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Bratisl Lek Listy 1997 Jun;98(6):315-20
Neurobiologicka klinika Fakulty Vseobecneho lekarstvi Karlovy university v Praze.
At present an increase of some autoaggressive diseases can be observed. The commonly used treatment consists of the administration of some immunosuppressive drug of some hormonal preparations. This type of therapy is accompanied by some undesired side effects, as these drugs influence also some other cell systems besides the immunologically active cells. These drugs are also known to lower the resistance to some intercurrent infections. Due to these undesired side effects some naturally occurring factors are introduced into the therapy. These are e.g., TGF-beta, or some interleukins (IL-10 etc.). In our department and immunosuppressively acting substance was isolated from DHL which had the ability to inhibit the AA (adjuvant arthritis) in rats. In humans this SF (suppressive factor) stimulates the CD 8+ cells which are known to have suppressoric activity. This SF was successfully applied in some autoaggressive diseases, e.g., atopic eczema, multiple sclerosis, some polyradiculoenuritis, amyotropic lateral sclerosis etc. In this paper the results in the ALS patients are given. Amongst other possibilities of the therapy the application of antilymphocyte sera, monoclonal antibodies to some CD markers of lymphocytes and some methods of hyposensitizations of tolerance induction are mentioned. Further, an original method using antigen bound to isosoluble carrier is described. This administration of encephalitogen bound onto Sforon (polyacrylate spheres) sis not only inhibit the EAE manifestations but also enable the survival of guinea-pigs which had already manifested the clinical signs of EAE.
PMID: 9333422, UI: 97446588
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Acta Neurol Scand 1997 Jul;96(1):14-21
Department of Neuromedicine, Bangur Institute of Neurology, Calcutta, India.
A clinical study about the pattern of motor neurone disease in eastern India was carried out from July 1993 to June 1995 at Bangur Institute of Neurology, Calcutta and SSKM Hospital, Calcutta. A total of 110 cases were studied and they constituted 0.11% of all neurological cases seen in the general OPD. Of 110 cases, amyotropic lateral sclerosis (ALS) constituted 43.6%, progressive muscular atrophy (PMA) 10.9%, post-polio progressive muscular atrophy (PPMA) 1.8%, spinal muscular atrophy (SMA) 20%, atypical form Madras pattern of MND (MMND) 0.9% and monomelic amyotrophy (MMA) 22.7% of cases. Disease is more common in males than females and average duration of symptoms before presentation varied from 1 to 12 months. Most of the patients were either agricultural labourers or manual workers in ALS variety whereas MMA variety was evenly distributed in both hard labourers and sedentary workers. Most of the patients in MMA and SMA groups presented before 30 years of age whereas ALS and PMA group presented after 30 years. Trauma was the commonest antecedent event in ALS and MMA followed by electrocution in the same two groups. Family history was found to be absent in SMA group though the disease is considered as a hereditary one. Weakness of the limbs and wasting of the muscles were common presenting symptoms and signs. Bulbar symptoms and signs were found only in the ALS group. EMG showed neurogenic pattern and mixed pattern in most of the patients in all groups. Only a few patients showed myopathic pattern. Neuroimaging study helped in exclusion of compressive lesion excepting two cases of MMA where facetal hypertrophy was present. Monomelic amyotrophy, a special variety of motor neurone disease, is not rare in this part as compared to other parts of India and Asia.
PMID: 9262127, UI: 97404041
Can J Physiol Pharmacol 1996 Apr;74(4):341-61
Department of Psychology, College of Arts and Sciences, University of North Carolina at Greensboro 27412, USA.
