Cases reported "Nervous System Diseases"

Filter by keywords:



Filtering documents. Please wait...

1/10. Cord monitoring changes and segmental vessel ligation in the "at risk" cord during anterior spinal deformity surgery.

    STUDY DESIGN: Retrospective analysis of all cases of anterior spinal deformity surgery that had intraoperative spinal cord monitoring (somatosensory-evoked potentials, SSEPs). OBJECTIVES: The prime purpose of this study was to determine the incidence of significant SSEP changes in patients undergoing anterior spinal deformity surgery. A secondary objective was to ascertain if patients with "cords at risk" were more likely to produce significant intraoperative SSEP changes and what proportion of these changes resulted in postoperative neurological deficit. SUMMARY OF BACKGROUND DATA: There is anecdotal evidence to suggest that patients with intraspinal abnormalities are at greater risk of postoperative neurological deficit after spinal deformity surgery. To date, there have been no studies detailing this risk and how it relates to the type of surgery performed. This is a question of increasing relevance with the modern trend towards more anterior scoliosis correction and instrumentation. Recent reports have suggested a low incidence of neurological complication with anterior deformity procedures. There is controversy as to whether SSEP monitoring is required for these anterior procedures and whether soft clamping of segmental vessels before their division is a necessary precaution. METHOD: This study is a chart review of all patients who had an anterior deformity operation between 1990 and 2001. Those patients who had a complete data set (preoperative magnetic resonance imaging scan, patient and procedural documentation, and adequate intraoperative SSEP traces) were included in this study. A significant SSEP change was correlated with the type of procedure performed, whether that patient had a "cord at risk" (CAR) and the degree of postoperative neurological deficit if present. RESULTS: During the study period, 871 patients underwent elective anterior spinal deformity surgery. Ninety five (11%) patients had intraspinal abnormalities on magnetic resonance imaging. From this group, 27 (3%) were termed CAR. Twenty six (3%) patients had significant change in the intraoperative SSEP monitoring. Seventeen (2% total) occurred in the CAR group and nine (1% of total) in the normal cord group. There were five patients (0.6%) with significant postoperative neurological deficits, four (0.5%) in the CAR group, and one (0.1%) in the normal cord group. These patients had also demonstrated changes in their SSEPs. The sensitivity of SSEP monitoring for the whole series was 100%, specificity 97.5%, the positive predictive value was 19% and the negative predictive value was 100%. The CAR group was significantly more likely to have significant SSEP changes during any operation and was more likely to have postoperative paresis. CONCLUSION: patients with identified cords at risk should undergo spinal cord monitoring (SSEP) if they undergo anterior spinal deformity surgery. Soft clamping of segmental vessels is indicated with cord monitoring to prevent the risk of postoperative neurological sequelae.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

2/10. Molecular characterization of a t(2;6) balanced translocation that is associated with a complex phenotype and leads to truncation of the TCBA1 gene.

    The molecular characterization of balanced chromosomal rearrangements has often been a powerful tool for the positional identification of genes associated with specific diseases. In some instances, these rearrangements may be associated with a variety of different phenotypes, and thus establishing a genotype-phenotype correlation may be a complex process. However, molecular characterization of the rearrangement remains a useful tool for diagnoses or prognoses, or for identifying new genes and establishing a gene-to-function relationship. In this work we describe the characterization of a de novo balanced translocation t(2;6)(q24.3;q22.31) found in a patient with a complex phenotype. The major clinical finding was a severe neurological involvement. Thanks to the molecular characterization of this translocation we found that the rearrangement led to the truncation of the TCBA1 gene on chromosome 6q. We found that the gene is transcribed in different splice variants and is highly specific for the central nervous system. TCBA1 does not show any similarity with other known genes, and no information is available about its function. However, the gene appears to be well conserved among species, and we were able to infer the sequence of a putative mouse homolog of TCBA1. This allowed us to perform a more detailed expression study in mice, thus confirming its specificity for the nervous system. This finding is of particular interest because it suggests that TCBA1 may be correlated with the neurological phenotype of our patient, and possibly mutated in genetic diseases with a neurological phenotype.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

3/10. Minipolymyoclonus in congenital nemaline myopathy: a nonspecific clinical marker of neurogenic dysfunction.

    The authors report a seven-month-old boy with severe hypotonia, poor spontaneous movements, breathing difficulties and recurrent respiratory infections, dysmorphisms and a peculiar movement disorder: minipolymyoclonus (MPM), previously reported only in spinal muscular atrophies. MPM is characterized by nonrhythmic myoclonic jerks associated with a rhythmic tremor of the extended fingers polygraphically detected. A muscle biopsy showed pathological changes typical of congenital nemaline myopathy (CNM). The relationship between MPM and CNM may be explained on the presumptive basis of the "neurogenic" nature of this congenital myopathy or by the non-specificity of this clinical sign.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

4/10. Lewy body prevalence in the aging brain: relationship to neuropsychiatric disorders, Alzheimer-type pathology and catecholaminergic nuclei.

