A Case of Pachydermoperiostosis with Watery Diarrhea,Giant Gastri Rugae,and Endocrine Disorder

Abstract: A 31-year-old man, cook, who had had persistent watery diarrhea for about a month visited our hospital. He had already been diagnosed as having pachydermoperiostosis. An examination of the upper gastrointestinal tract revealed that he had giant gastric rugae. The histology was compatible with hypertrophic gastritis accompanied with marked hyperplasia of the fundic gland and foveolar epithelia. The output of gastric juice was high in volume, although its acidity normal. The barium transit time of the small intestine was reduced to 15 minutes. His diarrhea was, thus, considered to be induced by gastrointestinal hyperfunction. The patient's glucose tolerance was abnormal and basal Cortisol secretion level was high. Pachydermoperiostosis has been the focus of attention for skin and bone changes, and the frequency of this disease with endocrine disorders seems rather high, but accompanying gastrointestinal disorders have so far not often been reported in Japan. The results of our investigation strongly suggest that pachydermoperiostosis is a systemic disease.     

Computerized Analysis of EEG Background Activity in Epileptic Patients

Background activity was studied in 128 idiopathic epilepsy patients and 30 normal controls using EEG topography and t-statistic significance probability mapping (t-SPM). In epileptic patients, EEG background activity showed a marked increase in delta, theta, alpha 1, and beta 1, and a decrease in alpha 2 activity as compared with controls. Untreated epileptic patients had a significant increase in delta, theta, and alpha 1 as compared with controls. For epileptic patients treated with antiepileptic drugs (AEDs), the most marked slowing was observed in the polytherapy group, followed by the monotherapy group and then the untreated group. Among seizure types, patients with partial seizures (PS) tended to exhibit more slowing than patients with only generalized tonic-clonic seizures (GTC). Moreover, PS had a right-left asymmetry in alpha 2 and beta 1 activities. In a comparison of AEDs, patients receiving carbamazepine (CBZ) and phenobarbital (PB) showed no significant difference as compared with the untreated group. In contrast, patients receiving valproate (VPA) showed a decrease in slow and fast activities. EEG changes associated with each AED were different in GTC and PS. Patients receiving VPA for GTC showed a decrease in theta and beta 1 activities, but those with PS showed a decrease only in delta activity.     

A Thermoconductive and Thermoselective Leukocyte Filter

Abstract: Previously, it was reported by our group that the superphysiological temperature of over 43°C affected leukocyte properties. It was speculated that heat treatment might alter the immunological properties of leukocytes. In order to take advantages of this phenomenon, attempts were made to develop a thermoconductive and thermoselective leukocyte filter. A stainless steel fiber was selected for the new material of a leukocyte filter due to its good heat conductance, stability during thermal change, and inertness to any chemicals. Preliminary studies were conducted to evaluate the effect of fiber diameter and temperature on the filtration of blood cells. As a result, more than 99% of white blood cells (WBCs) and platelets were removed utilizing a 4 μm diameter fiber filter. The environmental temperatures of the filtration process were varied to 4, 20, 36, and 50°C for 5 min. Almost 95% of WBCs and platelets were removed at 4, 20, and 36°C. However, the reduction of platelets was remarkably suppressed at 50°C (54%). Additionally, the removal of lymphocytes was significantly lower than that of neutrophils at 50°C (69% vs. 83%; p = 0.0002). These data demonstrate that effective heat treatment and removal of leukocytes are possible with an ultrafine stainless steel fiber filter.     

IgM anti-GQ1b monoclonal antibody inhibits voltage-dependent calcium current in cerebellar granule cells

Miller–Fisher syndrome (MFS), which is known to be associated with anti-GQ1b antibodies and to cause ataxia, is a variant of an acute inflammatory neuropathy. However, the pathogenic role of anti-GQ1b antibodies remains unclear. In this study, we investigated the effects of mouse IgM anti-GQ1b monoclonal antibody (IgM anti-GQ1b mAb) on the spontaneous muscle action potential of a rat spinal cord-muscle co-culture system and on the voltage-dependent calcium channel (VDCC) current in cerebellar granule cells and Purkinje cells using the whole-cell patch clamp technique. The frequency of spontaneous muscle action potential of the innervated muscle cells was transiently increased by IgM anti-GQ1b mAb and then was blocked completely, which was the same finding as reported previously. Moreover, the cerebellar granule cell VDCC current was decreased by 30.76 ± 7.60% by 5 μg/mL IgM anti-GQ1b mAb, whereas IgM anti-GQ1b mAb did not affect the VDCC current in cerebellar Purkinje cells. In immunocytochemistry, IgM anti-GQ1b mAb stained the whole cell surface of cerebellar granule cells, but not that of Purkinje cells. Therefore, the clinical symptoms of Miller–Fisher syndrome, such as cerebellar-like ataxia, may be explained by the inhibitory effects of anti-GQ1b antibodies on VDCC current in cerebellar granule cells.     