A number of different factors contributed to the rationale for providing a critical review of the field of excitatory amino acids in the cerebral cortex at this time. In addition to the recent growing realisation by clinicians that the excitatory amino acids are linked critically to a number of neurological conditions, including neurodegenerative syndromes such as Alzheimer's disease, cortical damage due to stroke and cerebral ischemia, epilepsy, amyotropic lateral sclerosis, and schizophrenia, the recent cloning and membrane reconstitution of specific receptors known as AMPA, NMDA, kainate, and metabotropic receptors and their known subunits have prompted a surge of renewed interest in this important family of synaptic transmitter molecules. Moreover, recent advances in our understanding of the molecular events involved in growth promotion in the early stages of the development of the cortex have shown that both NMDA and non-NMDA receptor subtypes perform important roles in certain aspects of target selection and neurite outgrowth, in cone stimulation and guidance, and in spine formation and morphological alterations. A recent explosive growth in interest in the possible roles of nitric oxide and related short-lived radicals in plasticity, disease, and synaptic transmission also is related closely with the actions of excitatory amino acids. All these relatively new advances have transpired in parallel with ongoing work that has extended our appreciation for the roles of excitatory amino acids in the areas of synaptic plasticity (long-term potentiation, long-term depression, receptive field reorganisation following drug-induced or peripheral sensory disturbances, such as denervation or amputation), in processes of normal transmission at functionally and electrophysiologically identified neurones of the cerebral cortex, and in distinct proposed roles for cortical glia. A greater appreciation of the diverse types and properties of the burgeoning family of receptors for the metabotropic receptor also contributed to our desire to feature that aspect of the field in the context of glia and neurones of the cerebral cortex. That part of the field of neuroscience concerned with the functions of excitatory amino acids has grown so large over the past 10 years or so, that a review paper focusing on the contributions to a specialized meeting devoted solely to cerebral cortex could easily be supported by material comprising a sufficient body of communications from top-quality research laboratories. The present account endeavours to summarize and discuss the biochemical characteristics, physiological roles, pharmacological properties, clinical relevance, developmental involvements, and anatomical-morphological aspects pertaining to the excitatory amino acid transmitters in cerebral cortex.
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PMID: 8828881, UI: 96426602
Ann Neurol 1996 Feb;39(2):203-16
Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA.
Numerous studies of amyotrophic lateral sclerosis have suggested that increased intracellular calcium is a common denominator in motoneuron injury. In experimental models, IgG from patients with amyotrophic lateral sclerosis enhanced calcium entry and induced apoptotic cell death in vitro as well as increased intracellular calcium and induced ultrastructural alterations of the motor nerve terminals in mice in vivo. To determine whether similar increases in intracellular calcium and altered morphology are present in motor nerve terminals of amyotrophic lateral sclerosis patients in vivo, muscle biopsy specimens from 7 patients with amyotrophic lateral sclerosis, 10 nondenervating disease control subjects, and 5 patients with denervating neuropathies were analyzed with ultrastructural techniques, employing oxalate-pyroantimonate fixation to preserve in situ calcium distribution. Motor nerve terminals from amyotrophic lateral sclerosis specimens contained significantly increased calcium, increased mitochondrial volume, and increased numbers of synaptic vesicles compared to any of the disease control groups, without exhibiting excess Schwann envelopment specific to denervating terminals. These results parallel the effect of amyotrophic lateral sclerosis IgG passively transferred to mice, and provide the first demonstration that neuronal calcium is, in fact, increased in amyotrophic lateral sclerosis in vivo.
PMID: 8967752, UI: 96197944
Vopr Med Khim 1994 Sep-Oct;40(5):42-5
Protein composition of human skeletal muscle impaired with lateral amyotropic sclerosis was studied by means of two-dimensional electrophoresis. Some protein fractions were altered. Characteristic property of all the preparations studied proved to be disappearance of three protein fractions of 35 kDa molecular mass and with pI 5.9, 6.0 and 6.1.
PMID: 7839670, UI: 95141624
J Neural Transm Park Dis Dement Sect 1993;5(1):63-5
Department of Biochemistry, St. Mary's Hospital Medical School, London, United Kingdom.
Exons 16 and 17 of the beta-amyloid precursor protein gene has been sequenced in individuals with the amyotropic lateral sclerosis/Parkinson's dementia complex of Guam to test the hypothesis that this disease is an allelic variant of Alzheimer's disease and to test whether sequence differences within beta-amyloid in this population contributes to the non-deposition of this peptide in the disorder. The sequence was normal.
