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PubMed1976

[Renal excretion of glycosaminoglycans under normal conditions and in several nervous system diseases in children].

The authors studied 101 children with diseases of the nervous system (with hereditary and acquired pathology) and 205 practically normal children. The urine excretion of specific components of the connective tissue (glycosaminoglycanes) was higher than in normals. Lesions of the connective tissue stroma in such forms of pathology is expressed not only in quantitative changes of glycosaminoglycane, but in their qualitative characteristics. Disturbances of the fractional compounds of glycosaminoglycane in the urine of patients differs from the parameters of chromatograms of normals by a high content of fractions of heparansulfate in a relatively low level of chondroethylsulfatolike fractions. In such states the severity of the clinical picture is accompanied by expressed metabolic disturbances.

Adolescent↗
PubMed2004

Update on neuroimaging in infectious central nervous system disease.

PURPOSE OF REVIEW: Neuroimaging constitutes an important component in the diagnosis of the underlying infectious agents in central nervous system infection. This review summarizes progress in the neuroimaging of infectious central nervous system disease since January 2003. It focuses on imaging of viral encephalitis, including that caused by exotic and emerging viruses, and on imaging in immunodeficient patients. RECENT FINDINGS: Diffusion-weighted imaging has been shown to be superior to conventional magnetic resonance imaging for the detection of early signal abnormalities in herpes simplex virus encephalitis but also in enterovirus 71 encephalitis and in West Nile encephalitis. Several studies defined the pattern of magnetic resonance imaging signal changes in endemic diseases such as West Nile encephalitis, Murray Valley encephalitis, enterovirus 71 encephalitis and Japanese encephalitis, but also in encephalitides due to ubiquitous viruses such as measles virus and Lyssavirus (rabies). In patients with HIV infection, apparent diffusion coefficient ratios obtained by diffusion-weighted imaging were significantly greater in lesions due to Toxoplasma encephalitis than in primary central nervous system lymphomas. SUMMARY: The diagnosis of unclear infectious central nervous system diseases remains a challenge. More recent magnetic resonance imaging techniques, such as diffusion-weighted imaging and magnetic resonance spectroscopy, provide additional helpful information. However, the mainstay of diagnosis remains the detection of viral DNA or serological markers of specific infectious agents within the cerebrospinal fluid.

Brain↗
PubMed1998

A close temporal relationship of liver disease to antiribosomal P0 protein antibodies and central nervous system disease in patients with systemic lupus erythematosus.

OBJECTIVE: To determine whether there is a close temporal relationship of liver disease to serum IgG and/or IgM antiribosomal P0 protein antibodies (anti-P0) and central nervous system (CNS) lupus in patients with systemic lupus erythematosus (SLE). METHODS: The study included 70 patients with active SLE. Of these, 30 had IgG and/or IgM anti-P0 and 14 had CNS lupus other than psychiatric disease (nonpsychiatric CNS lupus). Of these 14 patients, 11 had anti-P0. Laboratory manifestations of liver disease were retrospectively analyzed. RESULTS: Liver disease not attributed to any cause other than SLE (SLE liver disease) was present in 8 of the 11 patients with anti-P0 with nonpsychiatric CNS lupus (72.7%), in none of the 19 patients with anti-P0 without nonpsychiatric CNS lupus (0%), and in one of the 40 patients without anti-P0 (2.5%). The prevalence of SLE liver disease was significantly greater in patients with anti-P0 with nonpsychiatric CNS lupus than in the other 2 groups (p < 0.0001). Mean levels of liver enzymes (lactate dehydrogenase, glutamic oxaloacetic transaminase, glutamate pyruvate transaminase, gammaglutamyl transpeptidase) were significantly higher in patients with anti-P0 with nonpsychiatric CNS lupus than in the other 2 groups. Serial studies in 3 patients showed that the appearance of anti-P0 and liver dysfunction slightly preceded the onset of nonpsychiatric CNS lupus. CONCLUSION: Anti-P0 may be related to the pathogenesis of CNS lupus and SLE liver disease found simultaneously in SLE. The appearance of anti-P0 and liver dysfunction may predict onset of CNS lupus.

Adolescent↗
PubMedDate not supplied

[Nervous system diseases in workers of a large metallurgic plant].

Epidemiological investigations were carried out in an industrial plant in a population of about 16 000 people. The overall morbidity was 1 241.8 cases of nervous system diseases per 100 000 of population and the annual prevalence of neurological diseases was 473.7 per 100 000. The most frequent disease was sciatic pain, followed in order of frequency by epilepsy, vasomotor headaches, subjective symptoms after craniocerebral trauma, Parkinson's disease, clinically evident cerebral atherosclerosis and disseminated sclerosis. No significant effect of the type of occupation on the development of nervous system diseases was observed.

Adolescent↗
PubMed1996

Somatic gene therapy for nervous system disease.

