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

Purely cortical cerebral calcification without involvement of the cerebellum was observed radiologically in a 3-month old girl; the calcification was extensive and symmetrical. During a three year period of observation, she showed severe psychomotor retardation, spasticity, microcephaly, a secondary scoliosis, subluxation of the hips and fits. These changes have only been observed so far following pre-natal herpes encephalitis. Following this condition there may also be periventricular calcification. Therefore it is assumed that the extensive cortical calcification is due to a virus, but not necessarily a specific one and that it depends on the age of the foetus at the time of the infection.
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PMID:[Symmetrical cortical calcification of the cerebrum following pre-natal encephalitis (author's transl)]. 14 6

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.
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PMID:Orthopaedic features in the presentation of syringomyelia. 15 24

A 19-year-old girl with carpal and tarsal osteolysis has been presented. The clinical course was characterized by normal early growth and motor development followed by the insidious onset in early childhood of a progressive, peripheral joint destruction, especially in the tarsal and carpal bones. The patient's course was complicated by acquired spasticity from platybasia, scoliosis, generalized muscle weakness, mild growth failure and corneal clouding.
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PMID:Carpal and tarsal osteolysis. 120 35

Between 1979 and 1991, spinal arachnoid cysts were found in 11 patients aged 19 months to 18 years (mean age 5 1/2 years). Of the 11 patients, six had a myelomeningocele and one diastematomyelia. The presenting symptoms included radicular pain (one patient), progressive weakness (three), increasing scoliosis (one), worsening spasticity (three), and recurrent urinary tract infections and progressive constipation (one). Two patients showed no symptoms from the spinal arachnoid cyst. The distribution of lesions was as follows: cervicomedullary (one patient), cervical (one), cervicothoracic (two), thoracic (four), lumbar (two), and sacral (one). Four of the 11 arachnoid cysts (all intradural) were located anterior to the spinal cord, three of which were in children with a myelomeningocele. Only two of the cysts were extradural; both were found in the lumbosacral region, and one was associated with diastematomyelia. Eight patients were treated with fenestration and/or resection of the cyst wall. Three patients with anterior cysts were treated with shunts, a cyst-to-pleural space shunt in two and a cyst-to-subarachnoid space shunt in one. All of the patients either improved or exhibited an arrest in the progression of their symptoms. Spinal arachnoid cysts are a treatable cause of progressive neurological deficits and, in this series, were frequently found in patients with neural tube defects.
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PMID:Spinal arachnoid cysts in the pediatric age group: an association with neural tube defects. 150 83

Nearly drowned children sustaining cardiac or respiratory arrest or altered neurologic status developed anoxic encephalopathy (30%), died in the ensuing days (36%), or were discharged neurologically normal (34%). In encephalopathic children, spasticity was more malignant than in children with other causes of spasticity. The most common musculoskeletal problem was contracture, especially equinus, hip adductor, hamstring, and quadriceps. Hip subluxation or dislocation occurred in 34% as early as 1 month after injury. Scoliosis developed in 18%, with some requiring bracing and surgery. Thirty-one percent ambulated, and the rest were quadriplegic. Independent sitting or better function by discharge was a predictor of ambulation potential. Of the quadriplegics, 66% were dependent sitters.
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PMID:Musculoskeletal consequences of near-drowning in children. 201 May 14

Fifty-five children with cerebral palsy had multiple-level laminectomies for selective posterior rhizotomies for the relief of spasticity. They were followed up clinically and radiologically to assess their spinal stability and the possible development of post-laminectomy deformity of the spine. The majority of the deformities found were related to cerebral palsy and did not appear to be due to the laminectomy: 16% had scoliosis, 5% kyphosis, 7% lordosis, and 9% spondylolysis/spondylolisthesis. Spondylolysis is the only abnormality that appeared to be more common in this group than in children with cerebral palsy.
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PMID:Incidence of spinal deformity in children after multiple level laminectomy for selective posterior rhizotomy. 231 Nov 12