PMID: 8439393, UI: 93176304
J Korean Med Sci 1992 Jun;7(2):167-9
Department of Neurology and Pathology, University of Ulsan, Asan Medical Center, Seoul, Korea.
Peripheral neuropathy or amyotropic lateral sclerosis can be associated with renal cell carcinoma. We report a 63-year-old male patient with renal cell carcinoma who developed an atypical, progressive neuropathy after nephrectomy.
PMID: 1326285, UI: 92398841
Ann Neurol 1992 Feb;31(2):119-30
Neurology Service, Massachusetts General Hospital, Boston 02114.
The etiology of nerve cell death in neuronal degenerative disease is unknown, but it has been hypothesized that excitotoxic mechanisms may play a role. Such mechanisms may play a role in diseases such as Huntington's disease, Parkinson's disease, amyotropic lateral sclerosis, and Alzheimer's disease. In these illnesses, the slowly evolving neuronal death is unlikely to be due to a sudden release of glutamate, such as occurs in ischemia. One possibility, however, is that a defect in mitochondrial energy metabolism could secondarily lead to slow excitotoxic neuronal death, by making neurons more vulnerable to endogenous glutamate. With reduced oxidative metabolism and partial cell membrane depolarization, voltage-dependent N-methyl-D-aspartate (NMDA) receptor ion channels would be more easily activated. In addition, several other processes involved in buffering intracellular calcium may be impaired. Recent studies in experimental animals showed that mitochondrial toxins can result in a pattern of neuronal degeneration closely resembling that seen in Huntington's disease, which can be blocked with NMDA antagonists. NMDA antagonists also block neuronal degeneration induced by 1-methyl-4-phenylpyridium, which has been implicated in experimental models of Parkinson's disease. The delayed onset of neurodegenerative illnesses could be related to the progressive impairment of mitochondrial oxidative phosphorylation, which accompanies normal aging. If defective mitochondrial energy metabolism plays a role in cell death in neurodegenerative disorders, potential therapeutic strategies would be to use excitatory amino acid antagonists or agents to bypass bioenergetic defects.
PMID: 1349466, UI: 92246456
Ann Ital Med Int 1990 Jul-Sep;5(3 Pt 2):232-44
II Cattedra di Farmacologia Medica, Universita degli Studi La Sapienza di Roma.
Pharmacological interest in the tripeptide thyrotropin-releasing hormone (TRH) is due to the multiple effects it produces. In fact, apart from taking part in regulating the activity of the hypothalamo-pituitary-thyroid axis, TRH produces various neuropharmacological effects which indicate a biological role that is probably more important than that of a releasing hormone. Trials performed in animals have shown, for example, the dose-dependent capacity of TRH to induce analgesia, probably by interacting with the opioid peptide system. Motor activity is affected by TRH. In fact this tripeptide elicits an increase in spontaneous motor and explorative activities by interacting with the dopaminergic neurotransmitter system at the nucleus accumbens level. The neuropharmacological activities of TRH include an interesting arousal effect and an analeptic action on generalized depression of the CNS whether this depression is of natural origin, such as hibernation, or induced pharmacologically (barbiturates, ethanol) or of a traumatic origin (coma). This analeptic action is attributable to stimulation of cholinergic neurons in the septo-hippocampal area and to the presence of terminals containing TRH in the lateral septum and TRH receptors concentrated especially in the medial septum and diagonal band of Broca. It has also been suggested that TRH localized in the pineal gland has a part in activating the neuronal mechanisms of arousal. Associated with the arousal effect and especially evident in variously originated shock conditions are the activating effects of TRH on vegetative functions (body temperature, circulation, the gastrointestinal tract). These stimulatory activities on the CNS were the rationale for therapeutic use of TRH in the initial treatment of coma due to brain trauma and for the treatment of endogenous depression. A most interesting property of TRH is that of counteracting the neurological deficit due to experimental lesion of the spinal cord particularly with regard to spasticity and ataxia. Electrophysiological trials have shown that TRH depolarizes the motoneurons in frog spinal cord thereby increasing the monosynaptic reflex. Furthermore, TRH has recently been shown to have a trophic effect on cultures of rat fetus spinal cord. On this basis TRH has been used successfully for the treatment of amyotropic lateral sclerosis (Charcot's syndrome) and spinocerebellar degeneration. Further support for this therapeutic strategy is given by the demonstration that deafferentiation of rat spinal cord produces an increased density of TRH spinal receptors. Recent studies have also given encouraging results on the possible therapeutic use of TRH for the treatment of Alzheimer's disease.
