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PubMed2002

Nervous system Lyme disease.

Nervous system involvement in Lyme borreliosis is a much-feared, often-misunderstood disorder. Both the peripheral and central nervous systems may be involved, typically in a multifocal, patchy fashion, perhaps suggesting a vasocentric mechanism. Clinical manifestations vary widely, depending on the site and severity of involvement. Although neurologic manifestations observed in Europe differ somewhat from those reported in the United States, there are also striking similarities, permitting some generalization of information obtained in each population. In general, diagnosis of neurologic disease requires objective evidence of nervous system damage, and must be differentiated from both psychiatric disorders and metabolic encephalopathies, both of which typically occur in the absence of significant neurologic damage or infection. Laboratory confirmation of nervous system involvement by Borrelia burgdorferi has limitations. However, neurophysiologic testing of the peripheral nervous system, imaging of the neuraxis, and examination of the cerebrospinal fluid can all be informative. In contrast, to date functional brain imaging has been of limited specificity. Treatment with one of several straightforward antimicrobial regimens, typically 2-4 weeks in duration, generally results in microbiologic cure. Although some symptoms may persist after this, the data do not suggest that these are responsive to further antimicrobial therapy.

Europe↗
PubMed1988

Serum complement activation in central nervous system disease in Sjögren's syndrome.

PURPOSE: Central nervous system disease and vasculitis are extraglandular manifestations of Sjögren's syndrome. In our experience, central nervous system disease develops in approximately 70 percent of patients with Sjögren's syndrome and biopsy documented peripheral vasculitis. In order to further investigate the pathogenesis of central nervous system disease and its relationship to peripheral vasculitis in Sjögren's syndrome, we examined sera of patients with Sjögren's syndrome with and without focal central nervous system involvement for evidence of terminal complement pathway activation. PATIENTS AND METHODS: Patients were classified as having active focal central nervous system involvement only when they had focal neurologic deficits on physical examination, plus at least one abnormal neurodiagnostic test result. Two thirds of these patients also had cognitive or psychiatric dysfunction. Patients were classified as having peripheral vasculitis if they had clinical and histopathologic documentation of vascular inflammation. Serum SC5b-9 was measured by a sensitive enzyme-linked immunoabsorbent assay. Total hemolytic complement assay, measurement of serum C3 and C4 by radial immunodiffusion, and determination of immune complexes were performed. RESULTS: Fluid-phase terminal complement complexes (SC5b-9) were detected in the sera of 25 of 30 (83 percent) patients with focal central nervous system involvement, but in only seven of 21 (33 percent) patients with Sjögren's syndrome without focal central nervous system disease (p = 0.00084 by Yates' chi-square analysis). Four of these seven patients without focal central nervous system disease, but who had serum SC5b-9, had psychiatric or cognitive dysfunction. SC5b-9 was also detected in sera from 14 of 15 (93 percent) patients with active biopsy-documented peripheral vasculitis in contrast to 18 of 36 (50 percent) patients without clinical evidence of peripheral vasculitis (p = 0.0094). Serum SC5b-9 was a more sensitive indicator of complement activation than circulating immune complex or complement assays. CONCLUSION: These findings suggest that terminal complement activation may participate in the pathophysiology of both central nervous system and peripheral vasculitis in Sjögren's syndrome. Serum SC5b-9 appears to be a useful diagnostic indicator of vascular inflammation in Sjögren's syndrome and appears to identify those patients at risk for central nervous system complications.

Antigen-Antibody Complex↗
PubMed1995

[Use of immunoglobulins G in the treatment of nervous system diseases].

Short term and long term beneficial effects of IgG administration have been studied in 4 groups of neuro-immunological diseases: Polymyositis, dermatomyositis and myasthenia. Peripheral neuropathies (myelinopathies), primarily in Guillain-Barré syndrome and in chronic inflammatory polyneuropathy. Children intractable Epilepsy. Multiple Sclerosis. Actual results are in favor of an objective and marked improvement (after 1 or 2 months) in the first 2 groups using intravenous IgG. In M.S. patients, the short term treatment of bouts (especially: optic neuritis) seems promising. Results of the only one long term (later than 10 years) trial are in favor of a significant slowing of the progression of M.S. comparatively to the predictive curve of disease.

Epilepsy↗
PubMed2005

Central nervous system Lyme disease.

Nervous system infection with Borrelia burgdorferi frequently causes meningitis and rarely causes encephalomyelitis. Altered cognitive function also can occur in the absence of central nervous system infection. Recently developed serodiagnostic tools, such as the C6 assay, and appropriate use of Western blotting promise to improve diagnostic accuracy. Treatment trials have demonstrated the efficacy of relatively brief courses of oral antimicrobial agents, even in peripheral nervous system infection and meningitis. Several well-performed studies have clearly shown that prolonged antimicrobial treatment of "post-Lyme disease" is ineffective. Diagnosis and treatment of Lyme disease continue to improve.

Anti-Bacterial Agents↗
PubMed2004

Central Nervous System Lyme Disease.

Nervous system infection with Borrelia burgdorferi frequently causes meningitis and rarely causes encephalomyelitis. Altered cognitive function also can occur in the absence of central nervous system infection. Recently developed serodiagnostic tools, such as the C6 assay, and appropriate use of Western blotting, promise to improve diagnostic accuracy. Treatment trials have demonstrated the efficacy of relatively brief courses of oral antimicrobial agents, even in peripheral nervous system infection and meningitis. Several well-performed studies have clearly shown that prolonged antimicrobial treatment of "post-Lyme disease" is ineffective. Diagnosis and treatment of Lyme disease continue to improve.

