It is not unconimm that the monoclonal gammopathies occur in patients with demyelinating peripheral neuropathy. The IgM.-protein from those patients reacted with peripheral nerve myelin, which was first identified as the MAG (myelinassociated glycoprotein). In this study the authors tried to understand the pathogenetic mechanism of anti-MAG antibod by studying the expression of the anti MAG antibody in Epstein-Barr virus transformed lymphocytes of umbrical cord. The positive bands for anti-NMG antibody were found in 10 out of 320 sera of peripheral neuropathies by the thin layer chromatography of the glycolipids from bovine peripheral myelin. The positive anti-MAG expressions were found in 5 of 31 supernatant wells from EB virus transformed lymphocytes of umbrical cords, which tended to. Develop the antibody activity in the supernatants cultured more than ten days. The study indicates. In part that the anti-MAG antibody may be occurred by the autoimmune mechanism as the antibodies are expressed m the EB virus transformed lymphocytes which have never been exposed to foreign antigens so far However the automimune mechanism can be supported more by knowing the mcreased frequency of CD5 positive B cells in cases with high titer of anti-MAG antibodies.
Antibodies ; B-Lymphocytes ; Chromatography, Thin Layer ; Glycolipids ; Herpesvirus 4, Human ; Humans ; Lymphocytes* ; Myelin Sheath ; Paraproteinemias ; Peripheral Nerves ; Peripheral Nervous System Diseases*

Recent researches on ALS pathogenesis are focusing on abnormal immunological factors, excitotoxic substances, neurotrophic factors, and oxidative stress. It is well known that glutamate and aspartate are major putative excitatory neurotransmitters and possess excitotoxic properties that lead to neuronal death. In this study the authors checked the plasma level of amino acids in ALS and control groups and tried to understand any association between excitotoxic amino acids and sporadic ALS. The concentration of amino acids was measured by the HPLC method in the fasting plasma of fifteen ALS and nine control subjects. When we evaluated 19 amino acids or their metabolites, none showed significant difference between ALS and control groups. The mean concentrations of glutamic acid in ALS and control groups were 42.3+26.7 mmol/L and 57.4+17.0 mmol/L respectively, which showed no significant difference (p>O. 05). It was not possible to compare the level of aspartic acid in ALS and control groups as the levels were very low in individuals of both groups. In conclusion, authors could not note any significant correlations between sporadic ALS and excitotoxic substances, such as glutamate and aspartate. However, further studies m the excitotoxic levels in cerebrospinal fluid, spinal cord and brain, could be helpful to understand the overexcitation character of motor neuron by excitatory amino acids.
Amino Acids* ; Amyotrophic Lateral Sclerosis* ; Aspartic Acid ; Brain ; Cerebrospinal Fluid ; Chromatography, High Pressure Liquid ; Excitatory Amino Acids ; Fasting ; Glutamic Acid ; Immunologic Factors ; Motor Neurons ; Nerve Growth Factors ; Neurons ; Neurotransmitter Agents ; Oxidative Stress ; Plasma ; Spinal Cord

