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)

Chronic progressive hepatocerebral degeneration with spastic paraparesis, dementia, dysarthria, ataxia, tremor, and neuropsychiatric symptoms follows long-standing portal-systemic shunting, is associated with structural changes in the central nervous system, and does not respond to conventional therapy for hepatic encephalopathy. A case of advanced chronic liver disease with severe, progressive hepatocerebral degeneration after 23 yr of portal-systemic shunting is reported in whom there was significant objective improvement in intellectual function and in the chronic neurological signs 3 mo after orthotopic liver transplantation and further improvement 12 mo after transplantation.
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PMID:Improvement in chronic hepatocerebral degeneration following liver transplantation. 231 62

A 44-year-old man suffered from repeated impairment of consciousness associated with flapping tremor, myoclonus and generalized convulsions, and died in coma 6 months after admission. He had had a psychosomatically underdeveloped childhood, with a propensity for legumes without a family history of the same or a record of consanguinity. On admission, he had disturbed consciousness and emaciation without other physical abnormalities. The EEG revealed diffuse slow waves with occasional appearance of triphasic waves. A high level of serum citrulline (534.7 nmol/ml) was recognized and the assay of urea cycle enzymes in the liver demonstrated decreased argininosuccinate synthetase (ASS) activity (0.062 U/g liver, 7.4% of that in normal liver), although no kinetic abnormality was found. Accordingly he was diagnosed as having type II citrullinemia. In addition, this case could be classified as cluster type of localization of the ASS in the liver by immunohistochemical study. There were characteristic findings concerning his clinical picture and laboratory data, such as a significant correlation between the grade of disturbed consciousness and arterial blood gas pH (r = 0.61, p less than 0.01). However, the blood ammonia level did not always correlate with the severity of disturbed consciousness. Oral treatment with sodium citrate and sodium benzoate was very effective, though transiently, for disturbed consciousness in this case. Pathological findings of the autopsied liver were fatty change and fibrosis. Neuropathologically, characteristic findings were brain edema with cerebellar tonsilar herniation, laminar necrosis with spongy formation in cerebral cortex, and Alzheimer type II glia. The relationship between citrullinemia and other hepatic encephalopathy was also discussed.
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PMID:[An autopsied case of type II citrullinemia--transient effectiveness with either citrate or benzoate to the consciousness disturbance]. 269 30

Extensive intrahepatic portal-hepatic venous anastomosis is very rare. This report describes a 47-year-old man with cirrhosis who presented with mental confusion and flapping tremor, and in whom percutaneous transhepatic portography and superior mesenteric angiography demonstrated shunting between the portal vein branches and the right hepatic vein. Measurements of pressure, ammonia, and immunoreactive insulin in blood of the portal and right hepatic veins clearly indicated that a large amount of portal vein blood was being shunted into the right hepatic vein. These findings suggest that hepatic encephalopathy in this patient is accounted for at least in part by an intrahepatic portal-hepatic venous shunting.
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PMID:Hepatic encephalopathy associated with extensive portal-hepatic venous shunts: a case report. 403 31

Chronic, excessive ingestion of alcohol, with its accompanying subnutrition and intermittent drug withdrawal (partial or complete), has produced many neurologic disorders. These problems include involuntary movement disorders which may be reviewed under three major headings: withdrawal tremulous states, cerebellar system dysfunction, and hepatic related disorders.The tremor of alcohol withdrawal resembles that of physiologic tremor when exacerbated by anxiety. It is the most common neurologic manifestation of alcohol withdrawal, and the tremor amplitude is usually greatest some 10 to 20 hours after cessation of drinking. A tremulous state, which may be transient or persistent, also occurs in infants born to alcoholic mothers.The common hepatic encephalopathy may be accompanied by a flapping tremor and multiple other tremors and jerking movements. Chronic porto-systemic encephalopathy is accompanied by choreoathetoid movements and persistent coarse tremors.
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PMID:Extrapyramidal dysfunction in alcoholism. 705 44

In mongrel dogs, the effect of end-to-side portacaval shunt on plasma, cerebrospinal fluid (CSF) and brain tyramine, tyrosine, dopamine, norepinephrine, and epinephrine were studied. It was found that the level of tyramine in plasma, CSF, and selected brain regions increased steadily after the construction of the shunts. These elevations became more pronounced when the dogs manifested symptoms of hepatic encephalopathy. In postshunted dogs with stage II and III hepatic encephalopathy, tyramine concentration in corpus striatum (1,312 +/- 371), hypothalamus (400 +/- 67.0), and midbrain (660 +/- 78.7 ng/g) was significantly (P less than 0.05) higher than the level in dogs with stage 0 and I hepatic encephalopathy and sham-operated dogs serving as controls (corpus striatum, 831 +/- 140; hypothalamus, 167 +/- 40.0; and midbrain, 132 +/- 37.4 ng/g). This was followed by a concomitant depletion of dopamine and norepinephrine in these brain regions (postshunt: dopamine 104 +/- 20.0, 3,697 +/- 977, and 105 +/- 14.1; norepinephrine 521 +/- 71.6, 81.6 +/- 13.7, and 218 +/- 31.7 ng/g; vs. sham group: dopamine 532 +/- 83.1, 8,210 +/- 1,126, and 192 +/- 35.0; norepinephrine 1,338 +/- 425, 124 +/- 21.3, and 449 +/- 89.7 ng/g) of encephalopathic dogs with portacaval shunt. Furthermore, tyramine, tyrosine, dopamine, and norepinephrine levels in plasma and CSF increased markedly as clinical features in the dogs' behavior characteristic of hepatic encephalopathy occurred, including hypersalivation, ataxia, flapping tremor, somnolence, and coma. Cerebral hypertyraminemia and a defect in sympathetic neurotransmission may contribute to the development of hepatic encephalopathy of liver disease.
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PMID:Evidence for central hypertyraminemia in hepatic encephalopathy. 746 24

