Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A tenth case (a subtype of complex partial seizures) is proposed as a Limbic (?) Psychotic Trigger Reaction. Upon crying, an infant girl was hit fatally by her devoted father while he was off anticonvulsants prescribed for Jacksonian and petit mal (?) seizures with "porencephalic cyst involving motor cortex and limbic system." Crying revived traumatic memories of frequently repeated ("kindling") experiences of his mother crying when hit by his father, in turn sometimes hit by patient while helping the mother. Hitting also had been helpful (cognitive mismatch between helpful and harmful hitting) during the victim's accidental choking 11 days earlier. This had occurred on the same day his distant mother died. Two days later he attempted suicide with anticonvulsants. Symptoms of the well remembered, unmotivated infanticide included flat affect, olfactory and command hallucinations, and delusions of grandeur (his mother leaving him millions and power).
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PMID:Infanticide in Limbic (?) Psychotic Trigger Reaction in a man with jacksonian and petit mal (?) seizures: "kindling" by traumatic experiences. 212 78

In awake rats, ranitidine was more effective than cimetidine in elevating blood pressure following intracerebroventricular (i.c.v.) injection, yet neither drug affected the hypotensive response to subsequent injections of muscimol (8.8 nmol i.c.v.). Bicuculline (0.01 nmol) microinjected into the inferior colliculus of rats caused clonic seizures whereas cimetidine (100 nmol) had no effect. The antihistamines did not prevent GABAB receptor-mediated inhibition of twitch responses in transmurally stimulated guinea-pig ileum. Ranitidine potentiated rather than inhibited GABAA receptor-mediated contractions of ileum longitudinal muscle. Cimetidine had no effect on these responses except at high concentrations (3 X 10(-4) M) which caused a slight dextral shift in the contractile response curve for GABA that may be attributed to antimuscarinic actions of cimetidine. Taken together, these data do not support the concept that the centrally mediated pressor effects of H2 antagonists are caused by GABA receptor blockade.
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PMID:Pressor responses to central injection of H2 antagonists not caused by GABA blockade. 287 34

The authors describe a case of Cushing's duodenal ulcer associated with Encephalitis. Diagnosis was made as the following criteria: neurological manifestations (fever, seizures, coma) and clinical manifestations of ulcer (hematemesis and melena); endoscopic evidence of ulcer and demonstration of increased gastric acid secretion; direct correlation between neurological lesion and duodenal ulcer. The good therapeutic result obtained with Ranitidine was noteworthy. The high dosage (20 mg/kg/die) allowed the control of the bleeding and the ulcer cicatrization.
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PMID:[Cushing's ulcer in childhood. Description of a case]. 653 40

Of 901 patients investigated 167 showed a focal disturbance in the EEG before and/or after 24-hours sleep deprivation. 459 patients suffered from epileptic seizures, the remainder had non-epileptic attacks or an organic cerebral disease without seizures. Of the 167 focal EEG changes 21.6% were seen only following sleep deprivation. In 9.6% focal changes present before could not be observed anymore following sleep deprivation. There was no significant difference between the three groups of patients mentioned above. The 24-hours sleep deprivation thus represents an activation procedure which may enhance diagnostic information relating to the question of focal EEG disturbances not only in epileptic patients. In the group of patients with epileptic seizures the activation or suppression respectively of the focal disturbance showed some dependence from seizure type and frequency as well as from the type of the focal change (spikes or slow waves). In our material the differences were however not statistically significant. More information could be gained from the waking EEG and from stage 1 than from sleeping stages 2 to 4 according to Rechtschaffen and Kales. Trigger functions could be attributed especially to the transitional phases from wakefulness to sleep and from stage 1 to stage 2 and vice versa particularly associated with arousal reactions. In our short sleep records REM stages could be rarely observed.
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PMID:[Sleep deprivation as activating procedure in EEG of patients with and without epileptic seizures. II. Focal discharges ]. 712 67

This analysis provides a specific example of the generally applicable process of creative delineation of a novel pattern while searching for an explanatory hypothesis for puzzling observations. In so doing, the neglected retroductive form of inference or abduction was used. Central to such a process is the delineation of a specific "generative mechanism" capable of uniting and explaining heretofore unexplained phenomena. Herein the neurophysiologically known mechanism of limbic seizure "kindling" is offered as a unifying explanation for a dozen bizarre phenomena, proposed as a new subtype of partial seizures, "Limbic Psychotic Trigger Reaction." This new syndrome has been proposed over 15 years in 17 male social loners. Upon encounter with an individualized stimulus, which revived in memory prior moderately hurtful experiences, these men suddenly committed motiveless, unplanned acts with flat affect, transient psychosis and autonomic arousal, showing no quantitative impairment of consciousness and so without memory loss for their perplexing homicidal acts (13 cases), firesetting (3 cases), or bank robbery (1 case). Events occurred in three phases reminiscent of seizures: (1) aura-like puzzlement, (2) transient ictus with a limbic release of predatory or defensive aggression (circa 20 min.), and (3) postictal inefficient actions, implicating a transient frontal lobe system dysfunction secondary to the limbic hyperactivation. The 17 men were of diverse backgrounds, but all without history of prior violence or severe emotional trauma. Seven of 17 had some abnormal tests at some time during their lives and eight known histories of typically overlooked closed-brain injury. Brain damage may facilitate seizure "kindling" but has been traditionally observed in mammals and in a few humans without such damage.
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PMID:Retroductive reasoning in a proposed subtype of partial seizures, evoked by limbic "kindling". 777 May 94

