Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report a case of a 33-year-old man who presented, during recovery from coma due to severe head injury, dysphagia and respiratory failure. Magnetic resonance, retrograde radionuclide myelography and computerized tomographic myelography identified a pseudomeningocele in the retropharyngeal space due to a tear of the left C2 radicular sleeve. After failed medical management, the patient underwent lumbo peritoneal shunt. Magnetic resonance controls showed progressive collapse of the collection. After 3 months the patient was able to breathe spontaneously and to swallow. The authors describe pathogenesis, diagnostic strategy and principles of treatment of traumatic retropharyngeal pseudomeningoceles.
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PMID:Post traumatic retropharyngeal pseudomeningocele. 1519 18

Hydrogen peroxide is an oxidising agent that is used in a number of household products, including general-purpose disinfectants, chlorine-free bleaches, fabric stain removers, contact lens disinfectants and hair dyes, and it is a component of some tooth whitening products. In industry, the principal use of hydrogen peroxide is as a bleaching agent in the manufacture of paper and pulp. Hydrogen peroxide has been employed medicinally for wound irrigation and for the sterilisation of ophthalmic and endoscopic instruments. Hydrogen peroxide causes toxicity via three main mechanisms: corrosive damage, oxygen gas formation and lipid peroxidation. Concentrated hydrogen peroxide is caustic and exposure may result in local tissue damage. Ingestion of concentrated (>35%) hydrogen peroxide can also result in the generation of substantial volumes of oxygen. Where the amount of oxygen evolved exceeds its maximum solubility in blood, venous or arterial gas embolism may occur. The mechanism of CNS damage is thought to be arterial gas embolisation with subsequent brain infarction. Rapid generation of oxygen in closed body cavities can also cause mechanical distension and there is potential for the rupture of the hollow viscus secondary to oxygen liberation. In addition, intravascular foaming following absorption can seriously impede right ventricular output and produce complete loss of cardiac output. Hydrogen peroxide can also exert a direct cytotoxic effect via lipid peroxidation. Ingestion of hydrogen peroxide may cause irritation of the gastrointestinal tract with nausea, vomiting, haematemesis and foaming at the mouth; the foam may obstruct the respiratory tract or result in pulmonary aspiration. Painful gastric distension and belching may be caused by the liberation of large volumes of oxygen in the stomach. Blistering of the mucosae and oropharyngeal burns are common following ingestion of concentrated solutions, and laryngospasm and haemorrhagic gastritis have been reported. Sinus tachycardia, lethargy, confusion, coma, convulsions, stridor, sub-epiglottic narrowing, apnoea, cyanosis and cardiorespiratory arrest may ensue within minutes of ingestion. Oxygen gas embolism may produce multiple cerebral infarctions. Although most inhalational exposures cause little more than coughing and transient dyspnoea, inhalation of highly concentrated solutions of hydrogen peroxide can cause severe irritation and inflammation of mucous membranes, with coughing and dyspnoea. Shock, coma and convulsions may ensue and pulmonary oedema may occur up to 24-72 hours post exposure. Severe toxicity has resulted from the use of hydrogen peroxide solutions to irrigate wounds within closed body cavities or under pressure as oxygen gas embolism has resulted. Inflammation, blistering and severe skin damage may follow dermal contact. Ocular exposure to 3% solutions may cause immediate stinging, irritation, lacrimation and blurred vision, but severe injury is unlikely. Exposure to more concentrated hydrogen peroxide solutions (>10%) may result in ulceration or perforation of the cornea. Gut decontamination is not indicated following ingestion, due to the rapid decomposition of hydrogen peroxide by catalase to oxygen and water. If gastric distension is painful, a gastric tube should be passed to release gas. Early aggressive airway management is critical in patients who have ingested concentrated hydrogen peroxide, as respiratory failure and arrest appear to be the proximate cause of death. Endoscopy should be considered if there is persistent vomiting, haematemesis, significant oral burns, severe abdominal pain, dysphagia or stridor. Corticosteroids in high dosage have been recommended if laryngeal and pulmonary oedema supervene, but their value is unproven. Endotracheal intubation, or rarely, tracheostomy may be required for life-threatening laryngeal oedema. Contaminated skin should be washed with copious amounts of water. Skin lesions should be treated as thermal burns; surgery may be required for deep burns. In the case of eye exposure, the affected eye(s) shod eye(s) should be irrigated immediately and thoroughly with water or 0.9% saline for at least 10-15 minutes. Instillation of a local anaesthetic may reduce discomfort and assist more thorough decontamination.
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PMID:Hydrogen peroxide poisoning. 1529 93

