Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the very few previous investigations of the CSF-proteins in muscular dystrophies the results have generally been reported as normal. In spinal muscular atrophies a barrier-damage pattern of CSF-proteins has been found in amyotrophic lateral sclerosis (ALS). In the present investigation the CSF-proteins were examined by isoelectric focusing and quantitative paper electrophoresis in 13 patients with muscular dystrophies and in 11 patients with spinal muscular atrophies. On isoelectric focusing, CSF-protein abnormalities were found in 85% of the cases with muscular dystrophies and in all patients with spinal muscular atrophies. Differences in the CSF-protein patterns were observed within the group of muscular dystrophies and between these and the cases of spinal muscular atrophies. In ALS and in myotonic dystrophy, abnormal CSF-protein fractions occurred mainly in the alkaline pH-range, while in limb-girdle dystrophy and the patient with facioscapulohumeral dystrophy, aberrant fractions appeared mainly in the acidic region. CSF-protein abnormalities were found in both the alkaline fractions (HAFs) with pI 9.2-9.6 and a fraction with PI 7.1 were found in half of the patients with myotonic dystrophy. The CSF electrophoresis in myotonic dystrophy showed increased levels of beta1-globulin in all cases examined. Signs of barrier-damage were commonly encountered in ALS in contrast to the muscular dystrophies, except for myotonic dystrophy. The results are discussed in terms of possible diagnostic value and with regard to pathogenetic significance, particularly in relation to the current hypothesis of a neural involvement in muscular disorders.
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PMID:Isoelectric focusing and electrophoresis of cerebrospinal fluid proteins in muscular dystrophies and spinal muscular atrophies. 5 65

An atypical case of amyotrophic lateral sclerosis (ALS) is described, characterized by early manifestation, a long lasting course with asymmetry of the lesions, absence of bulbar symptoms in the presence of an otherwise very advanced symptomatology, and constant signs of an inflammatory reaction in the CSF which was the reason to initiate extensive virological studies, including procedures for virus isolation. A virus belonging to the TbE complex of arbovirus group B (tick-borne flavivures), was finally isolated from the CSF. About 70% of the ALS cases in Hamburg/W. Germany, examined for antibodies, apparently had contact with this virus. The antibody pattern found made it possible to explain this exceptional case.
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PMID:An uncommon case of amyotrophic lateral sclerosis with isolation of a virus from the CSF. 5 28

A virus isolated from the CSF of a patient who had amyotrophic lateral sclerosis for 7 years, and prolonged pleocytosis in the CSF, was adapted to suckling mouse brain by subsequent serial blind passages. This Schu virus belongs to the tick-borne encephalitis complex of the genus Flavivirus (Togaviridae). Suckling mouse brain homogenate of the 13th passage was used for transmission experiments in various species of laboratory animals. Golden hamsters infected subcutaneously fell ill after a number of months, lost weight, and had paresis of the legs. Histologically they had petechial hemorrhages in different parts of the CNS and inflammatory changes in the gray substance of the spinal cord. Pilot studies with repeated inoculations of small doses of different flavivirus strains suggest a course of the disease in experimental animals which resembles slow-virus infections insofar as no encephalitis is produced and degenerative changes of the anterior horn cells prevail over inflammatory signs in the spinal cord. After intracerebral application of Schu virus, cynomolgus monkeys developed the typical lesions of togavirus panencephalitis with epileptic seizures, ataxia, and paresis. After subcutaneous application, the virus seems to spread along peripheral nerves to anterior spinal roots and spinal cord, where mainly motor neurons of the anterior horn are damaged, and from there to the brain. The histological findings are such that one may assume the disease of the patient was due to the infection with the virus isolated from his CSF. Therefore, the hypothesis may be advanced that at least some of the cases diagnosed as amyotrophic lateral sclerosis are due to a togavirus infection.
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PMID:Attempts to reproduce amyotrophic lateral sclerosis in laboratory animals by inoculation of Schu virus isolated from a patient with apparent amyotrophic lateral sclerosis. 8 63

Serum and CSF from 48 patients with amyotrophic lateral sclerosis and six patients with late-onset postpoliomyelitis progressive muscular atrophy were investigated for the presence of antibody to poliovirus types 1, 2, and 3, coxsackie viruses B3 and B4, influenza A, measles, rubella, mumps, herpes simplex types 1 and 2, cytomegalovirus, varicella-zoster, and Toxoplasma gondii. These results were compared with those from 53 control patients with neuromuscular disease matched for age, sex, race, and poliovirus vaccine exposure. There was no difference either in distribution of serum or CSF antibody titers or the geometric-mean antibody titers. There was no evidence suggesting the presence of locally produced specific viral antibody within the CNS to any of the agents studied. In particular, there was no serological evidence to suggest an association between persistent infection with any poliovirus type and amyotrophic lateral sclerosis or late-onset postpoliomyelitis progressive muscular atrophy.
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PMID:CSF viral antibodies. Evaluation in amyotrophic lateral sclerosis and late-onset postpoliomyelitis progressive muscular atrophy. 22 Sep 38

