Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A girl of 10-5/12 years is described, who had diabetes mellitus from the age of 5 years on and who developed bilateral ptosis, pigment degeneration of the retina and bilateral impairment of hearing at the age of nine years. A few weeks before death she suffered from an acute gastrointestinal infection which was successfully treated by a hydroxyquinoline derivative. In the days following a severe encephalopathy and signs of cardiac involvement appeared. A month later the girl died of bulbar paralysis and acute heart failure. Histology showed remnants of a granulomatous inflammation in the heart, the kidneys, the pancreas and the skeletal muscles. Furthermore there was a widespread spongiosis in the white substance of the brain, with large astrocytes, and partly also in the basal ganglia, the brain stem and the cerebellum. Foci of sudanophilic tissue necrosis resembling Wernicke's Encephalopathy were found in the medulla oblongata and the spinal cord. The peripheral nerves appeared partially demyelinated and showed axonal lesions. This case is classified as a Juvenile Type of so-called Canavan's Disease. It shows some resemblence to the "Progressive Chronic Ophthalmoplegia with Spongiform Encephalopathy described by Daroff, Kearn and Sayre. The possible neurotoxical effects of the hydroxyquinoline therapy are discussed.
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PMID:[Juvenile spongy dystrophy of CNS with necrosis of the medulla. A. complication of hydroxyquinoline therapy (author's transl)]. 124 13

The most common cause of bilateral internuclear ophthalmoplegia is multiple sclerosis. Wernicke's encephalopathy has been reported as a cause of unilateral internuclear ophthalmoplegia but not of bilateral internuclear ophthalmoplegia. In this report, we present the case of a patient with a history of alcohol abuse and acute onset of bilateral internuclear ophthalmoplegia whose clinical course and diagnostic studies are most consistent with a diagnosis of Wernicke's encephalopathy.
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PMID:Bilateral internuclear ophthalmoplegia in a patient with Wernicke's encephalopathy. 162 72

A pilot study of 31 consecutive alcohol-related deaths over an 8-month period revealed the presence of histologically diagnosed Wernicke's encephalopathy in 17 cases. Analysis of the clinical records revealed that a disturbance of the mental state was the commonest finding and neurological signs were present in only 2 of the 17 cases (ataxia 1, peripheral neuropathy 1). Analysis of 22 ward admissions for Wernicke's encephalopathy during the same 8-month period revealed that the diagnosis is easily made when neurological deficits (ophthalmoplegia, ataxia) accompany mental changes and when Wernicke's encephalopathy is the predominant illness. In patients with established alcohol-related disease attention is often directed to the presenting illness so that Wernicke's encephalopathy may easily be overlooked as a cause of deterioration in the mental state in these patients. It is recommended that routine management of patients with alcohol-related disease should include thiamine even if neurological signs are absent.
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PMID:Wernicke's encephalopathy and alcohol-related disease. 177 21

Clinical and postmortem findings of a case that had combined alcoholic pellagra encephalopathy and Wernicke disease are described. This 51-year-old malnourished and chronic alcoholic man presented with progressive mental deterioration, pellagra dermatitis, hypertonus of the neck and other musculatures, myoclonic jerks with bizarre involuntary movements, in addition to total external ophthalmoplegia and gait disturbance. After administration of multivitamins, including thiamine and nicotinamide, these neurologic abnormalities were dramatically improved in a few days. However, the patient died thereafter because of sepsis associated with pneumonia. Postmortem examination revealed marked abnormalities in CNS, characterized by diffuse atrophy of gray matter and widespread neuronal degeneration and characteristic central chromatolysis in pontine nuclei, dentate nuclei, cranial nerve nuclei in the brain stem, Betz cells of the cerebral cortex, and Clarke's column and anterior horn cells of the spinal cord. There were also atrophy and gliosis of the mammillary bodies, degeneration and vascular proliferation of periaqueductal gray matter, and massive gliosis around the third ventricle. These neuropathological changes were compatible with symptoms of both alcoholic pellagra encephalopathy and Wernicke's disease, but they were also strongly suspected on clinical grounds.
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PMID:Alcoholic pellagra encephalopathy combined with Wernicke disease. 188 55