Double aortic arch (DAA) is a complete form of vascular ring causing tracheoesophageal compression. We analyzed long-term results of a series of DAAs, over a period of 16 years. Between 1987 and 2003, 29 children underwent surgery for airway and/or esophageal compression secondary to a DAA. Dominant symptoms were stridor, dysphagia, choking episodes, and life-threatening apneic spells (n = 7). Diagnosis was established by barium studies, bronchoscopy, echocardiogram, angiogram, computed tomography (CT), and magnetic resonance imaging (MRI). Seven patients had concurrent cardiac anomalies. Two children had an associated tracheoesophageal fistula. Surgery was accomplished by left thoracotomy (n = 25), right thoracotomy (n = 2) or median sternotomy (n = 2). The operative mortality was zero. There was one late death due to respiratory failure. Four (13.8%) patients had a surgical complication (chylothorax, 3 cases; acute renal failure, 1 case). Follow-up (mean 7.1 years; range 6 months to 16 years) was complete in all patients, and showed complete improvement in 22 patients and partial improvement in 6 patients. Early surgical repair of DAA is associated with low mortality, and results in marked symptomatic relief in most patients. Patients with tracheomalacia or associated asthma, constitute a high-risk group and may manifest persistent symptoms and require adjunctive procedures.
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PMID:Surgical repair of double aortic arch: 16-year experience. 1579 42

We report a case of previously healthy student with acute rhombencephalitis and brainstem abscess caused by Listeria monocytogenes. The disease begun with uncharacteristic prodromal symptoms of gastrointestinal infection followed by headache and vertigo. After hospital admission the patient rapidly deteriorated, presenting pronounced dysphagia and respiratory failure requiring mechanical ventilation. The diagnosis was established upon clinical symptoms of infection, brainstem involvement, typical MRI findings and positive for L. monocytogenes blood culture. Infection was complicated by acute, demyelinating neuropathy, diagnosed upon clinical symptoms of frail palsy confirmed by ENG. Initially introduced empirical doxycyclin/ceftriaxon treatment was subsequently changed to targeted ampicillin/gentamycin therapy, mechanical ventilation, intravenous human immunoglobulin treatment, tracheostomy and endoscopic gastrostomy. Prolonged dysphagia resolved after rehabilitation. After one year the patient remains well with only slight dysmetria.
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PMID:Prolonged dysphagia due to Listeria-rhombencephalitis with brainstem abscess and acute polyradiculoneuritis. 1626 41

