Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The mortality of 128 consecutive severely brain-injured patients (Glasgow Coma Score less than or equal to 8) treated by a land advanced life support system and transported to a trauma center was compared to the mortality of 104 consecutive severely brain-injured patients treated during the same time period by an advanced care rotorcraft aeromedical emergency service and transported to the same trauma center. The two patient groups had statistically similar distribution of CNS lesions and Glasgow Coma Scores. The mortality of the patients treated by the land ALS system was 40%. The mortality of the patients treated by the aeromedical service was 31%. The 9% reduction in mortality in the patients treated by the aeromedical service was statistically significant (p less than 0.001). There was also a statistically significant (p less than 0.05) reduction in the Glasgow Outcome Scores of the patients treated by the aeromedical service compared with patients treated by the land advanced life support system.
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PMID:The impact of advanced prehospital emergency care on the mortality of severely brain-injured patients. 357 84

Lesion site, etiology, and clinical signs were analyzed in 50 cases of unilateral or bilateral corneomandibular reflex. The reflex was seen most commonly in acutely ill patients with a reduced level of consciousness and elevated intracranial pressure (ICP). Large cerebral hemispheric lesions with secondary brainstem pressure, intrinsic lesions of the upper brain-stem or diencephalon, diffuse or metabolic processes (often accompanied by raised ICP), and involvement of corticobulbar pathways in amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS) were the most common settings where the reflex occurred. The sign is useful in distinguishing structural from metabolic processes in acutely comatose patients, localizing lesions to the upper brainstem area, determining the depth of coma and its evolution, providing evidence of uncal or transtentorial herniation in acute cerebral hemisphere lesions, and suggesting involvement above the spinal level in cases of ALS and MS.
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PMID:Clinical significance of the corneomandibular reflex. 711 48

The authors investigated gastric emptying in 18 elderly patients with cerebral vascular diseases using the acetaminophen method. Subjects were divided into 2 groups according to their levels of daily activity. One group consisted of 10 comatose patients (71-92 years old), the other consisted of 8 patients (74-95 years old) who could walk by themselves. We also investigated gastric emptying in 6 comatose patients (38-83 years old) because of other diseases such as amyotrophic lateral sclerosis and in 11 elder controls (75-95 years old). In elderly controls, the acetaminophen concentration at 45 minutes was 9.08 +/- 1.71 micrograms/ml. In comatose patients due to cerebral vascular diseases, the concentration was 3.89 +/- 1.60 micrograms/ml, which showed significantly delayed gastric emptying (p < 0.05). In patients with cerebral vascular diseases who could walk, the concentration was 6.51 +/- 0.99 micrograms/ml. In comatose patients by another diseases, the concentration was 5.82 +/- 1.13 micrograms/ml. We suspected that delayed gastric emptying is related to the comatose state. Trimebutine significantly (p < 0.01) improved gastric emptying in comatose patients with cerebral vascular diseases.
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PMID:[Gastric emptying in elderly patients with cerebral vascular diseases and the effect of trimebutine]. 847 27

Outcome after prehospital cardiac arrest was examined in the EMS system of Bonn, a midsized urban community, and presented according to the Utstein style. The data were collected from January 1st, 1989 to December 31st, 1992 by the Bonn-north ALS unit, which serves 240,000 residents. Fifty-six patients suffered from cardiac arrest of non-cardiac aetiology and were excluded; 464 patients were resuscitated after cardiac arrest of presumed cardiac aetiology (incidence of CPR attempts: 48.33 per year/100,000 population). The collapse was unwitnessed, bystander witnessed or EMS personnel witnessed in 178, 214 or 72 patients, respectively. In these subgroups discharge rates and 1-year survival accounted for 7.3% (4.5%), 22.9% (15.9%) and 16.7% (11.1%), respectively. Thirty-four patients were discharged without neurological deficits (cerebral performance category 1: CPC 1), 22 and nine patients scored CPC 2 or CPC 3, respectively. Nine patients were comatose (CPC 4) when they were discharged and remained in this state until they died. Of the 50 1-year survivors 35 lived without neurological deficit, eight demonstrated mild (CPC 2) and five severe (CPC 3) cerebral disability at 1-year after resuscitation, and, finally, two patients remained comatose for more than 1 year. The Utstein template recommends the selection of patients who were found in VF after bystander witnessed collapse. In our cohort 118 patients met these criteria. Of them 41 (35%) could be discharged from hospital and 28 (24%) lived more than 1 year. The comparison of our data with those from double-response EMS systems of other communities revealed that, in midsized urban and suburban communities the highest discharging rates could be achieved. Our study demonstrated that survival depends crucially on short response intervals and life support which will be performed by well-trained emergency technicians, paramedics and physicians.
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PMID:One-year survival after out-of-hospital cardiac arrest in Bonn city: outcome report according to the 'Utstein style'. 904 96

