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

Double crush syndrome (DCS) was first described by Upton and McComas who proposed that focal compression of an axon often occurs at more than one level. The aim of the study was to support the hypothesis of DCS of the median nerve on the basis of available diagnostic methods. 30 patients (25 F and 5 M aged 33-73, mean 54.6+/-8.2 years) with coexisting carpal tunnel syndrome (CTS) and cervical radiculopathy (CR) were examined. Control group included 40 healthy volunteers (27 F and 13 M aged 17-82, mean 43.1+/-11 years). Medical evaluation comprised clinical examination, X-ray and MR imaging of the cervical spine, electroneurography (ENG) with F-wave and somatosensory evoked potentials (mSEPs) of median nerves. In clinical examination 96.6% of patients suffered from cervical spine pain and nocturnal paresthesies of at least one hand. Muscular atrophy was present in 43.3% in the proximal and in 70% in the distal part of the upper extremity. 30.3% of patients presented with a thoracic scoliosis. On X-ray examination, all patients showed cervical discopathy, mostly C5-C6 (70%) and C6-C7 (53.3%). Using MR investigation, the narrowing of intervertebral foramina was present in 81.25% and narrowing of vertebral canal in 37.5%. On ENG all patients presented with CTS, bilaterally in 73.3%. The F wave was abnormal in 73.3% and mSEPs in 66.7% of patients. Coincidence of MR and mSEPs in view of lateralization was observed in 71.4%. Results supported the DSC hypothesis. DCS evaluation requires both structural and functional diagnosis of peripheral neurones using MRI and electrophysiological examination.
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PMID:Double crush syndrome evaluation in the median nerve in clinical, radiological and electrophysiological examination. 1710 65

The purpose of the study was to further explore the radiological presentations of scoliosis associated with syringomyelia in relation to curve severity. 87 children and adolescents were divided into three groups: Group I (10 degrees <or=Cobb angle<or=30 degrees), Group II (30 degrees <Cobb angle<or=60 degrees), and Group III (Cobb angle >60 degrees). Curves were classified into typical and atypical patterns in the coronal plane and the sagittal profile measured. Cerebellar tonsillar descent or syrinx patterns in relation to curve severity and the frequency of atypical curves were also investigated. The frequency of atypical curve patterns from Group 1 to 2 was 46.2%, 45.2%, and 40.7% respectively. 65.3% patients with typical curve patterns had atypical features in all of the three groups. There was a significant difference of kyphotic angle among the three groups. Both the degree of cerebellar tonsillar descent and syrinx patterns had no correlation with the curve severity or the frequency of atypical curves. These results show that radiographic presentations, including atypical curve patterns, atypical features in typical curve patterns, and a normal to hyperkyphotic thoracic spine may suggest the need for preoperative MRI. There is no evidence to suggest that the degree of cerebellar tonsillar descent and syrinx patterns have an effect on the progress of scoliosis and the frequency of atypical curves.
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PMID:Radiologic presentations in relation to curve severity in scoliosis associated with syringomyelia. 1710 84

We explored the manifestations of an autosomal-recessive multisystemic disorder in several Saudi families. Recognized causes of progressive extra-pyramidal disorder and white matter disease were excluded and the neurological, imaging, endocrine, and skin manifestations of this syndrome described. The onset of these symptoms in these patients began in early adolescence and progressed more rapidly in males. All affected patients had total or partial alopecia, clinical and chemical evidence of hypogonadism (low levels of estradiol and testosterone); females had clear evidence of hypogonadism (streak or absent ovaries), and some patients had diabetes mellitus and/or sensorineural deafness. The constant biochemical abnormality was the low IGF-1. The neurological manifestations included moderate to severe intellectual decline and abnormality of muscle tone and posture with choreo-athetoid and dystonic movements resulting in gait difficulty, dysarthria, difficulty swallowing, and scoliosis. The MRI of brain demonstrated white matter involving cerebellum, brain stem, and cerebral structures, as well as abnormal decreased signal intensity in the basal ganglia with involvement of the substantia nigra. We conclude that the association of hypogonadism, alopecia, and persistent low IGF-1 is a significant autosomal recessive syndrome; it is prevalent in Saudi Arabia. We also demonstrate that the progressive extra-pyramidal disorder, white matter disease, and abnormal signals of the basal ganglia are common features of this syndrome. Sensorineural deafness and diabetes mellitus were recognized features.
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PMID:Autosomal-recessive syndrome with alopecia, hypogonadism, progressive extra-pyramidal disorder, white matter disease, sensory neural deafness, diabetes mellitus, and low IGF1. 1716 99

