Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During childhood, Wilson's disease becomes manifest mostly in the hepatic form. In children every case of cirrhosis of the liver, hemolysis with high levels of conjugated bilirubin in the serum, and otherwise in explicable tremor make it imperative to exclude or confirm the existence of Wilson's disease. A false diagnosis often delays the start of therapy with d-penicillamine and low-copper diet. The prognosis, which was still fatal a few years ago, has improved considerably thanks to new therapeutic possibilities.
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PMID:[Morbus Wilson--pathogenesis, diagnosis, therapy, and course (author's transl)]. 60 Feb 71

A patient with cryptogenic cirrhosis was found to have corneal pigmentation rings indistinguishable from Kayser-Fleischer rings on slit-lamp examination. Although she had hepatic encephalopathy that included confusion, tremor, and slurred speech, diagnosis of Wilson's disease was ruled out because urinary cooper excretion and hepatic copper concentrations were below the range found in symptomatic Wilson's disease. The exact nature of these rings could not be determined, and they were considered as Kayser-Fleischer-like rings.
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PMID:Kayser-Fleischer-like ring in a cryptogenic cirrhosis. 71 54

The clinical syndrome of portal-systemic encephalopathy is caused by far advanced cirrhosis of the liver in most cases; it is characterized by increasing drowsiness, disturbances of mentation, flapping tremor and hyperreflexia. An early diagnosis can be established by testing writing and drawing abilities. Increased occurrence of spider nevi, a dry, deep red tongue, and hemorrhagic lesions of skin and mucous membranes are symptoms of incipient hepatic insufficiency. The syndrome is initiated in most cases by excessive intake of protein or alcohol, by intestinal bleeding, by diuretics, or by intercurrent infections. Therapy has to include elimination of causes, reduced intake of protein, enemas with acetate buffer solution and oral medication with lactulose, bifidum milk, and certain amino acids in order to lower hyperammoniemia; in serious cases neomycin has to be given. At the same time a normalization of fluid and electrolyte balance has to be achieved; replacement of potassium is especially important, when hypokalemia and alcalosis are present. In general prognosis of portal-systemic encephalopathy however is serious, depending primarily upon the fact, whether or not sufficient functional hepatic parenchyma is present.
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PMID:[Clinical picture and therapy of portal-systemic encephalopathy (author's transl)]. 89 27

A 53-year-old woman developed symptoms of slow speech, mild dementia, increased deep tendon reflex, ataxic gait, flapping tremor, and dystonic posture during two years. She had liver cirrhosis and hyperammonemia, which suggested a diagnosis of portal-systemic encephalopathy. MR T1-weighted images showed increased signal intensity in globus pallidus, internal capsule, substantia innominata, and a part of hypothalamus. T2-weighted images revealed abnormal findings as follows: high intense area in middle cerebellar peduncle, and low signal intensity of corpus callosum and pontine base. This is the first report about MRI abnormalities in substantia innominata, corpus callosum, and pontine base in a patient with portal-systemic encephalopathy.
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PMID:[A case of portal-systemic encephalopathy presenting characteristic MR images in globus pallidus, hypothalamus, corpus callosum, pontine base, and middle cerebellar peduncle]. 161 82

In 14 patients with cirrhosis and chronic portosystemic encephalopathy, the effectiveness of treatment with a new non-assorbable antibiotic (rifaximine) was compared to neomycin. The parameters evaluated were: bradylalia, flapping tremor, performance, visual evoked potentials and the trial making test. Both treatments were combined with lactulose. The analysis of results showed a rate of positive results in the patients treated with rifaximine greater than that with neomycin. Differences, however, were not significant.
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PMID:Rifaximine versus neomycin in the treatment of portosystemic encephalopathy. 174 38

A 58-year-old male liver cirrhosis patient, who had a history of recurrent hepatic encephalopathy, was admitted to our hospital because of clouding of consciousness. He had an episode of recent head trauma. On admission, he was drowsy and exhibited flapping tremor, fetor hepaticus and ascites. Laboratory data showed an increase in blood ammonia level, and a decrease in peripheral thrombocytes, serum albumin and prothrombin value. A ratio of branched-chain to aromatic amino acids was reduced. Antibody against hepatitis C virus was positive. His electroencephalogram showed bilaterally large synchronous slow waves. He was treated with intravenous branched-chain amino acids, and recovered. Cranial computed tomography on day 9 demonstrated a crescent shaped area of low density in the right frontotempoparietal subdural region. He was diagnosed as chronic subdural hematoma, which was treated surgically. It has been suggested that chronic subdural hematomas develop as a complication of hemostatic deficiency due to liver cirrhosis.
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PMID:[A case of liver cirrhosis associated with chronic subdural hematoma and hepatic encephalopathy]. 174 68