    In a survey to determine the occurrence of Levy bodies in the elderly, the prevalence rate of Lewy body formation was found to be critically dependent on the psychiatric status of control cases. In 131 controls between 51 and 100 years screened to exclude psychiatric and neurological disorders, the Lewy body prevalence rate was 2.3%, but inclusion of cases with psychiatric disorders other than Alzheimer's disease increased the prevalence rate to 9%. An age-related decline in substantia nigra and locus coeruleus neuron numbers was observed in the control group. brain stem Lewy body formation (found in 3 cases) was not necessarily linked with neuron loss in substantia nigra, though in two of the cases significant locus coeruleus neuron loss was observed. Within the control group, there was no obvious relationship of Lewy body formation to the extent of Alzheimer-type pathology. These findings are compatible with the disease specificity of lewy bodies and suggest that Lewy body disorders have a relatively short preclinical phase in which Lewy body formation may precede both locus coeruleus and substantia nigra neuron loss. The increase of Lewy body positive cases found when individuals with psychiatric disorders are included in the population surveyed supports the emerging concept of a spectrum of Lewy body diseases ranging from purely psychiatric disorders through combined psychoneurological or neuropsychiatric symptoms, to the classically described neurological disorders of Parkinson's disease.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

5/10. Identification and characterization of gangliosides reacting with IgM paraproteins in three patients with neuropathy associated with biclonal gammopathy.

    IgM monoclonal antibodies from three patients with polyneuropathy associated with biclonal gammopathy reacted with monosialoganglioside GM1 on thin-layer chromatograms. An IgM paraprotein in one of the patients with a predominantly motor neuropathy also reacted strongly with the ganglioside GD1b and asialo-GM1. All three of these antigenic lipids have a Gal(beta 1-3)GalNAc moiety in common which would appear to be the antigenic determinant. However, this IgM also cross-reacted weakly with paragloboside which has an N-acetyllactosaminyl [Gal(beta 1-4)GlcNAc] terminal structure. The specificity of the other paraprotein in this patient is not known. The IgM paraproteins reacting with GM1 in both of the other patients exhibited different specificity because they did not react with GD1b and asialo-GM1, but reacted strongly with GM2 ganglioside. The data suggest that the epitope for both of these IgMs is in the GalNAc(beta 1-4)(NeuAc alpha 2-3)Gal(beta 1-4)Glc region of the gangliosides that is common to both GM2 and GM1. The second IgM paraproteins in both of these latter patients react with the myelin-associated glycoprotein (MAG) and two 3-sulfoglucuronyl glycolipids that share antigenic determinants with MAG.
- - - - - - - - - -
ranking = 2
keywords = specificity
(Clic here for more details about this article)

6/10. Quantification of thermal asymmetry. Part 2: Application in low-back pain and sciatica.

    temperature differences between the lower extremities were measured using a computerized thermometric scanning system in order to compare the degree of thermal asymmetry in 144 patients with low-back pain. The patients displayed highly significant thermal asymmetries, with the involved limb being cooler (p less than 0.001). When asymmetries exceeded 1 standard deviation from the mean temperature of homologous regions measured in 90 normal control subjects, the positive predictive value of thermometry in detecting root impingement was 94.7% and the specificity was 87.5%. These values indicate that calculation of temperature asymmetry is particularly effective in evaluating reported pain in psychosocially affected patient populations in whom the chance of positive myelography or impaired root function is low. In this group of patients, thermometric study provides physicians with important information for proper decision making. The test can be performed to avoid more invasive and probably less revealing diagnostic or exploratory surgical procedures.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

7/10. Importance of laboratory clinical investigation in the diagnosis of immune vasculitis with neurological manifestation.

    In this study 23 cases of immune vasculitis with predominance of neurological symptoms were analysed. Besides patient history and neurological examination, myelotomy is the most relevant means of diagnosis in immune vasculitis. For this reason myelotomy should be integrated in routine diagnostic methods if the diagnosis immune vasculitis is discussed. Other laboratory parameters have no great relevance in the diagnosis of immune vasculitis. A higher specificity of antibody tests, especially in the case of ANA, might be able to replace myelotomy in the diagnosis of immune vasculitis. The relevance of laboratory parameters in the diagnosis of immune vasculitis in neurological patients was examined.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

8/10. Loss of somatosensory evoked potentials during intramedullary spinal cord surgery predicts postoperative neurologic deficits in motor function [corrected]

