Gene/Protein Disease Symptom Drug Enzyme Compound
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Experimental seizures were induced in mice by application of 50 mA for 0.2 sec via corneal electrodes. The reproducible conclusive behavior was characterized by a sequence of 2 sec of tonic flexion, 13 sec of tonic extension, and 8 sec of clonus followed by a postictal depressive stage. The animals were frozen and tissues were prepared for analysis according to Lowry and Passonneau [Lowry, O. H. & Passonneau, J. V. (1972) A Flexible System of Enzymatic Analysis (Academic, New York)]. Freeze-dried samples (1-10 ng) of pyramidal cell bodies and adjacent neuropil from the parietal cortex and of Purkinje cell bodies and adjacent neuropil from the cerebellum were analyzed for glucose, ATP, and P-creatine (0.01-0.05 pmol). There were marked decreases in these energy stores after the maximal electroshock in three of the areas examined. In the Purkinje cell bodies, however, the metabolic stress was dampened; glucose concentrations decreased, but the levels of ATP were maintained and, to a lesser extent, those of P-creatine. The results indicate that the output from the Purkinje cells is less than in the other regions examined in the excitable stages of the convulsion. The lesser energy debt probably reflects lower energy demand as well as a lower discharge intensity. The fact that Purkinje cells are spared from the metabolic stress imposed on other regions may be a partial explanation of the seizure activity. A diminished output from the Purkinje cells could be a situation that permits cortical convulsive activity.
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PMID:Sparing of metabolic stress in Purkinje cells after maximal electroshock. 28 34

The contemporary behavior analyst, to operate ethically and effectively, must be aware of many more factors affecting behavior than simple consequences. Although the literature demonstrating the effectiveness of active behavior management is impressive, a compelling argument can be made that a great number of behavior problem seen in individuals with developmental disabilities may be attributable to factors other than consequences. Our experience has been more often than not that physiological, organic, medication, or situational variables are the actual culprits in maladaptive behavior. Individuals with severe or profound retardation may respond to aversive features of their environment by displaying noncompliance, tantrums, aggression, or self-injurious behavior. These antecedents can affect their behavior just as powerfully as can the consequences of their behavior. Behavior analysts must become sensitive to these potential factors and be prepared to employ behavioral diagnostic strategies in the search for the causes of maladaptive behavior. Finally, they must be prepared to design rather unconventional passive behavior management treatment programs involving the manipulation of the antecedent environment. In the case of Carrie, from the example at the beginning of this paper, the analysis yielded the hypothesis that her face scratching was a reaction to sinus blockage caused by seasonal allergies. Her treatment involved daily dosages of antihistamines administered by our nurses and subsequent elimination of the scratching. Tom was found to be suffering from "wheelchair fatigue." When he was allowed to recline on other surfaces (e.g., bean bag chair, mat, bolster) on a regular basis, he did not attempt any form of self-injury. Melissa was found to have a severe case of Pre Menstrual Syndrome as well as seizure disorder, and was treated with the appropriate medications. Her headbanging was reduced to a few minor incidents per month. Walter's tantrums on closer inspection seemed part of a chain of behavior leading to seizure-like attacks. Preliminary evidence suggests that when he is treated with phenobarbital the tantrums and aggression disappear. And finally, Debbie was found to be very sensitive to a variety of discomforting events. She would cry, sob, and scream when she was wet, thirsty, hungry, and tired. Changing her regularly, offering her water every hour and extra snacks in the morning as well as short naps in the early afternoon eliminated the crying and sobbing. She now participates with the other clients and seems to enjoy the house activities.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Behavioral diagnostics. 274 44

