condition may cause neurologic deficits when the cavity enlarges and compresses the spinal cord. We present the case of a 33 years-old female with progressive paraparesis caused by syringohydromyelia. This patient underwent previously multiple clinical and surgical treatments for severe form of neurocysticercosis. Surgical decompression of the posterior fossa and syringostomy resolved the neurologic symptoms. The possibility of syringohydromyelia should be considered in the case of patients who have previously undergone surgical and clinical treatment for severe form of neurocysticercosis. (+info)
Subdural hematoma caused by intracranial hypotension after syringosubarachnoid shunting--case report.
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A 32-year-old man developed an extremely rare subdural hematoma after syringosubarachnoid shunting for syringomyelia. He presented with a 4-year history of neck pain and spastic paraparesis resulting from T-7 and T-8 vertebral body fracture suffered in a traffic accident at age 22 years. Magnetic resonance imaging revealed syringomyelia between the craniocervical junction and the T-10 level. The symptoms were slowly progressive, and a syringosubarachnoid shunting was performed. His spasticity improved after surgery, but he developed orthostatic headache 7 days after surgery. Magnetic resonance imaging of the brain demonstrated a thin subdural hematoma over the right cerebral convexity. The subdural hematoma resolved spontaneously within a week with conservative treatment. Vigorous cerebrospinal fluid outflow observed during surgery presumably lowered the pressure in the syrinx cavity, leading to significant but transient intracranial hypotension and consequently the formation of subdural hematoma. (+info)
Factors influencing the outcome in symptomatic Chiari I malformation.
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BACKGROUND: To study the clinico-radiological determinants of outcome in patients with Chiari I malformation (CIM). MATERIALS AND METHODS: The disability assessment of 48 patients with C I M who underwent posterior decompression was done by modified Klekamp and Samii scoring system. The outcome was regarded as good when the patient was ambulant without any aid with an improvement in the disability score; and, poor when (a) there was postoperative deterioration or lack of improvement; (b) the patient was non-ambulant without aid, irrespective of the improvement in the clinical score; or, (c) there was a perioperative mortality. Patients with hydrocephalus; those who underwent syringo-subarachnoid or syringo-peritoneal shunt as the primary procedure; and, patients with atlanto-axial dislocation were excluded from the study. STATISTICAL METHODS USED: Categorical data were expressed in proportions and analyzed with Chi square test. Analysis of factors predicting clinical outcome at 6 months was done utilizing logistic regression analysis. RESULTS: The outcome assessed at six months showed that 30 patients (62.5%) had good outcome while 18 patients (37.5%; including two perioperative mortalities) had a poor outcome. CONCLUSIONS: Significant predictors of outcome in patients with CIM include the duration of symptoms (P value=0.006), respiratory distress (P value=0.001), and basilar invagination (P value=0.048). The effect of syringomyelia in predicting the clinical outcome could not be determined due to the differences in the number of patients in the groups with or without syringomyelia. (+info)
Terminal syringomyelia: is it as innocent as it seems?--Case report.
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A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases. (+info)
Orthopaedic features in the presentation of syringomyelia.
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The orthopaedic surgeon is often the first consultant to whom a patient with syringomyelia is referred. The disease is not as rare as he may suppose, but its early presenting features are very variable; if he relies solely on such familiar features as pes cavus and scoliosis, he may well miss the diagnosis. The commonest presenting symptom is pain in the head, neck, trunk or limbs; headache or neckache made worse by straining is particularly significant. A history of birth injury also may suggest the possibility of syringomyelia, especially if any spasticity subsequently worsens. Neurological features which may be diagnostic include nystagmus, dissociated sensory loss, muscle wasting, spasticity of the lower limbs or Charcot's joints. Radiographic features include erosion of the bodies of cervical vertebrae and widening of the spinal canal; if, at C5, the size of the canal exceeds that of the body by 6 millimetres in the adult, pathological dilatation is present. The presence of basilar invagination or other abnormalities of the foramen magnum, of spina bifida occulta and of scoliosis are further pointers. Thermography is a useful way of showing asymmetrical sympathetic involvement in early cases. A greater awareness of the prevalence of syringomyelia may lead to earlier diagnosis and to early operation, which appears to hold out the best hope of arresting what is all too commonly a severely disabling and progressive condition. (+info)
Inherited occipital hypoplasia/syringomyelia in the cavalier King Charles spaniel: experiences in setting up a worldwide DNA collection.
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Inherited diseases commonly emerge within pedigree dog populations, often due to use of repeatedly bred carrier sire(s) within a small gene pool. Accurate family records are usually available making linkage analysis possible. However, there are many factors that are intrinsically difficult about collecting DNA and collating pedigree information from a large canine population. The keys to a successful DNA collection program include (1) the need to establish and maintain support from the pedigree breed clubs and pet owners; (2) committed individual(s) who can devote the considerable amount of time and energy to coordinating sample collection and communicating with breeders and clubs; and (3) providing means by which genotypic and phenotypic information can be easily collected and stored. In this article we described the clinical characteristics of inherited occipital hypoplasia/syringomyelia (Chiari type I malformation) in the cavalier King Charles spaniel and our experiences in establishing a pedigree and DNA database to study the disease. (+info)
Cystic cervical intramedullary schwannoma with syringomyelia.
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We report a case of cervical intramedullary cystic schwannoma associated with segmental syrinx in a young adult without evidence of neurofibromatosis. The relevant literature is reviewed. (+info)
Significant scoliosis regression following syringomyelia decompression: case report.
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We present the case of a 5-year-old boy presenting with a 54-degree scoliosis secondary to a Chiari I malformation with a holocord syringomyelia extending from C1 to T10. Neurosurgical treatment involved posterior fossa craniectomy with decompression, and partial C1 laminectomy. At follow-up 7 years later, at age 12, radiographs revealed only a 4-degree scoliosis, and follow-up MRI revealed a deflated syrinx. We report this case to reveal the most significant scoliosis regression seen in our experience that may occur in younger patients after neurosurgical syringomyelia decompression for Chiari I hindbrain herniation. (+info)