Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

MRI is the best imaging method to evaluate syringomyelia. It is important to study from the posterior cranial fossa to the sacro-lumbar region and also the supra-tentorial structures. This complete analysis is essential to classify the syringomyelia and to investigate other associated malformations. Radiographs and CT scan are useful to analyze bone structures. For MRI, the new sequences with phased-array coils are also very important to study the entire spinal cord and the posterior fossa. It is essential to study the spinal cord with sagittal and axial spin echo T1 and fast spin echo T2 weighted images with sometimes coronal view, particularly when the patient presents a scoliosis, to have a correct morphological and functional evaluation. MRI gives an excellent study of the spinal cord with an excellent analysis of a primitive or foraminal syringomyelia, but also traumatic, infectious or post arachnoiditis syringomyelia. Spin echo T1 weighted images with injection of gadolinium can be used if an intra-medullary tumor is suspected. MRI is also useful for the post-operative follow up to evaluate the persistence of the medullary cyst and the enlargement of the foramen magnum.
 ...
PMID:[Imaging of syringomyelia]. 1042 Apr 8

We reported a 58-year-old man with neurofibromatosis and an intrathoracic meningocele. He was admitted to our hospital because of left-sided chest pain and dyspnea on exertion. He presented with severe kyphoscoliosis and showed a round, well circumscribed mass lesion in the paravertebral region of the left upper lung on a chest roentgenogram. Just before admission, pleural effusion accumulated in the left thoracic cavity, which had caused the respiratory symptoms. The mass was diagnosed as an intrathoracic meningocele by MRI and iotrolan CT myelography. The pleural effusion was transudate fluid and no leakage from the meningocele to pleural cavity was demonstrated. Posterolateral extradural approach with laminectomy was done and dural plasty to close the connection between the meningocele and the subarachnoidal space was carried out. After the operation, both the intrathoracic meningocele and the pleural effusion disappeared with remarkable improvement in the respiratory function. Intrathoracic meningocele is known to be seen in association with neurofibromatosis and scoliosis, but it is very rare to see an intrathoracic meningocele which causes respiratory failure due to massive pleural effusion like this report.
 ...
PMID:[A case of neurofibromatosis with intrathoracic meningocele presenting respiratory failure caused by pleural effusion]. 1055 89

We report 5 girls presenting Rett syndrome. All of them were from south Tunisia. They fulfilled the Rett syndrome diagnosis criteria (The Rett syndrome diagnosis criteria work group, 1988). Pregnancy, birth and psychomotor development during the first year of live were normal. The mean age at the onset was 19.8 +/- 2.5 months. The two revealing symptoms were psychomotor regression (3 cases) and epilepsy (2 cases). They were admitted to our ward at a mean age of 4.7 +/- 1.5 years. Clinical presentation was typical of Rett syndrome. Mental retardation, stereotypic hand movement (hand washing/wringing or clapping/tapping) and loss of purposeful manual skills were noted in all cases. Gait was apraxic and increase of head circumference was slowed. Additional features included, respiratory dysfunction (episodic hyperventilation and breath-holding), epilepsy, scoliosis (4 cases), growth retardation and spasticity (3 cases). Electroencephalography showed slow activity with multifocal epileptiform abnormalities. Sleep enhanced these EEG abnormalities. MRI and CT-scan disclosed non specific cortical and sub-cortical atrophy. All cases were isolated and parents were consanguineous in 3 cases. Rett syndrome is relatively frequent in Europe, but in Tunisia this disease remains rare and certainly underdiagnosed.
 ...
PMID:[Rett's syndrome: report of 5 cases in Tunisia]. 1060 40

The management of patients with Type I Chiari malformations (CM 1) with or without syringohydromyelia (SHM) has remained quite controversial, and many different surgical procedures have been advocated. Over the past several years, the authors have treated 7 children presenting with CM 1 and holocord syringohydromyelia with suboccipital decompression and duraplasty alone without intradural procedures. All children received MRI imaging at 2-4 months and 1 year postoperatively. On the early postoperative MRI examination, marked reduction in the syringohydromyelia was seen in 6 children, with minimal change in syrinx size in 1 child who was clinically improving after the operation. At 1 year, all children with early collapse remained collapsed, and the child with minimal early collapse demonstrated an approximately 50% reduction in syrinx size. Clinical follow-up (mean 30 months, range 21-50 months) showed good results in all patients: none of the children have required further neurosurgical intervention, and all have shown improvement in their preoperative function. One child with a 46 degrees scoliosis had a complete collapse of her SHM, but ultimately required spinal fusion. The presenting clinical findings, operative technique, MRI imaging and clinical outcomes will be discussed. The results from these 7 patients with CM 1 and holocord syringomyelia suggest that posterior fossa decompression alone (without intradural procedures) can provide excellent radiographic and clinical outcome.
 ...
PMID:Management of Chiari I malformations with holocord syringohydromyelia. 1070 56