Molecular pharmacology of glutamate transporters, EAATs and VGLUTs

-Glutamate serves as a major excitatory neurotransmitter in the mammalian central nervous system (CNS) and is stored in synaptic vesicles by an uptake system that is dependent on the proton electrochemical gradient (VGLUTs). Following its exocytotic release, glutamate activates fast-acting, excitatory ionotropic receptors and slower-acting metabotropic receptors to mediate neurotransmission. Na⁺-dependent glutamate transporters (EAATs) located on the plasma membrane of neurons and glial cells rapidly terminate the action of glutamate and maintain its extracellular concentration below excitotoxic levels. Thus far, five Na⁺-dependent glutamate transporters (EAATs 1–5) and three vesicular glutamate transporters (VGLUTs 1–3) have been identified.Examination of EAATs and VGLUTs in brain preparations and by heterologous expression of the various cloned subtypes shows these two transporter families differ in many of their functional properties including substrate specificity and ion requirements. Alterations in the function and/or expression of these carriers have been implicated in a range of psychiatric and neurological disorders. EAATs have been implicated in cerebral stroke, epilepsy, Alzheimer's disease, HIV-associated dementia, Huntington's disease, amyotrophic lateral sclerosis (ALS) and malignant glioma, while VGLUTs have been implicated in schizophrenia. To examine the physiological role of glutamate transporters in more detail, several classes of transportable and non-transportable inhibitors have been developed, many of which are derivatives of the natural amino acids, aspartate and glutamate. This review summarizes the development of these indispensable pharmacological tools, which have been critical to our understanding of normal and abnormal synaptic transmission.     

Daily injections of melatonin entrain the circadian activity rhythms of nocturnal rats but not diurnal chipmunks

It is well known that daily injections of melatonin entrain the free-running rhythms of nocturnal rodents (rats and hamster) and diurnal sauropside (birds and lizard). Here, we asked whether daily injections of melatonin entrain the free-running rhythm of the chipmunk, a diurnal rodent, and, if they do, is the phase relationship between the time of injection and onset of the activity interval similar to that in sauropside rather than that of nocturnal rodents? Contrary to our expectations, daily injections of melatonin did not entrain the free-running rhythm in 9 of 10 chipmunks, even when a high dose of melatonin (1 mg/kg, b.wt.) was used. These results indicate that the entraining effect of daily injections of melatonin on free-running rhythm varies among mammalian species.     

Gene locus for autosomal recessive distal myopathy with rimmed vacuoles maps to chromosome 9

Distal myopathy with rimmed vacuoles is an autosomal recessive muscular disorder, characterized clinically by weakness of the distal muscles in the lower limbs in early adulthood. Recently, the gene locus for familial vacuolar myopathy with autosomal recessive inheritance (hereditary inclusion body myopathy) was mapped to chromosome 9 by genome-wide linkage analysis of nine Persian-Jewish families. Since both disease conditions share similar clinical, genetic, and histopathological features, we analyzed seven families with distal myopathy with rimmed vacuoles using ten microsatellite markers within the region of the hereditary inclusion body myopathy locus. Significantly high cumulative pairwise lod scores were obtained with three markers: D9S248 (Z_max = 5.90 at Θ = 0), D9S43 (Z_max = 5.25 at Θ = 0), and D9S50 (Z_max = 4.23 at Θ = 0). Detection of obligate recombination events as well as multipoint linkage analysis revealed that the most likely location of the distal myopathy with rimmed vacuoles gene is in a 23.3-cM interval defined by D9S319 and D9S276 on chromosome 9. The results raise the possibility that distal myopathy with rimmed vacuoles and hereditary inclusio body myopathy in Persian Jews are allelic diseases.     

Relationship between Sibship Size and Neurotic Symptoms of Anxiety, Hypochondriasis and Depression in Japanese Neurotic Children and Adolescents

Abstract: Two hundred and sixty-eight Japanese aged 19 or below were studied retrospectively from their medical records to determine the relationship between the sibship size (number of siblings in the family) and the appearance of symptoms of anxiety, hypochondriasis and depression during the time period I (1955 + 1960 + 1965) and the time period II (1970 + 1975 + 1978). The results showed that only sons and either sex of a large sibship size (4 or more) possessed these three symptoms to a larger extent than only daughters and either sex of a medium sibship size (2 or 3) during both time periods. These three adult-type neurotic symptoms were thought to be manifested among the children and adolescents who communicated primarily with adults and/or those who were required by their parents to exhibit adult-type behavior whether in traditional or in modern Japanese families.