Diagnostic criteria for Rett syndrome (RS) were developed by representatives of the International Rett Syndrome Association and the Centers for Disease Control for use in future clinical and epidemiological studies. Necessary criteria are: normal prenatal and perinatal period; normal psychomotor development through the first 6 months of life; normal head circumference at birth, with subsequent deceleration of head growth; loss of purposeful hand skills; severely impaired expressive and receptive language; apparent severe mental retardation; and gait apraxia and truncal apraxia/ataxia. Supportive criteria include breathing dysfunction, seizures, spasticity, scoliosis, and growth retardation. The diagnosis of RS is considered tentative until 2 to 5 years of age. The differential diagnosis includes other disorders associated with mental retardation, cerebral palsy, and seizure disorders. These diagnostic criteria for RS should foster reliable communication among investigators and enhance the epidemiological and clinical research of this important disorder.
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PMID:Diagnostic criteria for Rett syndrome. The Rett Syndrome Diagnostic Criteria Work Group. 245 7

The purpose of this study was to develop a simple and reproducible model of scoliosis in the rat without producing direct trauma to the spine, in order to both provide a system for the study of human scoliosis and to ultimately study the conditions under which distraction-induced trauma might occur. Right lateral curvatures were produced in three groups of rats (N = 36, 59, 25) at 18-21 days of age by suturing the inferior angle of the scapula to the ipsilateral bony pelvis. In the first group of animals, it was found that a minimum period of 6 weeks of tethering was required in order to produce permanent structural curvatures after release of the tethering sutures. In the second group of rats, a number of morphologic and histologic changes characteristic of human scoliosis were noted, including apical wedging, deviation of the apical spinous process, pelvic asymmetry, rib hump deformity, vertebral rotation, displacement of the nucleus pulposus, disorganized columnization of disc cartilage cells, and an increased number of Type I muscle fibers in paravertebral muscle on the convex side of the curve. Finally, in the third group of rats, a mild spasticity was seen in the ipsilateral hindlimb in approximately one-third of the animals with curvatures greater than 40 degrees. This spasticity was not associated with a change of latency or amplitude of the somatosensory-evoked potential (SSEP). Each curvature group displayed SSEP characteristics that were not statistically different from unoperated control animals. These results indicate the validity of this model system for the study of scoliosis, and, in particular, its usefulness for the study of operative-induced trauma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Experimental scoliosis in the rat. I. Methodology, anatomic features and neurologic characterization. 305 39

This preliminary report describes two cases of progressive spasticity and scoliosis in children with myelomeningocele. Both patients had evidence of tethered spinal cord. Untethering of the spinal cord was successful in arresting the scoliosis but not the spasticity. Selective posterior rhizotomy was successful in resolving the spasticity in both cases. The technique of selective posterior rhizotomy is reviewed.
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PMID:Selective posterior rhizotomy for treatment of progressive spasticity in patients with myelomeningocele. Preliminary report. 345 37

Rett syndrome, named after Rett's first description in 1966, evolves typically in 3 stages: after normal early psychomotor development up to the age of 6-24 months, stagnation and regression occur over a few months resulting in severe dementia, loss of speech, of social response and of purposeful hand use. This is accompanied by particular stereotyped hand movements and usually also by deceleration of head growth. The further course is often stable for a prolonged period, or only slowly progressive. Common features are seizures, episodic hyperpnea, scoliosis, spasticity and vasomotor disturbances of lower limbs. Rett syndrome has been observed only in girls, all cases (with 2 exceptions) being sporadic. This is probably explained by a X-linked dominant new mutation lethal in males. The pathogenesis is still unknown: no consistent metabolic, morphologic or neuroradiologic abnormalities have been found. According to some epidemiologic investigations, Rett syndrome affects about 1:15,000 girls and is thus responsible for a considerable proportion of severely retarded girls. Within one year the authors have retrospectively diagnosed 15 cases, which is assumed to represent only about a third of patients in Switzerland.
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PMID:[Rett syndrome: a progressive neurological syndrome in girls]. 370 8


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