We studied the admission rate, risk factors, neurological complications and sequelae of heat stroke (HS) during the 1995 heat wave in Madison, Wisconsin. HS was epidemic in 1995 (2.3 cases/1000 admissions), compared to the ten-fold lower endemic rate in 1994 (0.2/ 1000). There were 11 cases of HS, 9 males and 2 females. Contributing factors were athletic events (2), working outdoors (3) and indoor activity with malfunctioning air-conditioning (6). Medical conditions contributing to poor temperature regulation included schizophrenia with neuroleptic treatment (2), amyotrophic lateral sclerosis receiving nortriptiline (1), multiple sclerosis (1), attention deficit disorder (1), cystic fibrosis (1) and alcoholism (1). Acute neurological complications occurred in all patients on presentation including coma (8/11.73%), stupor (2/ 11.18%) and seizures (1/11.9%). Two patients (1856) had persistent neurological sequelae in the form of a pan-cerebellar syndrome while the remaining 9 recovered fully. Importantly, avoidable factors contributed to all of the patients with underlying diseases. These patients are particularly at risk and should take adequate precautions during summer months.
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PMID:Epidemic heat stroke in a midwest community: risk factors, neurological complications and sequelae. 916 37

We report a 49-year-old man with progressive bulbar palsy and respiratory failure. He was well until his 48 years of the age (December 1994) when he noted a difficulty in speaking in loud voice. In February, 1995, he noted regurgitation of foods to his nose and difficulty in his speech. He was admitted to our service in May 29, 1995. On admission, he was alert and oriented to all spheres and he was not demented. His higher cerebral functions were normal. In cranial nerves, he showed dysarthria and dysphagia; muscle atrophies were seen in the tongue, the bilateral sternocleidomastoid, supraspinatus, and infraspinatus muscles. Fasciculations were seen in these muscles. He showed no muscle weakness in his limbs except for the upper limb girdle muscles, no ataxia, no reflex abnormalities, nor sensory changes. EMG showed neurogenic changes in the affected muscles. MRI of the brain and the spinal cord was entirely normal. He was discharged for out patient follow-up, however, in October of 1995, he noted difficulty in swallowing solid foods. Gastrostomy was placed and he was discharged to his home. In February 11th of 1996, he was found unresponsive and brought into the ER of our hospital. On admission, he was comatose without spontaneous respiration. BP could not be obtained. He was immediately intubated and artificial ventilation was started. On the following morning, he became alert and he was not demented. He continued to show marked dysarthria and dysphagia; again no weakness was noted in the distal parts of the upper and lower extremities. Laboratory examination showed increase in serum CK to 2,173 IU/L and amylase to 2,032 IU/L. He was extubated on February 15th, however, his spontaneous respiration was not suffice to maintain his blood gas. According to his will, he was not placed on respirator and he died on February 24th, 1996. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had ALS. Although no upper neuron signs were observed clinically, it is not uncommon to see degeneration in the corticospinal tract in post-mortem examination. The question was what might have been the cause of increase in CK and amylase. Many participants thought that they were secondary to multiple organ failure due to prolonged hypoxic state at his last admission; other possibilities raised included acute myocardial infarction and acute bowel necrosis. Post-mortem examination revealed muscle atrophy in the facial, lingual, cervical, intercostal, and the upper limb girdle areas. The lungs were unremarkable except for old organized pneumonic foci in the right middle and lower lobes. Marked to moderate congestion was seen in many internal organs, however, no other gross abnormality was found. It was thought that respiratory palsy itself was the direct cause of his agonal event. In the spinal cord, the anterior horns showed various degree of neuronal loss and gliosis. No clear evidence of pyramidal tract degeneration was seen at the light microscope level. Lower brain stem motor neurons were markedly reduced. But no Bunina body was found. The substantia nigra showed moderate degree of neuronal loss and extraneuronal neuromelanins. The locus coeruleus showed similar but milder changes. The degree of nigral degeneration appeared to be well beyond those which could be seen in usual ALS patients. The question was whether or not this patient might have been in an early stage of the extended form of ALS.
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PMID:[A 49-year-old man with progressive bulbar palsy and respiratory failure]. 949 5