Journal Article↗
PubMedDate not supplied

[Cerebrospinal fluid cAMP and cGMP in various peripheral and central nervous system diseases. Preliminary report].

In a group of 51 patients the concentrations of cAMP and cGMP and their ratio were determined in the cerebrospinal fluid in certain neurological diseases. Statistically significant differences were found in the values of the concentrations of the above metabolites in selected groups of neurological diseases. Generally speaking, apart from a group of multiple sclerosis cases the values of cAMP concentration were higher in central nervous system diseases than in peripheral nervous system diseases. The highest values of the cAMP/cGMP ratio were observed in the group of patients with inflammatory conditions of the central nervous system. This method of cerebrospinal fluid examination may have an important role in the diagnosis of neurological diseases, particularly in multiple sclerosis.

Adult↗
PubMed1978

[Nervous system diseases in workers of a large metallurgical plant. II. Impact of occupational factors].

Correlation between some factors connected with work performance in a big metallurgical plant, employing approximately 16000 persons, and the occurrence of nervous system diseases was analysed. It was found that among the most frequent diseases of nervous system cured in neurological dispensary of a big metallurgical plant are: ischias, epilepsy, syndrome of subjective ailments following a past cranio-cerebral trauma and vasomotor headaches. The studies performed did not reveal any clear correlation between the character and arduousness of work and the type of nervous system diseases occurring most frequently in a big industrial (metallurgical) plant. The prevalence of ischias is not significantly dependent on occupational factors. The possibility of continuation of work or necessity to stop working in result of a nervous system disease depend on the type of the disease.

Adult↗
PubMed2003

Abnormal hyperintensity within the subarachnoid space evaluated by fluid-attenuated inversion-recovery MR imaging: a spectrum of central nervous system diseases.

A variety of central nervous system (CNS) diseases are associated with abnormal hyperintensity within the subarachnoid space (SAS) by fluid-attenuated inversion-recovery (FLAIR) MR imaging. Careful attention to the SAS can provide additional useful information that may not be available with conventional MR sequences. The purpose of this article is to provide a pictorial essay about CNS diseases and FLAIR images with abnormal hyperintensity within the SAS. We present several CNS diseases including subarachnoid hemorrhage, meningitis, leptomeningeal metastases, acute infarction, and severe arterial occlusive diseases such as moya-moya disease. We also review miscellaneous diseases or normal conditions that may exhibit cerebrospinal fluid hyperintensity on FLAIR images. Although the detection of abnormal hyperintensity suggests the underlying CNS diseases and narrows differential diagnoses, FLAIR imaging sometimes presents artifactual hyperintensity within the SAS that can cause the misinterpretation of normal SAS as pathologic conditions; therefore, radiologists should be familiar with such artifactual conditions as well as pathologic conditions shown as hyperintensity by FLAIR images. This knowledge is helpful in establishing the correct diagnosis.

Adult↗
PubMed1996

[Gene therapy of nervous system diseases in children].

Gene therapy seems very promising for numerous diseases of the developing nervous system of infants. The efficacy of such an approach relies on different parameters: 1) choice of a target gene; 2) selection of a promoter permitting a robust and protracted expression of the delivered gene in the target cells; 3) selection of a vector permitting to deliver the gene to the right cells with efficacy and without side-effects; 4) choice of the way to administer the vector to the target cells. Although experimental and methodological tools have greatly improved during the last decade, gene therapy of child nervous system diseases is still in the field of basic research.

Brain Neoplasms↗
PubMed1986

Primary Sjögren's syndrome with central nervous system disease mimicking multiple sclerosis.

Central nervous system involvement has occurred in approximately 20% of patients with primary Sjögren's syndrome evaluated at our institution. Characteristically, the neurologic dysfunction is multifocal, involving both the brain and spinal cord, and is recurrent over time. We present the features of 20 patients with primary Sjögren's syndrome and central nervous system involvement whose neurologic findings, evoked potential abnormalities, and cerebrospinal fluid profiles (elevated IgG indices, oligoclonal bands on agarose gel electrophoresis, and mild pleocytosis with reactive lymphoid cells) closely resembled those of multiple sclerosis. In fact, multiple sclerosis was considered the most likely diagnosis in each of these patients before diagnosis of Sjögren's syndrome, and each patient met criteria for definite multiple sclerosis. The clinical effects of corticosteroid treatment during episodes of acute neurologic dysfunction appeared to be beneficial in these patients.

Adult↗
PubMedDate not supplied

Neuroblastoma presenting as central nervous system disease.

Neuroblastoma may be extremely difficult to recognize, particularly when the tumor presents as a primary central nervous system disease. Central nervous system involvement may be considered as primary intracerebral neuroblastoma, metastases to the cranium from an occult primary, primary intraorbital neuroblastoma originating in the ciliary ganglion, metastatic intraorbital neuroblastoma from an occult primary, primary intraspinal neuroblastoma originating in dorsal root ganglia, intraspinal metastatic disease, and distant effects such as myoclonic encephalopathy. Primary neuroblastoma within the ciliary ganglion and primary intraspinal neuroblastoma are extremely rare entities. Illustrative cases the demonstrate the broad spectrum of neurologic presentations are offered. The second known report of neuroblastoma in association with primary pulmonary hypoventilation (Ondine curse) is included.

Brain Neoplasms↗