PMID: 2127684, UI: 91175319
J Neurogenet 1987 Jun;4(4):201-13
The motor neuron degeneration (Mnd) is characterized by a progressive deterioration of motor function (stiff-legged gait, abnormal limb placements and grasping, and finally paralysis; moving from rear to forelimbs). There is a dramatic degeneration of spinal cord motor neurons, more severe in the lumbosacral than in the other regions, as well as variable pathology in the lower cranial nerves. Upper motor neurons of the red nucleus, reticular formation of the pons and medulla, and restricted areas of the cerebral cortex are also affected. Degenerating motor neurons share many characteristics seen in the human disease amyotropic lateral sclerosis, including loss of Nissl substance, increases in lipofuscin and abnormal cytoplasmic inclusions. Additionally, Mnd, like ALS, is a disease of later life.
PMID: 3612378, UI: 87283009
Lancet 1985 Dec 14;2(8468):1353
PMID: 2866399, UI: 86064559
Biochem Biophys Res Commun 1984 Jan 13;118(1):82-9
Gangliosides of healthy and pathologic muscles (amyotropic lateral sclerosis and facio-scapulo-humeral muscular dystrophy) were studied. Total ganglioside content of the affected muscles was approximately 2 fold higher than the unaffected muscles. Our results showed that ALS muscle contained a ganglioside which was absent in the unaffected and FSH muscular dystrophic muscles. Based on the results of hydrolysis with Vibrio cholerae neuraminidase and subsequent reactivity of the asialo derivative towards anti-globotetraosylceramide, we propose that the ALS ganglioside is sialosylglobtetraosylceramide, NeuAc(alpha 2-3)Ga1NAc(beta 1-3)Ga1(alpha 1-4)Ga1(beta 1-4)G1c-Cer.
PMID: 6696769, UI: 84128066
Neurochem Res 1982 May;7(5):627-36
Glutamate dehydrogenase (GDH) activity in leukocytes and platelets in spinocerebellar degenerations (SCD) was determined. In the same subject, GDH activity was higher and more reproducible in platelets than in leukocytes. GDH was decreased significantly in olivopontocerebellar atrophy (OPCA) (Ca. 30% decrease). Pyruvate dehydrogenase (PDH) in platelets showed non specific decreased activity in SCD and amyotropic lateral sclerosis. Energy metabolism in cerebellum may be diminished in some types of ataxia, and glutaminergic neurons may be more affected in OPCA than in other SCD.
PMID: 6811963, UI: 83013205
Zh Nevropatol Psikhiatr Im S S Korsakova 1977;77(2):198-201
In order to eliminate antibodies to water soluble proteins of the human brain which were preliminary divided into 10 fractions of DEAE-cellulose, the authors studied the serum of 30 patients with disseminated sclerosis and 30 patients with lateral amyotrophical sclerosis and determined their antithymic serum activity. The antibides were detected only to protein fractions 1,2 and 10. There was a tendency towards a more frequent detection of antibodies with an increase of the severity of the disease. It was not possible to determine antibodies with an increase of the severity of the disease. It was not possible to determine any correlation between the amount of antibodies and such clinical indices as the degree of severity, duration, form of the disorder, age and sex of the patients. The cytotoxic index of the patients was significantly higher than that in normals. The authors also found a correlation between the antibodies in the serum cytoplasmatic brain proteins and antithymic activity of the same sera.
PMID: 842237, UI: 77130716
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