We report four cases of scoliosis associated with diatematomyelia observed between 1984 and 1998. The patients were four girls aged 10 to 12 years. Skin lesions were found on the midline in 3 cases and 2 had a neurological disorder. A myeloscan was performed in two cases, tomomyelography in one and MRI in one. Three patients were operated on with good outcome. The fourth child is under orthopedic treatment. We reviewed the literature on scoliosis with diastematomyelia.
 ...
PMID:[Scoliosis and diastematomyelia: four cases and a review of the literature]. 1084 61

The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found.
 ...
PMID:Syringomyelia in children with primary scoliosis. 1085 15

The objective of the present study was to determine the incidence of unsuspected intraspinal pathology and to assess the value of atypical clinical features as predictors of these intraspinal pathologies, in patients with idiopathic scoliosis. Twenty-five consecutive patients (13 boys, 12 girls) with idiopathic scoliosis were prospectively evaluated with MRI. Magnetic resonance imaging detected intraspinal pathology in seven patients (28%), which included syringohydromyelia with Chiari I malformation (n = 5), and syringomyelia and dumb-bell neurofibromas in one patient each, respectively. Dural ectasia was also present in five patients. Atypical features, described in the literature as pointers to intraspinal pathologies such as the age < 11 years at presentation, presence of pain, hyperkyphosis, severe curves and the presence of the left thoracic or thoracolumbar curves, were seen to be equally distributed between the two groups (those with and without intraspinal pathologies), thus raising doubts about the importance of these features.
 ...
PMID:Magnetic resonance evaluation of idiopathic scoliosis: a prospective study. 1090 60

Spine deformities are a frequent symptom of neurofibromatosis Recklinghausen. Especially NF1 shows next to numerous alterations in the skeleton in some cases massive scoliosis and kyphosis. There are different theories for the development of the spine deformities, one of them is that specific alterations of the vertebra are caused by an elevated intraspinal pressure on the osteoporotic bone. A classification from a clinical point of view discriminates 3 types of severity. Type 1 shows instead of the in x-rays inconspicuous findings neurofibromatosistypical alterations in other diagnostic procedures (e.g. MRI). Extreme variations like short curved scoliokyphosis with massive destruction and severe spine imbalance are described as type 3. Operative treatment is dependent on the severity of the deformity. Intraspinal tumors have to be removed. Because of the elevated neurological risk the proceeding has to be very careful, sometimes there is a temporary Halo-extension necessary. Anterior substance defects are filled with bone or cages. The posterior instrumentation (in most of the cases a 2-rod-stabilization) is performed by transpedicular screws. Frequently there is a concave chest wall plastic (CTP) indicated. To prevent neurological complications early surgical procedure is sometimes necessary. Complications can be reduced by careful proceeding, exact preoperative diagnostic and classification. But next to operative experience a qualified anaesthesiological and intensive care units are absolutely necessary.
 ...
PMID:[Treatment of scoliosis and scoliokyphosis in Recklinghausen neurofibromatosis]. 1092 33

We report two cases of spinal osteoblastoma in two boys aged 16 and 19 years. The lesion was disclosed by scoliosis with signs of thoracic and lumbar neurological compression. The diagnosis was provided by the CT scan and magnetic resonance imaging and was confirmed by the histology study of the surgical specimen. Involvement of the vertebral column has been estimated to range from 30 to 40% for these rare tumors that account for less than 1% of all bone tumors. Localization on the convex aspect of scoliosis is rare. CT-scan provides an analysis of the tumor components and clearly demonstrates intraspinal extension. MRI is superior in visualizing neurological compression. In our experience, function outcome has been favorable after surgical decompression.
 ...
PMID:[Vertebral osteoblastoma and scoliosis. Two cases report]. 1128 55

We performed a retrospective review of 27 scoliotic patients with syringomyelia using MRI. Their mean age at the first MRI examination was 10.9 years, and at the final review 15.8 years. The mean ratio of the diameter of the syrinx to the cord on the midsagittal MRI (S/C ratio) decreased from 0.49 to 0.24; 14 patients showed a decrease of 50% or more (reduction group). In this reduction group, the cerebellar tonsillar herniation decreased from a mean of 11.3 mm to 6.0 mm, and some improvement in dissociated sensory disturbance was seen in nine of 13 patients. The scoliosis improved by 5 degrees or more in six patients in the reduction group. Our results indicate that spontaneous shrinkage of syringomyelia in children is not unusual and is associated with improvement in the tonsillar herniation, the scoliosis and the neurological deficit.
 ...
PMID:Natural history of scoliosis in children with syringomyelia. 1134 22


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>