    STUDY OBJECTIVES: To estimate the sensitivity and specificity of somatosensory evoked potentials (SSEPs) for predicting new postoperative motor neurologic deficits during intramedullary spinal cord surgery; to establish whether SSEPs more accurately predicted postoperative deficits in position and vibration sense than in strength. DESIGN: Prospective open and retrospective study. SETTING: University-affiliated hospital. patients: 20 patients with intramedullary spinal cord tumors scheduled for surgery with intraoperative SSEPs. INTERVENTIONS: Median, ulnar, and tibial nerve cortical and subcortical SSEPs were recorded continuously. MEASUREMENTS AND MAIN RESULTS: Conventional intraoperative SSEP criteria considered indicative of neurologic injury were modified and defined as either the complete and permanent loss of the SSEP or the simultaneous amplitude reduction of 50% or greater in the nearest recording electrode rostral to the surgical site and 0.5 millisecond increase in the central latency. Our definition required confirmation of both amplitude and latency changes on a repeated average. All patients had 1 or more SSEPs, which were reproducible and sufficiently stable for analysis throughout the operation. Six patients developed new postoperative neurologic deficits. One had new motor deficits in an extremity from which no baseline SSEPs could be elicited. In each of the other 5 patients, significant SSEP changes preceded the postoperative motor deficits in the extremity or extremities monitored. In no patient without a new postoperative motor deficit was there a significant change in the SSEP. In only 2 of these 5 patients was there a documented postoperative loss or diminution in vibration or position sense. CONCLUSIONS: Intraoperative SSEP changes during intramedullary spinal cord surgery are a sensitive predictor of new postoperative motor deficits, but such changes may not correlate reliably with postoperative deficits in position or vibration sense. In this setting SSEP monitoring serves primarily to reassure the operating team that, when the SSEPs remain constant, the surgery has not caused additional injury.
- - - - - - - - - -
ranking = 1
keywords = specificity
(Clic here for more details about this article)

9/10. Factors defining target specificity in antibody-mediated neuropathy: density-dependent binding of anti-GD1a polyclonal IgG from a neurological patient.

    IgM and IgG antibodies reacting with components of human brain gangliosides were detected in a patient bearing severe sensory ataxy. Using different chemical and immunological methods, the antigen was identified as the GD1a ganglioside. The antibodies showed antigen "density-dependent" binding, a property only observed in tumor-specific monoclonal antibodies. The relevance of this result in regard with target specificity of neuropathy-associated antibodies directed to ubiquitous glycolipids is discussed.
- - - - - - - - - -
ranking = 5
keywords = specificity
(Clic here for more details about this article)

10/10. Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes.

    BACKGROUND: autoantibodies specific for the acetylcholine receptor (AChR) of skeletal muscle (containing the alpha1 subunit) impair neuromuscular transmission in myasthenia gravis (MG). AChRs mediating fast synaptic transmission through autonomic ganglia are structurally similar to muscle AChR, but contain the alpha3 subunit. We propose that ganglionic AChR autoimmunity may cause dysautonomia. OBJECTIVE: To test serum of patients with autonomic neuropathy for autoantibodies of neuronal ganglionic AChR specificity. methods: We developed an immunoprecipitation radioassay by complexing epibatidine (125I-labeled high affinity agonist) to a Triton X-100-solubilized AChR antigen from peripheral neuroblastoma membranes. Monoclonal rat immunoglobulins (IgG) specific for muscle or neuronal AChRs validated the assay's specificity. We tested serum from 52 healthy subjects, 12 patients with subacute autonomic neuropathy, and 248 patients with other neurologic disorders. RESULTS: Twelve patients had antibodies that bound unequivocally to ganglionic AChR. Five had subacute autonomic neuropathy, and three (of six tested) had Isaacs' syndrome; four of these eight had a carcinoma (lung, bladder, rectum, thyroid). The remaining four seropositive patients (two Lambert-Eaton syndrome, one dementia, one sensory neuronopathy) all had Ca2 channel antibodies and three had small cell lung carcinoma. No healthy subject had ganglionic AChR antibodies, nor did 62 patients with MG and muscle AChR antibodies. CONCLUSION: Neuronal AChR antibodies are a novel serologic marker of neurologic autoimmunity. The pathogenicity of neuronal AChR autoantibodies in autonomic neuropathy, Isaacs' syndrome, or other neurologic disorders remains to be shown, as has been demonstrated for muscle AChR antibodies in MG. An autoimmune and potentially paraneoplastic etiology is implicated in seropositive patients.
- - - - - - - - - -
ranking = 2
keywords = specificity
(Clic here for more details about this article)
| Next ->


Leave a message about 'Nervous System Diseases'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.