The Chiari type 1 malformation is common. Unlike the Chiari type 2 and 3 malformations, it may remain latent for a long time, becoming symptomatic only in adulthood. The introduction of MRI has resulted in an increased number of diagnoses of this malformation in pediatric patients. It appears to be related to underdevelopment of the posterior cranial fossa. It must be differentiated from acquired tonsillar herniation, particularly when herniation results from intracranial hypotension; these cases are sometimes reported as acquired Chiari I malformation with spontaneous resolution. Tonsillar ectopia may cause symptoms by its direct effect on any or all of the medulla and the cerebellar and upper spinal cord. The most suggestive of the oculomotor disturbances is oscillopsia with downbeat nystagmus. Dysphonia and dysphagia are common. Potentially serious autonomic disturbances are also frequent: sleep apnea, respiratory failure, syncope and even sudden death. Another risk is syrinx formation, resulting from obstruction of CSF circulation in the cisterna magna. Syringomyelia is detected in 32 to 74% of patients with Chiari I malformation. Treatment is surgical. Posterior fossa decompression is achieved by suboccipital craniectomy combined with laminectomy of the upper cervical segments. Surgical intervention is indicated when the malformation is symptomatic and there is no doubt that it is the cause of the symptoms. When a Chiari I malformation is identified fortuitously on MRI, long-term monitoring is essential. The risk of developing symptoms increases over time. Patients should be advised not to participate in contact sports.
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PMID:[Chiari type 1 malformation and magnetic resonance imaging]. 1632 7

Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motoneuron disease caused by a CAG-repeat expansion in the androgen receptor (AR) gene and for which no curative therapy exists. However, since recent research may provide opportunities for medical treatment, information concerning the natural history of SBMA would be beneficial in planning future clinical trials. We investigated the natural course of SBMA as assessed by nine activities of daily living (ADL) milestones in 223 Japanese SBMA patients (mean age at data collection = 55.2 years; range = 30-87 years) followed from 1 to 20 years. All the patients were diagnosed by genetic analysis. Hand tremor was an early event that was noticed at a median age of 33 years. Muscular weakness occurred predominantly in the lower limbs, and was noticed at a median age of 44 years, followed by the requirement of a handrail to ascend stairs at 49, dysarthria at 50, dysphagia at 54, use of a cane at 59 and a wheelchair at 61 years. Twenty-one of the patients developed pneumonia at a median age of 62 and 15 of them died at a median age of 65 years. The most common cause of death in these cases was pneumonia and respiratory failure. The ages at onset of each ADL milestone were strongly correlated with the length of CAG repeats in the AR gene. However CAG-repeat length did not correlate with the time intervals between each ADL milestone, suggesting that although the onset age of each ADL milestone depends on the CAG-repeat length in the AR gene, the rate of disease progression does not. The levels of serum testosterone, an important triggering factor for polyglutamine-mediated motoneuron degeneration, were maintained at relatively high levels even at advanced ages. These results provide beneficial information for future clinical therapeutic trials, although further detailed prospective studies are also needed.
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PMID:Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients. 1662 16

Tetanus is still widely prevalent in many parts of the world especially in the developing countries. This study was conducted to assess the demographic and clinical features, treatment and outcome of tetanus in adults with a special emphasis on identification of predictors of mortality. This was a retrospective study of all adult patients admitted with tetanus over a three-year period from 1998 to 2000 at Lok Nayak Hospital, New Delhi, India. The demography, clinical features, treatment and complications of the patients were assessed and were evaluated against two possible outcomes- survival or death, to identify the predictors of mortality. There were 217 adults (148 males, 69 females) admitted with generalized tetanus during the study period. The mean age of the patients was 36.08 +/- 18.84 years. In most instances (63.5%), wounds / injuries served as the source of infection. Overall 31.8% had mild, 21.7% had moderate and 46.5% had severe disease at presentation. Respiratory failure and dysautonomia were the frequently observed complications. Of the 217 patients, 82 died - a mortality rate of 37.78%. Parameters that were significantly associated with increased mortality were: age (especially > 60 years); shorter duration of symptoms - trismus, rigidity and dysphagia; severe disease at presentation and shorter period of onset. Tetanus is a preventable disease associated with a high fatality rate. Universal coverage of immunization programme and appropriate wound prophylaxis is the corner stone of tetanus eradication. The treatment of tetanus is difficult and requires specialized and intensive care. The presence of parameters indicating an adverse outcome at the beginning in the patients will help identify the subset of patients that need more intensive management especially in resource poor nations.
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PMID:Tetanus in adults--a continuing problem: an analysis of 217 patients over 3 years from Delhi, India, with special emphasis on predictors of mortality. 1670 28