Neurotrophic factors are target-derived molecules that prevent neuronal degeneration during development and, in some cases, during adulthood. They offer substantial promise as therapeutic agents in neurological disease by preventing cell loss and promoting axonal regeneration. However, the optimal means of delivering neurotrophic factors to the nervous system, and the CNS in particular, is an unresolved issue. Neurotrophic factors rarely influence only a single target neuronal population, hence broad delivery of neurotrophic factors to the nervous system may results in effects on multiple non-targeted neuronal populations. Ideally, neurotrophin delivery to the nervous system should be target-specific, regionally restricted, chronic, safe, well-tolerated and of sufficient concentration to elicit responses from target neurons. In this paper we discuss the use of somatic gene transfer methods to deliver neurotrophic factors to the CNS in a manner that seeks to meet the above criteria.

Animals↗
PubMed2001

[Georges Gilles of Tourette, pioneer of gait analysis in the nervous system diseases].

Georges Gilles de la Tourette's contribution to neurology goes beyond the description of the neurological disorder named after him. On December 28, 1885, he defended his doctoral thesis devoted to "gait in the diseases of the nervous system, studied by the method of imprints". In collaboration with Albert Londe, he worked for two years in Charcot's department on "a simple method applicable to both healthy and unhealthy patients", establishing the scientific and modern basis of functional exploration of human gait. The purpose was to "record the modifications of the gait and to fix them permanently, using suitable devises to guarantee not only rigorous comparison, but also to prevent observers, making use of the same method, from disputing or canceling the results completely independent of the experimenter himself". Georges Gilles de la Tourette defined the various characteristic parameters and provided the normal reference values in males and females, determining the physiological asymmetry of steps. He described spastic gait, shaking palsy, and locomotor ataxia. He distinguished between disorders of nervous control and related joint diseases. He also classified gait disorders occurring during hemiplegia. Modern development of kinetic, kinematic and biomechanical studies is a good illustration of the current relevancy of Gilles de la Tourette's contribution.

France↗
PubMed1975

Acquired toxoplasmosis. A neglected cause of treatable nervous system disease.

The neurological manifestations of six cases of acquired central nervous system toxoplasmosis are compared with the 39 well-documented cases from the literature. Half of the patients had underlying systemic diseases (18 malignant neoplasms, two renal transplants, three collagen vascular diseases) treated with intensive immunosuppressive therapy. The remainder had primary toxoplasmosis. Three major neurological patterns were seen: (1) diffuse encephalopathy with or without seizures, (2) meningoencephalitis, and (3) singular or multiple progressive mass lesions. Routine neurological diagnostic studies were not helpful. The Sabin-Feldman dye test or IgM indirect fluorescent antibody test or both were effective in confirming the diagnosis. Twenty-seven patients died without a clinical diagnosis of toxoplasmosis. The diagnosis was made terminally in four additional patients. Thirteen of fourteen patients who received a full course of sulfadiazine or pyrimethamine or both did well. Toxoplasmosis should be considered in the immunosuppressed patient who appears with neurological involvement.

Adult↗
PubMed1992

Nervous system disease, immunological features, and HLA phenotype in Sjögren's syndrome.

Twenty seven patients with primary or possible Sjögren's syndrome with neurological manifestations were compared immunologically with 21 patients with Sjögren's syndrome with an intact nervous system. Patients with Sjögren's syndrome were divided into seropositive and seronegative subgroups with respect to the occurrence of one or more autoantibodies (antinuclear antibodies, rheumatoid factor, antibodies to SS-B) in their serum samples. This study of 48 patients indicates that the spectrum of nervous system disease in seronegative and seropositive subgroups is almost indistinguishable. No significant differences were found in the occurrence of circulating immune complexes, the levels of serum complement C3 and C4, or serum IgA, IgM, and beta 2 microglobulin with respect to the neurological manifestations. The serum IgG level, however, was significantly higher in the patients with Sjögren's syndrome with intact nervous systems than in those with neurological manifestations. No significant association was found between the HLA phenotype and nervous system disease. The occurrence of HLA-B8 and DR3 antigens was, however, significantly higher in those patients with antibodies to SS-B than in those without. This finding supports the suggestion that HLA-B8/DR3 may modulate autoantibody responses rather than disease expression in Sjögren's syndrome.

Adult↗
PubMed1999

Melanocortins and the treatment of nervous system disease. Potential relevance to the skin?

For several decades melanocortins have been implicated in the modulation of brain function. More recently, this idea has been supported by the identification and cloning of melanocortin (MC) receptors in the nervous system. MCs stimulate axonal growth in fetal neural tissue or in neural cell lines in culture. This feature was utilized in screening their neurotrophic or neuroprotection potential in animal studies of nervous system disease (peripheral nerve and spinal cord trauma, toxic and metabolic neuropathies, EAN, EAE, etc.). Some of these effects may be mediated by MC4 receptor activation, although as yet unknown receptors may also be involved (for instance, protection by Org 2766). To what extent MC-nervous system effects are related to known effects of MCs in skin- and neuro-immune systems, remains to be discovered. Nevertheless, it is of interest to note that activation of brain MC4 receptors profoundly affects care behavior for the body surface (skin and fur). The excessive grooming response in rodents exhibits a remarkable functional correlation with MSH activity in a brain-skin axis.

Adrenocorticotropic Hormone↗