Several data indicate that the dysfunction of some neuropeptide function may play a role in the pathogenesis of the amyotrophic lateral sclerosis. Therefore, the authors decide to determine a concentration of one of them in CSF, namely calcitonin. Calcitonin is widely distributed in CNS, including both anterior and posterior horns of the spinal cord. It was confirmed with immunohistochemical assays and an examination of the human CSF. Calcitonin concentration in CSF has been assayed in 12 patients with amyotrophic lateral sclerosis and in 12 patients of the control group. Calcitonin concentrations in CSF have been measured with RIA technique, using appropriate kits manufactured by Mallinckrodt Dgn. Mean calcitonin CSF concentration in patients with amyotrophic lateral sclerosis was 448. +/- 74.3 pg/ml, and was lowered in comparison with that in the control group, i.e. 613.9 +/- 147.2 pg/ml. The results confirm the authors' previous reports on the reduced content of some neuropeptides in CSF of patients with amyotrophic lateral sclerosis and suggest a possible calcitonin role in the pathogenesis of this disease.
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PMID:[Calcitonin level in cerebrospinal fluid of patients with amyotrophic lateral sclerosis]. 143 39

In 12 patients with amyotrophic lateral sclerosis (ALS) participating in a therapeutic trial with intrathecally applied human fibroblast interferon-beta (IFN-beta) and in 9 untreated ALS patients, we found significantly elevated circulating serum IgG immune complexes (CIC), quantitative immunoglobulin changes, and creatine kinase (CK) elevation; CK reached significantly more often pathological levels in non-bulbar disease. Dermal ultrastructural changes were equally present in all treated as well as untreated ALS patients. Some time ago IL-6 was quantitatively cleaned out of the Fiblaferon-preparation. Erythrocyte sedimentation rate (ESR) rose during intrathecal IFN therapy in 9/10 ALS patients. In 4/4 adequately monitored motoneuron patients, this elevation coincided with a decrease of serum CK, while ESR and CK did not correlate in 60 non-ALS non-IFN neurological controls. Collagen ultrastructure, CSF total protein or barrier function, immune complexes, immunoglobulin quantitation and serum CK may contribute to differentiated diagnosis and should be included in future study protocols.
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PMID:Dermal, serological and CSF changes in amyotrophic lateral sclerosis with and without intrathecal interferon beta treatment. 150 22

We report the case of a patient who presented with both amyotrophic lateral sclerosis and Huntington's disease. Interestingly, aspartate level was increased in the lumbar CSF. In vitro and in vivo studies have convincingly suggested that these two neurodegenerative diseases could be related to an excitotoxic mechanism.
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PMID:[Familial amyotrophic lateral sclerosis associated with Huntington chorea with increased aspartate level in the cerebrospinal fluid]. 153 26

Evidence that thyrotropin-releasing hormone (TRH) has prominent trophic effects on the motor system led to several negative therapeutic trials in amyotrophic lateral sclerosis, a disease of the motor system. Since TRH crosses the blood-brain barrier poorly, if at all, we postulated that the negative parenteral clinical trials could be a result of insufficient drug-receptor interaction. We thus carried out a blinded, placebo-controlled, crossover study of intrathecal TRH in 36 patients by delivery through an implanted, constant infusion pump achieving a steady-state CSF level comparable with that shown to be effective in tissue culture experiments. Utilizing a quantitative measurement technique to assess motor unit loss, we did not observe any alteration of the progressive course during 6 months on TRH and 6 months on saline placebo. However, the implanted pump delivery system proved to be safe, reliable, and well tolerated.
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PMID:Intrathecal thyrotropin-releasing hormone does not alter the progressive course of ALS: experience with an intrathecal drug delivery system. 157 28

This study presents the experience of one year of treatment in patients with amyotrophic lateral sclerosis, with intrathecal TRH administered daily by a subcutaneous reservoir connected to the intrathecal lumbar space by a double catheter system as to provide continuous circulation of CSF and to avoid sac formation since this would be a source of infection. Clinical evaluation was carried out with a scale developed by the authors with the main aim of evaluating the loss of vital motor abilities and not as a localized evaluation. Secondary effects due to the implantation of the reservoir in addition to its use are presented although data were not important. Intrathecal administration of TRH was carried out similarly (600 micrograms/day) with secondary effects being the same as those by other routes of administration although of a lesser intensity. The results of the clinical evaluation at the beginning and end of the treatment as well as after patient follow up demonstrated that beneficial effects do not occur equally in all patients but rather are transitory and do not improve the natural evolution of the disease. The authors conclude that, methodologically, this series study does not enter within the frame of an advisable statistical study since the aim is to provide data for a future controlled study.
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PMID:[Treatment of amyotrophic lateral sclerosis with daily intrathecal TRH. A year's experience. Pilot study II]. 159 3

We screened 140 patients with different neurological diseases for the presence of anti HTLV-1 virus antibodies. ELISA test confirmed with Western Blot analysis was performed in CSF and blood. Positive findings were obtained in 23 out of 52 patients with progressive spastic paraparesis (44%). All patients with multiple sclerosis, polymyositis, amyotrophic lateral sclerosis or chronic polyneuropathy were negative. Patients with progressive spastic paraparesis and positive HTLV-1 antibodies were most commonly women (78%) and middle aged (mean 46 years old), with a history of surgical interventions (70%) or blood transfusion (35%). A slowly progressive spastic paraparesis with asymmetric onset and minimal sensory complaints was observed in some cases. Mononuclear pleocytosis in the CSF was observed in 35% with an increased IgG index in 88%. A delayed latency and low amplitude of somatosensory evoked potentials was observed in 89% of patients.
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PMID:[HTLV-I retrovirus in Chile: study on 140 neurological patients]. 177 82


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