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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a patient with late-onset tethered cord syndrome who presented with slowly progressive spastic paraparesis. Final diagnosis was made with spinal MRI. A 55-year-old man developed disturbance in gait at 52 years. He had severe congenital scoliosis of the thoracic spine and skin anomaly and hypertrichosis in the midline of the sacral region. His feet revealed pes equinovarus and pes cavus. Deep tendon reflexes in the lower extremities were hyperactive with positive Babinski signs. Vibration sense was diminished in the lower extremities. He had sexual impotence but no vesical or rectal disturbances. The plain X-rays revealed scoliosis at the fourth and fifth thoracic vertebrae, multiple costal fusion from the fourth through the eighth ribs on the left, and spina bifida occulta at the lumbosacral vertebrae. On MRI, the conus was tethered downward at the level between the forth and fifth vertebral bodies, and a thickened filum terminale connected the conus with a coccygeal cyst. Tethered cord syndrome, though rare in adults, should be differentiated from other diseases which produce late-onset spastic paraparesis, and MRI of the lumbo-sacral region is very useful for its diagnosis.
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PMID:[A case of late-onset tethered cord syndrome presenting with spastic paraparesis]. 766 30

We report on a 4-year-old boy with typical frontonasal dysostosis and an apparently balanced de novo translocation involving chromosomes 3, 7, and 11, and four breakpoints. The karyotype was 46,XY,t(7;3)(3;11) (7pter-->7q21.3::3q27-->3qter;3pter-->3 q23::11q21-->11qter; 11pter-->11q21::3q23-->3q27::7q21.3-->7 qter). In situ hybridization with a chromosome 3 painting probe confirmed the interpretation from GTG banding. The child had a widow's peak, marked hypertelorism, absence of the nasal tip, and widely separated nares. He also had an atrial septal defect, micropenis, small testes, clubfeet, scoliosis, block C2-4, and structural brain abnormalities on MRI. In review we found two other cases of frontonasal dysostosis with chromosome abnormalities, neither of which was similar to our case. The presence of a de novo (apparently) balanced translocation in our patient may help to locate the gene(s) for frontonasal dysplasia and perhaps other midline craniofacial malformations.
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PMID:Syndromal frontonasal dysostosis in a child with a complex translocation involving chromosomes 3, 7, and 11. 776 93

Clinical and radiological data of 48 patients with scoliosis who underwent routine MRI were reviewed to elicit the value of MRI in the preoperative assessment of complex spine deformities. Because of its high diagnostic sensitivity MRI gives a new insight into the true incidence of spinal cord pathology in scoliosis. Moreover, it identifies clinically unsuspected dysraphia in a surprisingly high percentage of neurologically asymptomatic patients with left-sided scoliosis. Exact planning of operative interventions is facilitated by the comprehensive diagnostic information gained through MRI. Furthermore, MRI generally allows one to refrain from invasive, high-irradiation techniques such as myelography and contrast-enhanced CT. On the basis of the presented investigational data, routine use of MRI is conclusively recommended in patients with congenital or idiopathic left-sided scoliosis.
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PMID:[The value of MRI in the preoperative assessment of scoliosis]. 797 Jun 92

The majority of scoliosis (80%) and especially the idiopathic scoliosis are simply investigated by plain films of the entire spine with erect frontal and lateral views, and supine frontal view at the initial examination. Repeated erect frontal views are sufficient for the follow-up. The important radiation dose (average of 1.2 mG for the frontal view and 2.2 mG for the lateral views) imposes to reduce the frequency of these radiographs. This radiological study allows: to separate non structural and structural curves; the identification and the measurement of a structural curve, discussion for its etiology, appraisal of its progressiveness, discussion of the operative or non operative treatment, and radiological follow-up. Modern imaging (C.T, C.T myelography, MRI) is indicated only in very particular cases: idiopathic scoliosis with neurological symptoms, congenital scoliosis with structural vertebral abnormalities, especially these with posterior arch abnormalities, and progressive scoliosis in spinal dysplasiae. Painful scoliosis are unusual. If pain is present consideration should be given for diagnosis of an underlying lesion. In this case a relevant strategy using modern imaging becomes useful and will be discussed.
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PMID:[Imaging of scoliosis]. 823 94

A rare case of intrathoracic meningocele associated with neurofibromatosis was reported. A 47-year-old female was admitted to our institute because of an abnormal shadow on chest X-ray. The chest X-ray film showed homogenous opacity with a well defined margin in the right apex. Chest CT showed an enlarged intervertebral foramen and defect of the vertebral arch around the tumor. There was no scoliosis. Though preoperative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of dura mater with spinal fluid out of the spinal canal, covered by thickening tumorous tissue. Part of the wall was excised and the residual opening was carefully repaired and wrapped by the pedicled parietal pleura. Retrospectively, MRI showed characteristic findings of intrathoracic meningocele. The tumorous part of it was diagnosed as neurofibroma on histological examination.
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PMID:[A case of intrathoracic meningocele associated with neurofibromatosis]. 830 12

