Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reported an autopsy case with recent memory disturbance, characterized by localized atrophy of parahippocampal gyrus, subiculum and amygdala. This patient initially exhibited recent memory disturbance at the age of 73. She was disoriented to time and place and immediately forgot having had a meal. At the age of 75, she was hospitalized because of progressive forgetfulness and congestive heart failure. One year later, she was admitted to our medical center. On admission, she was alert, but showed severe recent memory disturbance and disorientation to time and place. By contrast, she had neither aphasia nor apraxia. No other neurological symptoms were found. Brain CT showed localized atrophy of the medial part of bilateral temporal lobes and brain SPECT (123I-IMP) revealed a decrease of cerebral blood flow in the same regions. We considered her as early stage of Alzheimer type dementia (ATD) clinically. She died of pneumonia and DIC at the age of 78. Her illness lasted about 5 years. General autopsy showed prolapse of mitral valves, bronchopneumonia and DIC. The brain weighed 1,150 gm. Coronal sections of the brain revealed locarized atrophy of bilateral mediobasal part of the temporal lobes including the rostral parahippocampal gyrus, subiculum and amygdala. There were severe neuronal loss with astrogliosis and a few neurofibrillary tangles (NFT) in the rostral para-hippocampus, CA1 of the hippocampal formation, prosubiculum and amygdala. There were neither senile plaques (SP) nor NFT in the cerebral neocortex. This case lacked neocortical SP and NFT and showed bilateral localized atrophy of rostral parahippocampal gyrus, CA1, subiculum and related structure of the ventromedial temporal lobe with severe neuronal loss and astrogliosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsy case with recent memory disturbance, characterized by localized atrophy of parahippocampal gyrus, subiculum and amygdala]. 833 75

Subicular neurons receive direct afferent connections from the vast majority of CA1 pyramidal cells and send their axons to the various brain areas. Because of this strategic position, subicular cells can modulate output of the hippocampus and, thus, play a significant part in memory, spatial processing, and seizure amplification and propagation from the hippocampus. Despite its important role as a hippocampal interface with different brain regions, present knowledge of the subiculum and the plastic properties of the synapses on the subicular neurons is rather limited. By using IR-DIC videomicroscopy and whole-cell patch-clamp recordings in mouse hippocampal slices, I demonstrated that long-term potentiation (LTP) in CA1-subicular cell synapses can be readily induced by high-frequency stimulation (HFS) of the afferents, but not by pairing of low-frequency stimulation with depolarization of postsynaptic cells. This tetanus-induced LTP is input specific, insensitive to the N-methyl-D-aspartate (NMDA) receptor antagonist 3-[(R)-2Carboxipiperazin-4-yl]-propyl-1-phosphonic acid (R-CPP), and reduces paired-pulse facilitation in potentiated synapses. Subsequent morphologic analysis of the recorded cells, which were filled either with Lucifer Yellow or Biocytin, revealed pyramidal-shaped neurons localized predominantly in the deep layer of the subiculum, close to the CA1 border. Axons of the majority of these neurons extended to the alveus and on toward the hippocampus, probably exiting it via the fornix. These data indicate that CA1-subicular cell synapses in mice exhibit LTP, which can be expressed presynaptically, and its induction does not require NMDA-receptor activation. The observed activity-dependent plasticity might play an important role in the integrative mechanisms of the subiculum and may influence transfer of information from the hippocampus to subcortical and cortical brain areas.
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PMID:Long-term potentiation of single subicular neurons in mice. 1115 14

We present an autopsy case of ornithine transcarbamylase (OTC) deficiency with grumose degeneration in the dentate nucleus of the cerebellum. The patient had intractable neonatal convulsions and hyperammonemia from the 3rd day after birth. Diagnosis of OTC deficiency was made based on null activity of the enzyme and four-base deletions in exon 9 of the OTC gene. Death was due to sepsis as well as disseminated intravascular coagulation at 1 year and 2 months of age. Neuropathology showed multiple cystic changes and ulegyria in the bilateral frontal and parietal lobes. Multiple cysts were associated with the region, which was infiltrated with macrophages surrounded by astroglia showing palisading pattern. Ferrugination was marked in the thalamus and severe neuronal loss with astrogliotic change in the CA1-2 area of the hippocampus. Grumose degeneration was noted in the dentate nucleus of the cerebellum. This is the first report of grumose degeneration in OTC deficiency.
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PMID:An autopsy case of ornithine transcarbamylase deficiency. 1189 Oct 99