The goal of this study was to evaluate neuroprotective effects of nitroglycerin and phenytom using in the developing rat brains Seven days old Sprague-Dawley rats underwent right carotid ligation followed by 8% 02 exposure (humidified, balanced with mtrogen) for 3 hours under the halothane anesthesia (control group, N=58). Body temperature of rats was accurately controlled before and during hypoxia. In nitroglycerin treated group (N=33), nitroglycerin was delivered chronically via patches (in escalating doses to 4mg/kg/hr) for 36hrs before and 48hrs postischemia. Phenytoin treated group(N=17) received intraperitoneal phenytoin(30mg/kg) before ischemia. Combined treated group(N=31) with nitroglycerin and phenytoin received two compounds with same method as above. Animals. Sacrificed one week later and histopathological evaluation for ischernic neuronal damage were conducted employing hemat,oxylin eosin staining and measurements of the hemispheric weight difference. Phenytoin was effective in reducing neuronal damage in terms of weight comparison(24+2.4%, atrophy in control vs. 5+2.9% in phnytoin group, p<0.001) and ischemic changes in hippocampal area(p<0.05 in CA1, CA2, and CA3 area). Transdermal nitroglycerin did not show any beneficial effects compared with control group(23+3.0% in nitroglycerin group vs. 24+2.5% in control). Combined treated group showed neuroprotection on weight comparison(24+2.4% in control vs 4+1.8% in combined group, p<0.001) and ischemic changes in hippocampal area(p<0.05 in CA1, CAZ and CA3 area) but dldnot show any additional neuroprotective effects compared with phenytoin group on weight comparison and histological changes. These data suggest that the degree of the injury from hypoxic-iwhemic insults of developing rat brain may be reduced by the compounds that modulate voltagedependent sodium channels such as phenytom but not by nitroglycein.
Anesthesia ; Animals ; Anoxia ; Atrophy ; Body Temperature ; Brain* ; Eosine Yellowish-(YS) ; Halothane ; Ischemia ; Ligation ; Neurons ; Neuroprotective Agents* ; Nitroglycerin* ; Phenytoin* ; Rats* ; Rats, Sprague-Dawley ; Sodium Channels

Thiol-specific antioxidant protein (TSA) is the antioxidant protein which specifically inhibits the inactivation of various enzymes by a nonenzymatic mixedfunction oxidation (MFO) system containing a sulfhydryl compound as reducing equivalent but not by the MFO system containing a nonsulf hydryl reducing equivalent. TSA was isolated and purified from Saccharomyces cerevisiae and bovine brain. But localization in the brain and physiological role of TSA as an antioxidant enzyme a-re known very little. The localization of TSA protein in the rat brain and rabbit spinal cord was examined with polygonal antibodies to bovine TSA made in rabbit. Tissues were fixed with 4% paraformaldehyde, frozen in dry ice, sectioned on a sliding microtome, incubated with these antibodies, and then processed for avidin-biotin peroxidase complex staining. The irrimunoreactive (IR) cellular element for TSA in the central nervous system - ne-om The IR product for TSA was mainly located m neuronal soma and proximal part of neuronal process such as apical dendnte of pyranudal cell of the cerebral cortex. The glial cell, blood vessel and nucleus of neuron did not show the TSA IR TSA IR neurons were found at every nucleus and cortex mcluding cerebral cortex, hippocampus, corpus striatum, cerebellar cortex, thalamus, septum and spinal gray matter. In hypoxia rabbit spinal cord, there were dense and light IR neurons, and the former was considered to be miured by hypoxic msult These results indicate that TSA is ubiquitous protem in neurons of mammalian central nervous system and show uneven distribution among individual neurons in same nucleus and different nucleus. And TSA may be induced by increased oxidative pressure after ischemia.
Animals ; Anoxia ; Antibodies ; Blood Vessels ; Brain ; Carisoprodol ; Central Nervous System ; Cerebellar Cortex ; Cerebral Cortex ; Corpus Striatum ; Dry Ice ; Hippocampus ; Ischemia ; Neuroglia ; Neurons ; Peroxidase ; Peroxiredoxins* ; Rats ; Saccharomyces cerevisiae ; Spinal Cord ; Thalamus

Hypoxia-induced cell damage is known to be mediated by increase in intracellular calcium. In the present study, the effect of calcium channel blockers on the hypoxia-induced cell damage was investigated in iat pheochromocytoma cells line, PC12 cells. The cultured cells were exposed to hypoxia under 95% N2 plus 5% C02 gas phase and incubated in the media devoid of fetal bovine seruril The cell demage was assessed by measuring the release of lactate dehydrogenase (LDH) from the cells into the incubation media. Exposure of the cells to hypoxia for 2 hours caused a 28% of the total LDH to be released from cells -into media. The pretreatment of the cells with 1 mM each of diltiazem, nifedipine, and verapamil depressed the LDH release to the extent of 52%, 42%, and 30% inhibition, respectively. The inhibitory effects of diltiazem and verapamil were more marked at 1 mM than at 10 mM. The influx of 45 Ca2+ into the cells was rapidly increased within 2 minutes after exposure of the cells to hypoxia. Diltiazem at 1 mM almost completely inhibited Ca2+ influx, while nifedipine and verapamil exhibited only, 30% inhibition of Ca2- influx. The results lend support to the notion that mcreased intracellular calcium triggers a series of cascade reactions leadmg to cell death. It is suggested that the inhibitory effects of various calcium antagonists on hypoxia-induced cell damage differ from each other in their potency.
Animals ; Anoxia* ; Calcium Channel Blockers ; Calcium* ; Cell Death ; Cells, Cultured ; Diltiazem ; L-Lactate Dehydrogenase ; Nifedipine ; PC12 Cells* ; Pheochromocytoma ; Verapamil