The P3 component to an auditory "oddball" stimulus was compared between 30 epilepsy patients and 27 age-matched normal controls. The P3 latency was significantly increased in the patients, but an increase beyond 3 standard deviations on two trials occurred in only 10 percent of the total sample and 13 percent when the age was restricted to less than 50 years. There were no significant differences in latencies for different seizure type or anticonvulsant levels. The best relationship on neuropsychologic performance was with the Trails B test. Since the exact latency of the P3 peak is at times difficult to determine and may involve arbitrary choices, it is proposed that when the test is used for clinical diagnostic purposes of dementia, the interpretation be limited to those instances where the P300 peak is either clearly normal or clearly present but prolonged beyond 3 standard deviations from age-matched normals. In the first instance, significant cognitive impairment is unlikely while it becomes very probable in the latter instance.
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PMID:The cognitive evoked potential in epilepsy patients. 275 89

Neuropsychological performance data from 106 children with epilepsy were evaluated to determine the effects of seizure type and age of onset. The performance of children with partial seizures (N = 49) was similar to that of children with generalized seizures (N = 57). Only one of 13 tests showed a significant difference between groups, with children with partial seizures performing better on that test. The effects of age of onset were also similar in the two seizure groups. Children whose seizures began before the age of 5 years performed significantly worse than children whose seizures began later on four measures (Verbal IQ, Performance IQ, Trails A, and Trails B) and performed more poorly, but not significantly so, on the other nine measures in the battery. A breakdown of the partial group into simple partial, complex partial, and secondarily generalized partial seizure groups found a significant difference between the groups on only one variable, but there were suggestions in the data that the performance of the partial secondarily generalized group was worse than the other two groups. These results indicate that variables associated with an early onset of seizures, regardless of type, place a child at risk for cognitive dysfunction.
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PMID:Effects of age of onset of partial and generalized seizures on neuropsychological performance in children. 641 48

Zenker's solution is a tissue fixative containing mercuric chloride, potassium bichromate, sodium sulfate, and glacial acetic acid. In 1956, Anderson and Johnson reported its use in clinical neurosurgery. They applied the solution to the exposed dura after craniectomy. Delayed bone formation was thought to be due to the suppression of the osteoblastic activity of the outer layer of the dura. The fixative has since become a well-accepted adjuvant to the treatment of craniosynostosis. In 1972, Pawl and Sugar reported postoperative seizures in 6 of 34 patients treated with this solution. They assumed that the fixative penetrated the dura and irritated or damaged the cortex. To clarify the effect of Zenker's solution on the underlying brain, we performed bilateral parasagittal craniectomies in a group of kittens and adult cats. Zenker's solution was applied to one side and the other side served as a control. The animals were killed after periods varying from 24 hours to 2 months. We then examined the cortex under the craniectomies. There was immediate breakdown of the blood-brain barrier, as evidenced by the penetration of intravenous Evans blue. In the postoperative period investigated, an inflammatory response in the underlying brain with thickening of the arachnoid occurred. The results and implications of these experiments are presented. (Neurosurgery, 6: 45--48, 1980)
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PMID:Effect of the dural application of Zenker's solution on the feline brain. 735

We describe a patient with progressive myoclonus epilepsy (late-onset Lafora's disease). Onset was in early adult life, and death was at age fifty-four. The initial symptoms were epileptic seizures and progressive dementia, with later occurrence of myoclonus. Lafora bodies were ubiquitous and in neuronal perikarya in many areas. Dust-like granular bodies predominated in the neuropil of cerebral cortex, substantia nigra, and striatum. Abnormal deposits were also found in the myocardium.
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PMID:Late-onset Lafora's disease with typical intraneuronal inclusions. 817 May 76

Both early (N1 and P2) and late (N2 and P3) event-related potentials (ERP) were obtained in 16 patients with complex partial seizures, 12 with left hemispheric ictal focus and 4 with right, to see if they help in lateralizing the seizure focus, and also to determine if they correlate with behavioral (MMPI, Bear-Fedio), attentional (Trails A and B), cognitive (WAIS-R, Boston Naming, Warrington Word and Face recognition) and mental speed (Stroop color naming and reading) tasks. Early waves were more often lateralized than late waves but both were often falsely lateralizing. Early waves were better correlated with behavioral tasks whereas late waves were better with those measuring mental speed, attention and cognition. These data tentatively discourage the utility of ERP in preoperative lateralization of seizure focus but argue for their potential value in psychophysiological correlations.
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PMID:Event-related potentials in complex partial seizures. 826 Mar 25