Muscle spasticity causes pain, disability, and difficulties in the rehabilitation of patients with cerebrovascular lesions, head, brain or spine trauma, coma, or neurologic diseases such as multiple sclerosis, amyotrophic lateral sclerosis, or cerebral palsy. Regional blocks have a threefold use in patients with painful spasticity: diagnostic, prognostic, and therapeutic. Blocks are feasible on an outpatient or day-hospital basis. Blocks are applied most often to 4 peripheral sites: the pectoral nerve loop, median, obturator, and tibial nerves. The main indication is debilitating or painful spasticity. Peripheral blocks with local anesthetics are used as tests, to mimic the effects of motor blocks and determine their potential adverse effects, transiently and reversibly. Peripheral neurolytic blocks are easy to perform, effective, and inexpensive.
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PMID:[Peripheral nerve blocks and spasticity. Why and how should we use regional blocks?]. 1877 34

Corneomandibular reflex is a pathological phenomenon evident in cases of severe brainstem damage. It is considered to be a pathological exteroceptive reflex, associated with precentro bulbar tract lesions. The sign is useful in distinguishing central neurological injuries to metabolic disorders in acutely comatose patients, localizing lesions to the upper brainstem area, determining the depth of coma and its evolution, providing evidence of uncal or transtentorial herniation in acute cerebral hemisphere lesions, and it is a marker of supraspinal level impairment in amyotrophic lateral sclerosis and multiple sclerosis. This sign was evident in a patient with severe brain damage. We discuss the literature findings and its relevance in prognosis establishment.
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PMID:Corneomandibular reflex: Anatomical basis. 2675 10

Immortality is thought to be achieved through heroic deeds, reincarnation, and the afterlife. The present studies reveal an alternative path to transcending death: dying while conscious. Seven studies demonstrate that dying while more awake, aware and/or lucid leads people to see a richer postmortem mind-an effect we call conservation of consciousness. People see more mind in the deceased when they die with their eyes open (Study 1), and while awake (vs. in a coma), while suffering from ALS (vs. from Alzheimer's), while on hallucinogens (vs. sedatives), and while dreaming (vs. in a deep sleep; Study 2). This effect is robust, holding even in a between-subjects design, and even when participants are explicitly encouraged to interpret the mind perception items literally (Study 3). Perceived conservation of consciousness after death is driven more by general perceived awareness than by fear of death (Study 4) and predicts perceptions of mind beyond having a vivid (vs. dull) life (Study 5). The last wishes of the dying are also given more moral weight if made by those who ultimately die while conscious (Study 6). Perceived conservation of consciousness also occurs in the real-world context of a historic cemetery (Study 7). These results reveal a simple way to increase your influence after death and highlight both the power of endings and the subjective nature of mind.
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PMID:How people perceive the minds of the dead: The importance of consciousness at the moment of death. 3238 22