We describe an autopsy case of MM1-type sporadic Creutzfeldt-Jakob disease (CJD), the duration of which was 93 days. The patient was a 59-year-old Japanese man with no family history of prion disease or known iatrogenic exposure to CJD. His first symptom was dysesthesia in the left arm, suggestive of cervical cord involvement, and he showed rapidly progressive neurologic signs, such as dysarthria, dysphagia, lethargy, sleep apnea and respiratory failure, suggestive of brainstem involvement. Progressive mental deterioration combined with episodes of myoclonic seizure and periodic synchronous discharges on the electroencephalogram were observed in the later disease stage. Autopsy showed typical spongiform change to be wide-spread in the cerebral and cerebellar cortices, thalamus and basal ganglia. Synaptic-type PrP deposition was marked in the cerebral cortex, thalamus and basal ganglia. In the cerebellum, although the granular, molecular and Purkinje cell layers were well preserved from neuronal loss and gliosis, PrP deposition was marked in the molecular and granular cell layers. Spongiform degeneration and neuronal loss were not seen in the brainstem and spinal cord, but relatively marked PrP deposition was observed in the quadrigeminal body, substantia nigra, pontine nucleus, inferior olivary nucleus and posterior horn. Immunohistochemical staining for HLA-DR showed proliferation of activated microglia in the cerebral and cerebellar cortices, pontine nucleus, inferior olivary nucleus and posterior horn. The mechanisms underlying the neurologic symptoms and signs were unclear, but we speculate that, in addition to widespread involvement of the cerebral cortex, PrP deposition and microglial activation in the brainstem and spinal cord were responsible.
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PMID:Autopsy case of sporadic Creutzfeldt-Jakob disease presenting with signs suggestive of brainstem and spinal cord involvement. 1720 92

Lance-Adams syndrome, described in 1963, is caused by anoxia of central nervous system, generally in the course of primary respiratory failure. It is characterized mainly by action myoclonus, associated cerebellar ataxia and very mild intellectual deficit. Occurrence of Lance-Adams syndrome is rare; about 100 cases have been described yet. The authors present the case of Lance-Adams syndrome in 36-year-old woman with many years' bronchial asthma. Three times acute cardiopulmonary arrest appeared during status asthmaticus. After successful cardiopulmonary resuscitation action myoclonus developed with cerebellar syndrome, aphonia, dysphagia and generalized convulsive seizures of tonic-clonic type. Electroencephalography showed polyspikes and complex of polyspikes-slow wave, synchronized with myoclonus. CT of the brain was normal. Action myoclonus responded appropriately to sodium valproate. The authors indicate the importance of the correct diagnosis and proper treatment.
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PMID:[Lance-Adams syndrome in patient with anoxic encephalopathy in the course of bronchial asthma]. 1720 60

The case of a term, male neonate (birthweight 3785g) with cephalic presentation, Caesarean-section (C-section) delivery, and failure to thrive is reported. The infant presented with generalized hypotonia and respiratory failure immediately following birth. An initial diagnosis of hypoxic-ischemic encephalopathy was made. However, ventilator dependency and slow recovery of generalized tonus over the following weeks could not be explained. Late cervical magnetic resonance imaging showed extensive syringomyelia from C2 to C7. To the authors' knowledge, this is the first report of syringomyelia after a C-section delivery following cephalic presentation without any associated abnormalities. Follow-up at 2 years of age revealed no improvement on neurological examination: poor head control, difficulty swallowing, flaccid paralysis of upper limbs, and spasticity of lower limbs with exacerbated deep reflexes and spontaneous clonus. Difficulties in establishing the diagnosis and managing the case are discussed.
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PMID:Syringomyelia and chronic respiratory failure in a term infant delivered by Caesarean section. 1759 28


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