Radicular pain can be caused by disc herniation, lateral stenosis, isthmic spondylolisthesis with foraminal stenosis, or foraminal encroachment due to asymmetrical disc degeneration or scoliosis. Surgery is indicated following failure of conservative treatment. Minimally invasive discectomy is indicated for subjects presenting with radicular pain with or without neurological deficit and appropriate sized herniation in MRI. It offers equivalent efficacy but quicker recovery than microdiscectomy. Minimally invasive fusion is indicated for radicular pain due to foraminal compression in isthmic spondylolisthesis, asymmetric disc degeneration or scoliosis. It allows decrease in blood loss and postoperative pain. A less invasive technique should nevertheless not replace properly conducted conservative treatment.
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PMID:[Surgical treatment of radicular pain using minimally invasive techniques]. 1723 99

Several factors, such as immobilization, metabolic bone disease and immunosuppressive drugs, can compromise the quality of bone in children who have undergone solid organ transplantation. In contrast to adults, decreased bone mineral density has been reported in only a small proportion of pediatric transplant patients, and the relationship between low bone mineral density and fracture risk has not been established in children. Nevertheless, fractures, scoliosis, and joint and spinal degeneration are common in patients who received solid organ grafts as children. Avascular bone necrosis occurs infrequently in this patient population. Future studies should evaluate the effects of the underlying disease, transplantation and immunosuppression on the metabolism of bone and cartilage. On the basis of our own clinical experience and literature review, the growing spine of children who have received transplants should be continuously evaluated, and follow-up of bone mineral density is indicated. By contrast, routine MRI of the joints seems unnecessary.
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PMID:Therapy insight: orthopedic complications after solid organ transplantation in childhood. 1725 97

We describe a Japanese brother and sister with Martsolf syndrome. They had short stature, severe mental retardation, cataract, hypogonadism, craniofacial dysmorphism, and bone and joint symptoms including scoliosis, lax finger joints, and talipes valgus. Previously undescribed findings included proximal femoral epiphyseal dysplasia reminiscent of Legg-Calve-Perthes disease in both patients, and Klippel-Feil malformation and osteopathia striata in one patient. Brain MRI showed mild frontal and temporal lobe atrophy, and mild ventricular enlargement. Severe GH deficiency was demonstrated after insulin tolerance and glucagon/propranolol tolerance tests. No responses to serum LH and FSH after a gonadotropin-releasing hormone (GnRH) test suggested secondary hypogonadism, that is, hypogonadotropic hypogonadism, due to hypothalamus-pituitary axis insufficiency in both patients.
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PMID:Martsolf syndrome in Japanese siblings. 1739 1

Spondylitis/spondylodiscitis is still an uncommon diagnosis often with a delay in diagnosis and treatment due to the uncharacteristic symptoms. The aim of this study is to increase the awareness and outline a pattern of investigation and treatment. We present six children with an average age of 23 months (19-33 months) at time of diagnosis, conservative treated and with a mean follow-up of 31 months (12-65 months). The evaluation included past medical history, clinical symptoms, X-rays, MRI-investigations and laboratory studies [CRP, erythrocytes sedimentation rate (ESR), white blood count (WBC) and blood cultures] during the course of treatment and follow-up. The predominate clinical findings were: limp, refusal to walk and/or back pain. The mean duration of symptoms until presentation at our clinic was 24 days (4-42 days), the final diagnosis was set after an average of 12 days (7-14 days). Laboratory findings were unspecific but ESR best correlated with the clinical symptoms during the therapy. Five patients were treated by parenteral antibiotics for a minimum of 3 weeks, followed by oral antibiotics adapted to the clinical and laboratory findings. One child received a combined antituberculous chemotherapy after positive skin test for tbc. All six children were immobilized with a body-plaster-cast for an average time of 15 weeks (5-26 weeks). Four patients additionally were treated by further corset therapy for an average of 10 months (3-18 months). Radiological findings on plane X-rays (a.p. and lateral views) at time of diagnosis were decreased height of the disk space and erosions of adjacent vertebral endplates and residues of these radiological changes with signs of bony healing (sclerotic vertebral endplates or partial fusion) were seen at the latest follow-up. There was no case of instability or deformity like scoliosis or kyphosis. The MRI showed the earliest detectable typical vertebral bone involvements and confirmed the diagnosis in combination with laboratory findings and clinical symptoms. Spondylitis/spondylodiscitis should be considered as diagnosis in children with refusal to walk or gait disturbances especially in combination with elevated ESR. MRI is the tool of choice to set the diagnosis early. With an adequate and early therapy of bracing (body-plaster-cast), antibiotics and clinical monitoring good long-term result without spine instability or deformity can be achieved.
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PMID:Remodeling of the spine in spondylodiscitis of children at the age of 3 years or younger. 1741 Mar 70

