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At least 307 records · Page 17Linked to original sources
PubMed2006

Dystonic movement disorders and spinal degenerative disease.

The occurrence of degenerative spinal disease subsequent to dystonic movement disorders has been neglected and has received more attention only recently. Spinal surgery is challenging with regard to continuous mechanical stress when treatment of the underlying movement disorder is insufficient. To characterize better the particular features of degenerative spinal disease in patients with dystonia and to analyze operative strategies, we reviewed the available published data. Epidemiologic studies reveal that degenerative spinal disorders in patients with dystonia and choreoathetosis occur much earlier than in the physiological aging process. Dystonic movement disorders more often affect the spine at higher cervical levels (C(2-5)), in contrast to spinal degeneration with age which manifests more frequently at the middle and lower cervical spine (C(5-7)). Degenerative changes of the cervical spine are more likely to occur on the side where the chin is rotated or tilted to. Various operative approaches for treatment of spinal pathologies have been advocated in patients with dystonic movement disorders. The available data do not allow making firm statements regarding the superiority of one approach over the other. Posterior approaches were first used for decompression, but additional anterior fusion became necessary in many instances. Anterior approaches with or without instrumented fusion yielded more favorable results, but drawbacks are pseudarthrosis and adjacent-level disease. Parallel to the development of posterior fusion techniques, circumferential surgery was suggested to provide a maximum degree of cord decompression and a higher fusion rate. Perioperative local injections of botulinum toxin were used initially to enhance patient comfort with halo immobilization, but they are also applied in patients without external fixation nowadays. Treatment algorithms directed at the underlying movement disorder itself, taking advantage of new techniques of functional neurosurgery, combined with spinal surgery have recently been introduced and show promising results.

Dystonic Disorders↗
PubMed2005

A meta-analysis of surgery versus conventional radiotherapy for the treatment of metastatic spinal epidural disease.

Radiotherapy has been the primary therapy for managing metastatic spinal disease; however, surgery that decompresses the spinal cord circumferentially, followed by reconstruction and immediate stabilization, has also proven effective. We provide a quantitative comparison between the "new" surgery and radiotherapy, based on articles that report on ambulatory status before and after treatment, age, sex, primary neoplasm pathology, and spinal disease distribution. Ambulation was categorized as "success" or "rescue" (proportion of patients ambulatory after treatment and proportion regaining ambulatory function, respectively). Secondary outcomes were also analyzed. We calculated cumulative success and rescue rates for our ambulatory measurements and quantified heterogeneity using a mixed-effects model. We investigated the source of the heterogeneity in both a univariate and multivariate manner with a meta-regression model. Our analysis included data from 24 surgical articles (999 patients) and 4 radiation articles (543 patients), mostly uncontrolled cohort studies (Class III). Surgical patients were 1.3 times more likely to be ambulatory after treatment and twice as likely to regain ambulatory function. Overall ambulatory success rates for surgery and radiation were 85% and 64%, respectively. Primary pathology was the principal factor determining survival. We present the first known formal meta-analysis using data from nonrandomized clinical studies. Although we attempted to control for imbalances between the surgical and radiation groups, significant heterogeneity undoubtedly still exists. Nonetheless, we believe the differences in the outcomes indicate a true difference resulting from treatment. We conclude that surgery should usually be the primary treatment with radiation given as adjuvant therapy. Neurologic status, overall health, extent of disease (spinal and extraspinal), and primary pathology all impact proper treatment selection.

Aged↗
PubMed1995

Symptomatic spinal epidural lipomatosis in a patient with Cushing's disease.

Spinal epidural lipomatosis (SEL) is a rare complication of iatrogenic Cushing's syndrome (CS). There is only one case reported of symptomatic SEL in association with endogenous CS. We present a patient with compressive myeloradiculopathy due to SEL and Cushing's disease and suggest that in SEL of hypercortisolism, the excess of corticosteroids is the stimulus for the growth of adipose tissue in the spinal canal and that treatment should be aimed at correcting the endocrine abnormality.