A case of a patient with severe liver dysfunction and hyperammonemia undergoing splenectomy and liver biopsy was reported. Preoperative examination revealed that this patient's liver function was severely impaired due to liver cirrhosis (ICG15 = 60%, HPT = 29%, serum NH3 = 110 micrograms.dl-1). Preoperatively, kanamycin 2 g.day-1 and lacturose 60 ml.day-1 were given and FFP 3-5 units.day-1 were infused. With no premedication, general anesthesia was induced with dTc 3 mg, thiopental 200 mg and SCC 80 mg. Anesthesia was maintained with N2O-O2-enflurane and pancuronium. Though N2O concentration was kept at 50% to prevent intraoperative hypoxemia, the necessary enflurane concentration was low (almost 1% or lower). Serum NH3 level during operation was stable (100-110 micrograms.dl-1), and the level decreased (66-90 micrograms.dl-1) postoperatively. Postoperatively, this patient's consciousness level fluctuated with or without flapping tremor. The treatment of hepatic encephalopathy with lactulose, aminoleban EN and maalox were effective. Problems of perioperative and anesthetic management of a patient for upper abdominal surgery with severe liver dysfunction associated with hyperammonemia were discussed.
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PMID:[Anesthetic experience of a patient for splenectomy with severe liver dysfunction and hyperammonemia]. 223 30

Hepatolenticular degeneration (Wilson's disease) is a hereditary disease in which metabolic disorder of copper leads to its accumulation in the liver, brain, cornea and kidneys with consequent pathologic changes in those organs. Hereditary mechanism of the disease is autosomal recessive with prevalence of 30-100 per 1,000,000 inhabitants. Etiology of this disease is not yet explained. There are two hypotheses. The first one is that it is the disorder of ceruloplasmine metabolism caused by insufficient synthesis of normal ceruloplasmine, or synthesis of functionally abnormal ceruloplasmine. The second one is: the block of copper biliar excretion which is the consequence of the liver lysosomes functional defect. Pathogenetic mechanism of disease is firstly long-term accumulation of copper in the liver, and later, when the liver depo is full, its releasing in circulation and accumulation in the brain, cornea, kidneys and bones, which causes adequate pathologic changes. Toxic activity of copper is the consequence of its activity on enzymes, particularly on those with -SH group. There are two basic clinical forms of the disease: liver disease or neurologic disease. Before puberty the liver damage is more frequent, while in adolescents and young adults neurologic form of the disease is usual. The liver disease is nonspecific and characterized by symptoms of cirrhosis and chronic aggressive hepatitis. The only specificity is hemolytic anemia which, in combination with previous symptoms, is important for diagnosis of the disease. Neurologic symptoms are the most frequent consequence of pathologic changes in the basal ganglia. In our patients the most frequent symptoms were tremor (63%); dysarthria, choreoathetosis and rigor (38%); ataxia and mental disorders (31%); dysphagia and dystonia (12%), diplopia, hypersalivation, nystagmus and Babinski's sign (6%). Among pathologic changes in other tissues and organs the most important is the finding of Kayser-Fleischer ring in the cornea as a result of copper accumulation. Its importance for precise diagnosis is great. The diagnosis of the disease is based on anamnesis, clinical examination, specific and nonspecific laboratory tests. The therapy of choice is penicillamine. If we use it early, the result will be good remission in the majority of patients. Late diagnosis or delay in treatment cause death which is the result of bleeding from esophageal varices or basal ganglia disease. Immunologic damages caused by penicillamine demand interruption of therapy and substitution by three-ethyl-tetra-amine (TETA). We also use zinc salts and tetratiomolibdate in therapy of this disease. Pathogenesis, clinical picture and therapy of the disease are based on our own results.
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PMID:[Hepatolenticular degeneration]. 226 49

We have recently experienced a case of Vibrio vulnificus septicemia which occurred in a patient with hepatic cirrhosis, and as we were able to give early antibiotic treatment, the patient survived. We would like to report this case here together with another case experienced 2 years ago. Case 1 was a 58-year-old male who was attending our hospital as an outpatient for hepatic cirrhosis. At 5:30 pm on August 8, 1987, he consumed abalone and giant clam and at 9 pm complained of high fever with shaking chills. He was admitted to our department as an emergency case. Cefoperazone was administered resulting in a decline of fever on the following day. During the course of treatment he fell transiently into pre-DIC, but due mainly to the administration of antibiotics his condition was subsided. Case 2 was a 53-year-old male who was under medical care in our hospital for grave hepatic cirrhosis. On October 11, 1985, he consumed sushi and two days later suffered chills and pyrexia. A blood culture revealed Vibrio vulnificus. His condition improved transiently with administration of Cefazolin, but oliguria, hypotension and ascites occurred subsequently, and finally the patient died on the 22nd day.
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PMID:[Two case reports of septic shock due to Vibrio vulnificus with liver cirrhosis]. 250 32

Liver histology demonstrated progressive cirrhosis in a 19-year-old girl with a subacute form of Wilson's disease. Despite D-penicillamine administration her liver functions rapidly deteriorated further. Orthotopic liver transplantation was performed. Postoperatively there were two mild rejection episodes, an organic psychiatric syndrome and generalized tremor. Copper metabolism and clinical symptoms became normal postoperatively. Five months after the transplantation she was in a good general condition, able to continue her education.
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PMID:[Orthotopic liver transplantation in Wilson's disease and acute liver failure]. 329 86


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