Consecutive series of 90 hips that had had a primary cementless arthroplasty with use of straight collarless stems between January 1988 and September 1992 were reviewed. Twenty-eight hips with Omnifit Normalized Stems (Fit-N) and 45 hips with Omniflex Normalized Stems (Flex-N) without porous coatings were compared clinically and radiographically with 17 hips with Omnifit Microstructured Stems (Fit-M) with porous coating. The average follow-up periods for the 3 stems were 97 months, 73 months, and 93 months. Revision was performed or awaited in 9 cases (32.1%) in the Fit-N group, 18 cases (40.0%) in the Flex-N group, and 1 case (5.8%) in the Fit-M group. The average annual subsidence was 0.62 mm/y, 0.73 mm/y, and 0.17 mm/y. Osteolysis of more than 3 mm occurred in 28.6%, 37.8%, and 23.5%. Fit-N stems and Flex-N stems without porous coatings are not appropriate for cementless total hip arthroplasty.
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PMID:Catastrophic failure of cementless total hip arthroplasty using a femoral component without surface coating. 1061 81

The nociferous cortex hypothesis predicts that electrophysiological normalization to distal extratemporal brain regions following anterior temporal lobectomy (ATL) will result in improvements in executive functioning. The present study examined the effects of seizure laterality and seizure control on executive function change. The authors administered the Wisconsin Card Sorting Test (WCST), Trails B, and the Controlled Oral Word Association Test to 174 temporal lobe epilepsy patients who underwent ATL. No significant changes were found on the WCST or Trails B tests, regardless of surgery side or seizure-free status. However, verbal fluency significantly improved in seizure-free patients. Findings were consistent with the nociferous cortex hypothesis suggesting selective executive function improvement following ATL. These findings are discussed in terms of recent research demonstrating extrahippocampal metabolic normalization following surgery.
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PMID:Investigation of executive function change following anterior temporal lobectomy: selective normalization of verbal fluency. 1105 52

This study was conducted to better define the pathophysiology, risk factors, and therapeutic approach to exercise-associated hyponatremia. Medical records from all participants in the 1998 Suzuki Rock 'N' Roll Marathon who presented to 14 Emergency Departments (EDs) were retrospectively reviewed to identify risk factors for the development of hyponatremia. Hyponatremic patients were compared to other runners with regard to race time and to other marathon participants seen in the ED with regard to gender, clinical signs of dehydration, and use of nonsteroidal anti-inflammatory drugs (NSAIDs). An original treatment algorithm incorporating the early use of hypertonic saline (HTS) was evaluated prospectively in our own ED for participants in the 1999 marathon to evaluate improvements in sodium correction rate and incidence of complications. A total of 26 patients from the 1998 and 1999 marathons were hyponatremic [serum sodium (SNa) < or =135 mEq/L] including 15 with severe hyponatremia (SNa < or = 125 mEq/L). Three developed seizures and required intubation and admission to an intensive care unit. Hyponatremic patients were more likely to be female, use NSAIDS, and have slower finishing times. Hyponatremic runners reported drinking "as much as possible" during and after the race and were less likely to have clinical signs of dehydration. An inverse relationship between initial SNa and time of presentation was observed, with late presentation predicting lower SNa values. The use of HTS in selected 1999 patients resulted in faster SNa correction times and fewer complications than observed for 1998 patients. It is concluded that the development of exercise-associated hyponatremia is associated with excessive fluid consumption during and after extreme athletic events. Additional risk factors include female gender, slower race times, and NSAID use. The use of HTS in selected patients seems to be safe and efficacious.
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PMID:Exercise-associated hyponatremia in marathon runners: a two-year experience. 1139 89


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