Proust detailed inexplicable behavior long before the neurobiologists Goddard and McIntyre in 1972 demonstrated that intermittent repetition of harmless stimuli can cause "kindling" of a seizure (with or without motor convulsions). Such brief seizures can occur especially in the evolutionarily old limbic system which mediates basic drives, their concomitant emotions, and certain aspects of memory. It appears that in humans the influence of specific external stimuli that revive the memory of repeated past experiences may "kindle" a transient episode of limbic overactivation. Thereupon the normal balance between the limbic and frontal lobe systems is disturbed (for a few minutes) as are normal human decision making and control of action. Linked with such a transient frontolimbic imbalance is out-of-character behavior, psychosis (hallucinations or delusions), autonomic activation, and severe distortion of affect and of action, culminating in extreme cases in a "Limbic Psychotic Trigger Reaction," as proposed by Pontius in 1981, in motiveless homicidal acts with mostly preserved consciousness and memory for the acts.
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PMID:Overwhelming remembrance of things past: Proust portrays limbic kindling by external stimulus--literary genius can presage neurobiological patterns of puzzling behavior. 823 14

A neuropsychiatric and -psychological update of the crime "profile" and "signature" is a necessary addition to the traditional sociopsychological model likely to miss limbic system dysfunctioning. Thus, occurrence of a brief (c. 20 minutes) limbic seizure has been proposed based on behaviors of 12 white male homicidal loners, who showed a dozen symptoms and signs: Limbic Psychotic Trigger Reaction. Readily overlooked can be (a) a transient psychosis (hallucinations and/or delusions), (b) autonomic hyperactivation (e.g., loss of bladder control, nausea, ejaculation), (c) motiveless, out-of-character, unplanned, and well-remembered homicidal acts, (d) committed with a flat affect (not emotionally or impulsively provoked), (e) typically involving a stranger who happened to provide an objectively harmless and only subjectively important stimulus. (f) Such an individualized stimulus triggered the memory revival of mild to moderate but repeatedly experienced hurts. Such a specific sequence of events implicates the specific mechanism of limbic seizure, "kindling," which does not necessarily involve motor convulsions. Repetition of such limbic episodes with "criminal acts" is conceivable under specific circumstances including cases in which the triggering stimulus is associated with pleasurable delusions (e.g., of grandiose power or wealth) or constitutes a specific aspect of a basic drive motive. For example, eating or sexual activities might be planned but degenerate into a limbic episode with a specific core symptomatology.
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PMID:Neuropsychiatric update of the crime "profile" and "signature" in single or serial homicides: rule out limbic psychotic trigger reaction. 830 91

23 unselected juvenile firesetters (M age 12.0 yr.) consisted of seven with schizophrenia, three with organic mental disorder, six with posttraumatic stress disorder, two with severe mental retardation, and two with conduct disorders. Three previously nondestructive boys (M age 11.0 yr.), all of them loners, did not fit such traditional diagnoses. Their fleeting (c. 20 min.) symptoms included flat affect, autonomic arousal, and delusions or hallucinations. It appeared that their motiveless, unplanned acts were each preceded by a chance encounter with an individualized stimulus which revived the three boys' repeatedly ruminated memories of intermittently experienced merely moderate stresses associated with fire, smoke, or matches. Such a sequence of events is characteristic of seizure kindling. One boy's abnormal EEG was congruent with seizures in the temporal lobe area, which includes the amygdala, i.e., that part of the limbic system particularly susceptible to seizure kindling. The three boys' consistent symptomatology was very similar to that reported for 17 men with bizarre homicidal acts implicating a kindled partial seizure called "Limbic Psychotic Trigger Reaction." In primates, too, similar partial nonconvulsive "behavioral seizures" with psychosis-like symptoms can be elicited through experiential kindling.
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PMID:Motiveless firesetting: implicating partial limbic seizure kindling by revived memories of fires in "Limbic Psychotic Trigger Reaction". 1040 7

Acute necrotizing encephalopathy (ANE) is a devastating and rapidly progressive neurologic disorder that occurs in healthy children after common viral infections. Typically, ANE is sporadic and does not recur. However, familial (ANE1) and recurrent cases have been reported and were recently linked to mutations in RANBP2 (RAN-binding protein 2). We report here a multiply affected kindred with recurrent familial ANE. These affected male siblings (a set of twins and their older brother) all presented with prodromal fever and upper respiratory tract infection that progressed within 72 hours to seizures, coma, and ultimately death, a course that is typical of ANE. It should be noted that 1 brother was treated with early aggressive management, including corticosteroids, and he survived for an additional 5 years. This represents the second reported case of familial ANE in the United States and the only case of male siblings with consanguineous parents. We hope that early recognition and growing awareness can lead to more effective treatment and better outcomes in the future.
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PMID:Acute necrotizing encephalopathy in 3 brothers. 2014 83

Acute necrotising encephalopathy (ANE) is a rare encephalitis-like syndrome usually reported in East Asia. This clinical syndrome tends to be triggered by viral febrile illness with rapid deterioration to seizures, coma and a generally poor outcome. Diagnosis is usually made on Magnetic Resonance Imaging (MRI). Its epidemiology is unknown largely due to under-recognition. Recurrent ANE has recently been associated with a newly discovered autosomal dominant mutation RAN-binding protein 2 now termed ANE1. There had been reports encouraging the use of empirical corticosteroids as treatment for this condition. However, there have not been any clinical trials to date. Here we report an unusual case of a Caucasian toddler who had suffered two episodes of ANE, but did not receive any specific treatment and has normal physical and cognitive outcome at 1 year follow up. He has this missense mutation in the gene of the RAN-binding protein 2 as have his mother and brother who are both well. This case adds to the worldwide literature and expands on the spectrum of outcomes in order to bring about better recognition in the Caucasian population.
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PMID:Untreated recurrent acute necrotising encephalopathy associated with RANBP2 mutation, and normal outcome in a Caucasian boy. 2047 21


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