Adult↗
PubMed2002

Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1.

Deletion or mutation of the SMN1 (survival of motor neurons) gene causes the common, fatal neuromuscular disease spinal muscular atrophy. The SMN protein is important in small nuclear ribonucleoprotein (snRNP) assembly and interacts with snRNP proteins via arginine/glycine-rich domains. Recently, SMN was also found to interact with core protein components of the two major families of small nucleolar RNPs, fibrillarin and GAR1, suggesting that SMN may also function in the assembly of small nucleolar RNPs. Here we present results that indicate that the interaction of SMN with GAR1 is mediated by the Tudor domain of SMN. Single point mutations within the Tudor domain, including a spinal muscular atrophy patient mutation, impair the interaction of SMN with GAR1. Furthermore, we find that either of the two arginine/glycine-rich domains of GAR1 can provide for interaction with SMN, but removal of both results in loss of the interaction. Finally, we have found that unlike the interaction of SMN with the Sm snRNP proteins, interaction with GAR1 and fibrillarin is not enhanced by arginine dimethylation. Our results argue against post-translational arginine dimethylation as a general requirement for SMN recognition of proteins bearing arginine/glycine-rich domains.

Amino Acid Substitution↗
PubMed2001

Dysfunctional elimination syndrome in children without obvious spinal cord diseases.

Elimination dysfunction is a functional disease that encompasses the urinary and GI tracts. Because the presentation is varied and ED is a disease of exclusion, a thorough history and physical examination are mandatory. Noninvasive management that includes the "fundamental four" and continuous engagement and encouragement of the patient and family are the pillars of treatment. For patients who are refractory to this approach, new research in children-directed biofeedback research shows promise.

Biofeedback, Psychology↗
PubMed2006

Spinal cord stimulation for refractory angina pectoris and peripheral vascular disease.

Spinal cord stimulation has been used in clinical practice for more than three decades. The primary use of this therapy has been in spine-related disorders. In recent years, the therapy has been used more extensively in diseases of the vascular system. Increasingly, interest has piqued in using this mode of treatment for refractory angina and ischemic pain secondary to peripheral vascular disease. In this publication, we review the current literature on these two indications and present case examples of both therapies.

Angina Pectoris↗
PubMed1989

Our experience with spinal cord stimulation (SCS) in peripheral vascular disease.

Spinal cord stimulation (SCS) by epidural electrodes is being used more often in the treatment of patients with severe intractable ischemic pain. The promising clinical results and the objective increase in lower extremity blood flow (plethysmography, thelethermography, etc.), suggests that spinal cord stimulation may have an important role in the management of advanced arterial disease when other forms of treatment have failed. The selection criteria for implantation of SCS are very important. Our indications in patients with peripheral vascular disease are: 1. Peripheral vascular disease with severe, intractable symptoms that are untreatable by medical or surgical therapy. 2. Inflammatory or diabetic arterial disease resistant to medical treatment and/or sympathectomy. 3. Persistent and severe ischemic pain and/or ulcers in patients with patent grafts. 4. Patients for lumbar sympathectomy who have a high myocardial risk. Contraindications to implantation of SCS are claudication intermittent, large necrotic lesions (gangrene) and patients who have undergone prolonged narcotic therapy.

Adult↗
PubMed1985

Magnetic resonance imaging in the diagnosis of spinal cord diseases.

Experience with magnetic resonance imaging in 22 patients with diseases of the spinal cord is reported. Important additional diagnostic information as compared to conventional neuroradiological techniques (myelography, spinal CT) was gained especially in cases of hydrosyringomyelia, intraspinal tumour and multiple sclerosis. It is suggested that magnetic resonance imaging may become the method of choice in the diagnosis of structural spinal cord diseases.

Adult↗
PubMed1992

Repair and regeneration: experimental aspects of spinal cord disease.