Author has noticed bilateral ophthalmoplegia in consequence of B1 avitaminosis due to alcoholism. Recognition of the cerebral beriberi with ophthalmoplegia as a leading symptom (Wernicke's encephalopathy) is easy. The importance of the diagnosis is that this life threatening condition can be abolished promptly by the administration of Vitamin B1. On the basis of the frequent occurrence of alcoholism it seems likely, that several similar beriberi cases could be found nowadays.
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PMID:[Cerebral beriberi with ophthalmoplegia as the leading symptom in an alcoholic patient]. 195 87

A young obese female with acute pancreatitis complicated by pseudocyst formation and intermittent gastric outlet obstruction, who had been maintained on high-calorie enteral feeds, developed a sudden onset of confusion and ophthalmoplegia associated with papilloedema and retinal haemorrhages. A possible diagnosis of Wernicke's encephalopathy (WE) was made, and the patient was treated with parenteral thiamine. Clinical resolution was complete. Any patient with suspicious or unusual neurological symptoms and signs associated with possible malnutrition, hyperemesis or malabsorption should be given intravenous thiamine without delay to avoid the potential morbidity and mortality associated with undiagnosed WE.
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PMID:Wernicke's encephalopathy in association with complicated acute pancreatitis and morbid obesity. 210 39

Wernicke's encephalopathy should be considered as a possible diagnosis in comatose and hypothermic patients. The classic triad of confusion, ophthalmoplegia (or nystagmus) and ataxia may be absent, and the history of alcohol abuse or other causes of thiamine deficiency may be unknown. Left untreated, acute Wernicke's encephalopathy has a 17 percent mortality rate. Since the morbidity from Wernicke's encephalopathy is potentially reversible with parenteral thiamine, and large doses of thiamine can be given without documented ill effects, it is recommended that all comatose or hypothermic patients, as well as those with more classic presentations of Wernicke's encephalopathy, be given parenteral thiamine before administration of glucose.
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PMID:Wernicke's encephalopathy. 218 37

Wernicke encephalopathy is a disorder caused by a deficiency of thiamine which is a cofactor of several metabolic enzymes. The symptoms include mental confusion, ataxia, and ocular signs in adults, infants, and children. Patients often have somnolence and weakness combined with ophthalmoplegia. Alcoholics are the best known risk group; however, Wernicke encephalopathy occurs in poorly nourished patients of all ages. We present 2 children with malignant disease in whom Wernicke encephalopathy--an underdiagnosed and potentially fatal, but preventable and treatable disease--was diagnosed postmortem.
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PMID:Wernicke encephalopathy--a preventable cause of death: report of 2 children with malignant disease. 267 84

Records of the emergency medical admissions to a large teaching hospital over a one year period were examined for evidence of Wernicke's encephalopathy or Korsakoff's syndrome. It was found that only 0.4% of the population studied had the classical triad of Wernicke's encephalopathy, namely confusion, ophthalmoplegia, and ataxia. If two of these three criteria are allowed in the absence of other causes then 2.2% of the population had this limited Wernicke's encephalopathy or Korsakoff's syndrome. It is concluded that the diagnosis of Wernicke's encephalopathy should not rely on the presence of all three criteria; any two of the three in the absence of other causes will suffice for the diagnosis.
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PMID:Diagnosis of Wernicke's encephalopathy. 346 66

A 21 year old male, well-nourished and non-alcoholic, died after five weeks illness. He had suffered epileptic fits, bilateral internuclear ophthalmoplegia, bulbar and pontine paralysis, tetraparesia, ataxia and dystonia. A CT brain scan showed low density lesions of the striatum bilaterally. Post-mortem studies revealed pathological anomalies compatible with Leigh's disease, although the presence of haemorrhages and involvement of the mamillary bodies could also suggest Wernicke's encephalopathy.
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PMID:Necrotising haemorrhagic encephalomyelopathy in an adult: Leigh's disease. 357 37


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