Neuronal migration disorders (NMDs) can be associated with neurological dysfunction such as mental retardation, and clusters of disorganized cells (heterotopias) often act as seizure foci in medically intractable partial epilepsies. Methylazoxymethanol (MAM) treatment of pregnant rats results in neuronal heterotopias in offspring, especially in hippocampal area CA1. Although the neurons in dysplastic areas in this model are frequently hyperexcitable, the precise mechanisms controlling excitability remain unclear. Here, we used IR-DIC videomicroscopy and whole cell voltage-clamp techniques to test whether the potent anti-excitatory actions of neuropeptide Y (NPY) affected synaptic excitation of heterotopic neurons. We also compared several synaptic and intrinsic properties of heterotopic, layer 2-3 cortical, and CA1 pyramidal neurons, to further characterize heterotopic cells. NPY powerfully inhibited synaptic excitation onto normal and normotopic CA1 cells but was nearly ineffective on responses evoked in heterotopic cells from stimulation sites within the heterotopia. Glutamatergic synaptic responses on heterotopic cells exhibited a comparatively small, D-2-amino-5-phosphopentanoic acid-sensitive, N-methyl-D-aspartate component. Heterotopic neurons also differed from normal CA1 cells in postsynaptic membrane currents, possessing a prominent inwardly rectifying K(+) current sensitive to Cs(+) and Ba(2+), similar to neocortical layer 2-3 pyramidal cells. CA1 cells instead had a prominent Cs(+)- and 4-(N-ethyl-N-phenylamino)-1,2-dimethyl-6-(methylamino) pyrimidinium chloride-sensitive I(h) and negligible inward rectification, unlike heterotopic cells. Thus heterotopic CA1 cells appear to share numerous physiological similarities with neocortical neurons. The lack of NPY's effects on intra-heterotopic inputs, the small contribution of I(h), and abnormal glutamate receptor function, may all contribute to the lowered threshold for epileptiform activity observed in hippocampal heterotopias and could be important factors in epilepsies associated with NMDs.
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PMID:NPY sensitivity and postsynaptic properties of heterotopic neurons in the MAM model of malformation-associated epilepsy. 1242 9

We have developed a novel device for the quantification of edematous morphology changes in acute brain slices. We can also carry out real-time monitoring of detailed hippocampal cells. The device we developed is based on infrared differential interference contrast microscopy (IR-DIC) and a custom-made real-time computerized image-analysis system for quantification of the morphological dynamics of cells in slice preparations. We applied the coefficient of variation (CV) of light intensity in IR-DIC images to evaluate the change in morphological dynamics. We examined three kinds of edema in the CA1 region of rat hippocampal acute slices under conditions of hypotonic, strong excitation, and experimental ischemia, together with field excitatory postsynaptic potential (fEPSP) recording from radiatum in CA1 the region. There were notable close relationships among the edema formations, the light transmittance, the extent of changes in CV, and features of fEPSP during the three different insults. The present results indicate that CV is a reliable quantification index for edema formation in brain tissue and confirm that applying CV for the analysis in addition to the light transmittance analysis presents additional important information on brain tissue swelling.
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PMID:Novel method for quantification of brain cell swelling in rat hippocampal slices. 1513 31

We have investigated spontaneous synaptic transmission in hippocampal nodular heterotopias in rats exposed to methylazoxymethanol (MAM) in utero. Pregnant Wistar rats were injected with MAM at E16. Acute hippocampal slices were prepared from the rat pups P14 to P40. Whole-cell voltage-clamp recordings were made from visually identified neurons using IR-DIC video microscopy. Synaptic events were recorded from either heterotopic neurons in the CA1 region or "slice-matched" normotopic CA1 pyramidal neurons. Both the spontaneous inhibitory (sIPSC) and excitatory synaptic transmission (sEPSC) to the same neurons were recorded. We found a profound reduction in the frequency of sIPSCs in the heterotopic neurons vs. normotopic neurons. No significant differences in the frequency of sEPSCs were found. We also found a profound reduction in the frequency of spontaneous IPSCs in normotopic neurons following application of the GABA reuptake blocker, NO-711, even in the presence of a GABA(B) receptor antagonist (CGP 55845). Preferentially blocking extrasynaptic GABA(A) receptors caused an increased frequency of sIPSCs in the heterotopic neurons. Our data suggest that there is a predominant change in inhibitory synaptic transmission, as measured by changes in sIPSCs, with no change in excitatory synaptic transmission to heterotopic neurons in hippocampus of rats exposed to MAM in utero. We suggest that this change is caused by an increase in the extracellular concentration of GABA but is not mediated via activation of presynaptic GABA(B) receptors. Rather, we propose that the increased extracellular GABA concentration in the heterotopias dampens the activity in inhibitory neurons via activation of extrasynaptic GABA(A) receptors.
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PMID:Altered spontaneous synaptic inhibition in an animal model of cerebral heterotopias. 2128 7