Multi-minicore Disease (MmD) is a recessively inherited neuromuscular disorder characterized by multiple cores on muscle biopsy and clinical features of a congenital myopathy. Prevalence is unknown. Marked clinical variability corresponds to genetic heterogeneity: the most instantly recognizable classic phenotype characterized by spinal rigidity, early scoliosis and respiratory impairment is due to recessive mutations in the selenoprotein N (SEPN1) gene, whereas recessive mutations in the skeletal muscle ryanodine receptor (RYR1) gene have been associated with a wider range of clinical features comprising external ophthalmoplegia, distal weakness and wasting or predominant hip girdle involvement resembling central core disease (CCD). In the latter forms, there may also be a histopathologic continuum with CCD due to dominant RYR1 mutations, reflecting the common genetic background. Pathogenetic mechanisms of RYR1-related MmD are currently not well understood, but likely to involve altered excitability and/or changes in calcium homeoestasis; calcium-binding motifs within the selenoprotein N protein also suggest a possible role in calcium handling. The diagnosis of MmD is based on the presence of suggestive clinical features and multiple cores on muscle biopsy; muscle MRI may aid genetic testing as patterns of selective muscle involvement are distinct depending on the genetic background. Mutational analysis of the RYR1 or the SEPN1 gene may provide genetic confirmation of the diagnosis. Management is mainly supportive and has to address the risk of marked respiratory impairment in SEPN1-related MmD and the possibility of malignant hyperthermia susceptibility in RYR1-related forms. In the majority of patients, weakness is static or only slowly progressive, with the degree of respiratory impairment being the most important prognostic factor.
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PMID:Multi-minicore Disease. 1763 Oct 35

Background. Syryngomyelia was first described by Olliver in 1824. Burwell and Williams reported a 50% incidence of scoliosis in patients with syringomyelia. Malformations of the central nervous system among patients with scoliosis are very difficult to diagnose on the basis of clinical and radiological examination. MRI examination is a useful tool in diagnosing neurogenic malformations. The aim of this study is to evaluate the prevalence of neurogenic malformations in a group of patients with scoliosis, and the evaluation of shared clinical symptoms in this group. Material and methods. Clinical, radiological and MRI assessment was performed on a group of 323 patients referred to the Clinic due to scoliosis with a Cobb angle > 20. Results. Four patients were diagnosed with syringomyelia, 1 had a Chiari malformation type I and 1 female patient aged 14 was diagnosed with tethered cord. Conclusions. In the group, the prevalence of neurogenic malformation was 1.9%. MRI examination in patients with scoliosis seems to be justified in the following cases: clinical findings suggests a neurogenic background of spine deformation; scoliosis with onset before 8 years of age; prior to the planned surgical correction of scoliosis.
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PMID:Diagnosis of syringomyelia and Chiari malformations in patients with scoliosis. 1767 54

Little is known about the natural history of scoliosis found in patients with syringomyelia, including the factors affecting scoliosis curve progression and the effect of syrinx drainage treatment. Twenty patients having scoliosis with syringomyelia diagnosed by MRI were followed up for 6.6 (range 2.0-12.6) years on an average. Various factors potentially influencing curve pattern or progression in these patients were then retrospectively reviewed. The convex side of major curve of scoliosis tended to be on the same side as the syrinx and as the unilateral neurologic abnormality. No correlation was found between the location and the size of the syrinx and the location and size of the major curve of the scoliosis, or between the severity of neurologic deficit and the size of the major curve of the scoliosis. In patients under the age of ten at the time of diagnosis of scoliosis and with a flexible curve, decompression of the syrinx improved or stabilized scoliosis. In most patients over the age of ten, surgical treatment of the scoliosis was necessary because of the large initial size of the curve or progression of the curve even after syrinx drainage. Other factors including gender, location of the syrinx, type of the curve, and severity of neurologic deficits did not correlate with the progression of the curve. The results of this retrospective study suggest that early diagnosis and decompression of a syrinx in scoliosis patients especially under the age of ten is crucial and may decrease the curve size and limit scoliosis curve progression.
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PMID:Scoliosis associated with syringomyelia: analysis of MRI and curve progression. 1770 Dec 26


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