We report a familial case of neuronal type of Charcot-Marie-Tooth disease diagnosed by sural nerve biopsy, nerve conduction study, and electromyography.
Biopsy ; Charcot-Marie-Tooth Disease* ; Electromyography ; Neural Conduction ; Neurons* ; Sural Nerve

Transient ischemic attacks or embolic strokes caused by the cardiac myxoma are very rare in its freguency. To detect cardiac myxoma, as a source of cerebral embolism originating from the heart, has a great significance because of the occurrence and recurrence of the possible stroke can be prevented by surgical procedure. We present a patient who showed typical clinical symptoms and signs of the left atrial myxoma which was subsequently diagnosed by 2-dimensional real time echocardiography and confirmed by sugery. Following the successful removal of the left atrial myxoma, now, the patient is in stable neurological condition.
Echocardiography ; Heart ; Humans ; Intracranial Embolism* ; Ischemic Attack, Transient ; Myxoma* ; Recurrence ; Stroke

Hypereosinophilic syndrome comprises several disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. It is likely that organ damage is due to infiltration by eosinophils or to eosinophil-derived products. We report a case of hypereosinophilic syndrome with multiple mononeuropathy verified by EMG, nerve conduction study, and biosy..
Eosinophilia ; Eosinophils ; Hypereosinophilic Syndrome* ; Mononeuropathies* ; Neural Conduction

Thrombosis of deep cerebral veins including internal cerebral vein, great vein of Galen and straight sinus is frequently associated with thalamic and other deep cerebral infarctions or hemorrhages, which leading to severe nourologic deficits or death. Although the diagnosis might be dependent on cerebral angiogram in selected cases, the brain CT scan has been proved to have a great diagnostic value. The present report describes a 29 year-old unmarried female with abruptly developed deep cerebral vein thrombosis followed by secondary bithalamic hemorrhagic infarcts. Despite of prolonged periods of impaired consciousness after extraventricular drainage, the overall recovery was good with mild neurologic sequale.
Adult* ; Brain ; Cerebral Infarction ; Cerebral Veins* ; Consciousness ; Diagnosis ; Drainage ; Female ; Hemorrhage ; Humans ; Single Person ; Thrombosis* ; Tomography, X-Ray Computed

Churg-Strauss syndrome is one of idiopathic, systemic necrotizing vasulitides and characterized by a history of asthma or allergic rhinitis, eosinophilia of peripheral blood and variable symptoms of the systemic vasculitis. This is a case report of classical Churg-Strauss syndrome in a 6- year-old male who has the multiple mononeuropathy as a symptom of the systemic vasculitis. The sural nerve biopsy showed a few myelin digestion chambers suggesting axonal degeneration which was not suspected on the electrophysiological study, and the specimen of right gastrocnemius muscle revealed the typical pathological findings of eosinophilic infiltration, extravascular granuloma and necrotizing small vessel vasculitis. The therapeutic response of cyclophosphamide looked better than the oral prednisolone treatment only.
Asthma ; Axons ; Biopsy ; Churg-Strauss Syndrome* ; Cyclophosphamide ; Digestion ; Eosinophilia ; Eosinophils ; Granuloma ; Humans ; Male ; Mononeuropathies ; Muscle, Skeletal ; Myelin Sheath ; Prednisolone ; Rhinitis ; Sural Nerve ; Systemic Vasculitis ; Vasculitis