We report two sibs with Angelman syndrome or an apparently new syndrome. In addition to severe mental retardation and seizures, clinical examination showed an ataxic and stiff legged gait, truncal hypotonia with hypertonia of the limbs, dysmorphic facial features (brachycephaly, large mouth, pointed chin and a prominent jaws) and scoliosis. Brain CT scan and MRI revealed ventricular enlargement and squared frontal horns. Pregnancy and delivery were uneventful. Karyotypes were normal. No deletion of chromosome 15q11-13 region was shown by molecular genetic techniques. The parents who are normal are second cousins. The condition is therefore probably inherited as an autosomal recessive one.
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PMID:Mental retardation, ataxia, seizures, dysmorphia, and hydrocephaly in two sibs. Angelman syndrome or new syndrome. 835 66

A rare case of spinal cord injury at C3 level associated with Arnold-Chiari malformation is reported. A 33 year old male was admitted with a complaint of walking difficulty. MRI revealed cerebellar herniation, and a plain X-ray showed spinous process adhesion of C2-3 and slight scoliosis. Percent vital capacity was reduced to 69%, and blood cell count showed a slight anemia. The patient was anesthetized for an orthopedic surgery with pethidine 70 mg, thiamylal 200 mg, 60% nitrous oxide, 40% oxide, and 0.5-1.2% isoflurane. There was a mild decrease in arterial blood pressure during surgical procedures, but this could be easily treated by fluid therapy, and there was no episode of autonomic hyperreflexia. Postoperative course of the patient was uneventful, except a transient pneumonia and urinary tract infection. The key in the anesthetic management of Arnold-Chiari malformation with spinal cord injury is the control of intracranial pressure, care for respiratory dysfunction and the prevention of infection as well as autonomic hyperreflexia.
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PMID:[The anesthetic management of Arnold-Chiari malformation with spinal cord injury]. 846 88

To investigate the pathology of cervical flexion myelopathy among younger people, clinical, morphological and biochemical studies were performed in six cases (four males and two females) treated by posterior fusion. X-ray films showed, in all cases, scoliosis in the cervical spine as well as a reduction in the sagittal diameter and an anterior deviation in the spinal cord at the time of flexion. Spinal atrophy in the sagittal plane and crescent-shaped high contrast areas posterior to the dural tube were also observed on MRI images. Surgical findings, showed substantial expansion by the veins posterior to the dural tube in five cases with four showing accompanying organization. Morphological studies showed that in the musculus splenius capitis, the type 1 fibers were slightly more than in normal tissues. Moreover in one case, we observed neurogenic lesions such as the predominance and atrophy of type 2 fibers. Biochemical analyses of the collagen from muscles and skin showed immature collagen fibers, which was marked particularly in skin. The above results suggested that abnormalities in the collagen fibers in the soft tissues were related to the pathogenesis of cervical flexion myelopathy.
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PMID:[Cervical flexion myelopathy: clinical, morphological and biochemical studies on the soft tissues of the cervical spine in young-age operated cases]. 853 Aug 81

Neurological deficiency can occur during or after spinal surgery. The most severe complications are seen after instrumental correction for scoliosis or kyphosis. Regression of paraplegia, paraparesia and Brown-Sequard syndrome is never a certainty and usually incomplete. Preoperative manoeuvres and evoked potentials do not provide absolute security and metal instrumentation should always be used prudently. The main risk factors are vertebral malformation, major kyphosis, preoperative signs of neurological deficit, excessive correction and double anterior and posterior access. Finally, the canal is poorly vascularized from T4 to T8 or T9 which can raise further problems. Cordal deficiency during or following almost always requires removal of the metal implant, and exploration of the canal possibly with MRI. Injury include direct contusion of the spinal cord, devascularization and compressive haematomas. The frequency of neurological complications is currently about 1% and only extreme prudence and knowledge of causes can reduce this rate.
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PMID:[Neurologic complications of surgery of the spine in children]. 866 10

In a prospective trial we performed MRI of the spine and hind brain in 31 patients with scoliosis of onset between the ages of four and 12 years. In eight patients (26%) there was a significant neuroanatomical abnormality; there were six cases of Chiari-1 malformation associated with a syrinx, one isolated Chiari-1 malformation and one astrocytoma of the cervical spine. Four of these patients had left-sided curves. There were no clinical features which could reliably identify those patients with abnormalities on MRI. In particular, the unilateral absence of abdominal reflexes was found to be non-specific (1 of 8 of patients with neuroanatomical abnormalities (12.5%) v 2 of 23 with normal scans (8.7%). In view of the established risks of surgical correction of scoliosis in the presence of undecompressed syringomyelia and the possible improvement that may follow decompression of the foramen magnum, we feel that MRI of all patients with scoliosis of juvenile onset should be obligatory.
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PMID:MRI of 'idiopathic' juvenile scoliosis. A prospective study. 866 49


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