Regeneration of spinal cord neurons after injury depends on first, the presence of trophic factor(s) which prevent death, induce growth and maintain the viability of the neurone and second, the regenerating neurites must overcome glial scars, extracellular matrices and white matter barriers to reinnervate their target. Attempts at promoting regeneration by using grafts of prosthetic material, Schwann cells and neural tissue have largely been unsuccessful. Although growth could be induced, penetration of regenerating neurites through damaged areas of the spinal cord has been limited. There is as yet very little beneficial functional recovery.

Animals↗
PubMed2006

Atypical presentations of spinal muscular atrophy type III (Kugelberg-Welander disease).

Spinal muscular atrophy type III (SMA III, Kugelberg-Welander disease) typically presents with symmetric proximal weakness, areflexia, and hypotonia. We present four children with spinal muscular atrophy type III who had atypical phenotypes. Three patients clearly had asymmetric weakness at presentation and two had upper motor neuron signs in the lower extremities (one patient had both features). Two of the patients had prolonged evaluations before the diagnosis was made. All patients had Gowers signs and two had pes planus. In patients with proximal muscle weakness the presence of asymmetrical weakness, upper motor neuron signs, or both, may be compatible with spinal muscular atrophy type III. The diagnosis of spinal muscular atrophy should be considered when other possibilities have been excluded.

Brain↗
PubMed1993

Myelography in the assessment of children with medulloblastoma.

Forty-seven out of 49 patients with a histologically-proven diagnosis of medulloblastoma admitted to our institution between 1 January 1984 and 13 December 1990 were examined post-operatively using either myelography or gadolinium-enhanced magnetic resonance imaging in an attempt to detect clinically occult spinal metastatic disease. Spinal spread of the tumour was identified in nine (19%) patients. Of the 16 patients for whom a 5-year follow-up has been available 12 had no myelographic evidence of spinal disease and of these 10 (83%) remain disease-free. Of the six who have died during this period four had evidence of spinal metastases at presentation. Of all the children in the series with spinal spread identified at the time of their initial hospitalization, only one survives to date (15 months after diagnosis). The remainder had an average life expectancy of 18 months.

Cerebellar Neoplasms↗
PubMed2003

Treatment of metastatic spinal epidural disease: a review of the literature.

OBJECT: Spinal cord compression is one of the most dreaded complications of metastatic cancer. It can lead to a number of sequelae, including pain, spinal instability, neurological deficits, and a reduction in the patient's quality of life. Except in selected circumstances, treatment is palliative. Treatment options include surgery, radiation, and chemotherapy. The goal of this study was to summarize the existing data on the outcomes of various treatment methods for metastatic spinal epidural disease and to make appropriate recommendations for their use. METHODS: The authors used a search strategy that included an electronic database search, a manual search of journals, analysis of bibliographies in relevant review papers, and consultation with the senior author. There is good evidence, including Class I data, that steroid drugs constitute a beneficial adjunctive therapy in patients with myelopathy from epidural compression. Historically, conventional radiation therapy has been viewed as the first-line treatment because it has been shown to be as effective as a decompressive laminectomy, with a lower incidence of complications (Class II data). Nevertheless, in the last 20 years there has been remarkable progress in surgical techniques and technology. Currently, the goals of surgery are to achieve a circumferential decompression of the spinal cord, and to reconstruct and immediately stabilize the spinal column. Results in a large body of literature support the belief that surgery is better at retaining or regaining neurological function than radiation and that surgery is highly effective in relieving pain. Most of the data on the treatment of metastatic spinal disease are Class II or III, but the preliminary results of a well-designed, randomized controlled trial in which surgery is compared with standard radiation therapy represents the first Class I data. CONCLUSIONS: As the number of treatment options for metastatic spinal disease has grown, it has become clear that effective implementation of these treatments can only be achieved by a multidisciplinary approach.

Adrenal Cortex Hormones↗
PubMed1979

Spinal fluid aluminum levels in patients with Alzheimer disease.

Spinal fluid from 180 male patients was examined for aluminum levels. Values were coordinated for age and protein determinations. In addition, values were listed according to the patient's diagnosis. Although in most instances no correlation could be made, 10 patients with Alzheimer disease showed significantly low levels of aluminum.

Adult↗