Lactitol, a non-absorbable synthetic disaccharide, was administered at a dose of 36g/day for 3-4 weeks to 8 patients with liver cirrhosis and hepatic encephalopathy in order to investigate its effects on fecal bacterial flora and clinical symptoms of hepatic encephalopathy. Lactitol significantly increased occupation ratio (ratio to total bacterial number) of anaerobic Bifidobacterium (before administration 7.1% --> after 4 weeks 46.0% (p < 0.05) as well as bacterial count of Lactobacillus. On the other hand, bacterial counts of Bacteroides and Clostridium, which are considered to be NH3-producing bacteria, and that of total aerobic bacteria were not markedly changed, but their occupation ratio were decreased after the administration. Further, tendencies toward decreased fecal pH, increased frequency of defecation and soft stools were observed. As for clinical efficacy, a decrease in blood ammonia concentration, improvement in mental state and flapping tremor were also observed.
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PMID:[Effects of lactitol on fecal bacterial flora in patients with liver cirrhosis and hepatic encephalopathy]. 764 58

Despite the low morbidity and mortality of laparoscopic cholecystectomy, trauma and infection have been reported. Such complications can produce a misleading clinical picture, as in two cases we observed. Case 1. A symptomatic 56-year-old female patient underwent laparoscopic cholecystectomy. During the operation, the gall bladder ruptured and the contents had to be aspirated from the abdominal cavity. The patient complained of hepatalgia 2 weeks after the operation, then was not seen again for more than 1 year when fever and hepatalgia did not respond to symptomatic treatment. An inter-hepato-renal collection (6 cm in diameter) was punctured under echography. Aspirate culture yielded Pseudomonas aeruginosa. Adapted antibiotic therapy was unsuccessful and surgery was required to empty the abscess then remove a fibrous conjunctive tissue formation. Case 2. A 55-year-old female patient with a history of complete remission after mammectomy for breast cancer underwent laparoscopic cholecystectomy in 1991. Two days after the operation, fever (39 degrees C) was accompanied by abdominal defence. Biliary peritonitis due to imperfect suture of the bile duct was repaired followed by peritoneal lavage-drainage. Per-operative blood samples revealed type 6 Pseudomonas aeruginosa. Despite adapted parenteral antibiotics, fever persisted at 39 degrees C and intense jaundice was observed. A second laparoscopy 14 days later showed inflammatory narrowing of the main bile duct which was drained into a small bowel loop. Eight days later computed tomography revealed multiple abscess in the liver. Transparietal cholangiography was performed and showed that the contrast medium entered the abscesses via the biliary canals. The state of sepsis persisted, jaundice worsened and hepatic encephalopathy developed with obnubilation and flapping tremor. After 1 month of general antibiotherapy, no improvement was seen on computed tomography images and needle biopsy of an abscess led to the identification of resistant type 6 P. aeruginosa. Antibiotics were adapted and administered iv with no clinical improvement. Selective catheterism of the hepatic artery via the femoral access was performed to allow intra-hepatic antibiotic delivery. Three weeks later clinical situation remained unchanged when acute respiratory distress highly suggestive of pulmonary embolism led to death. Autopsy was not performed. In both of these rare cases of infectious complications due to P. aeruginosa after laparoscopic cholecystectomy, the source of contamination remained unknown. Nosocomial infection was suspected.
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PMID:[Celioscopic cholecystectomy. 2 cases of infectious complications]. 782 63

A 52-year-old patient presented with paroxystic episodes of generalized apraxia, anomia, agraphia and acalculia. The transient character of these attacks was supported by several neuropsychological examinations. Initially a tentative diagnosis of multiple TIA's was made. Treatment consisted of antiplatelet aggregation therapy. Three years later, however, paroxystic neuropsychological symptomatology occurred more frequently with an increase of severity. The patient was again seen and the differential diagnosis included epilepsy or a metabolic disturbance, in casu hepatic encephalopathy. A therapeutic trial with carbamazepine was started but the patient deteriorated further. He developed a flapping tremor and became stuporous. The blood ammonia was high and there were triphasic waves on the EEG. A probable diagnosis of hepatic encephalopathy was made and carbamazepine therapy was withdrawn. There was a good response on low protein diet and lactulose.
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PMID:Paroxystic neuropsychological symptoms as the early expression of hepatic encephalopathy. A case report. 824 70

We have described several kinds of involuntary movements occurring in metabolic encephalopathies. They are often associated with slowing of electroencephalographic basic activities and with loss of consciousness. Laboratory studies disclose the cause of the encephalopathies. Asterixis, first reported in hepatic encephalopathy, is produced by EMG silence during voluntary contraction. Myoclonus is associated with a short excessive EMG discharges. Tremor is caused by grouping EMG discharges. Some other kinds of involuntary movements are seen in metabolic encephalopathies. Clinical features of hepatic, uremic, hypoxic, and hypoglycemic encephalopathies are briefly described.
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PMID:[Dyskinesia in metabolic encephalopathy]. 827 69

Hyperintense globus pallidus on T1-weighted MRI is present in most patients with advanced liver disease. We evaluated the relationship between the signal intensity of the globus pallidus and clinical or laboratory data of 77 patients eligible for liver transplantation. There was a significant correlation between the intensity of the signal and the Child-Pugh score (as indication of severity of liver disease), presence of postural tremor, previous episodes of variceal bleeding or hepatic encephalopathy, prothrombin activity, serum aspartate and alanine aminotransferase, bilirubin, and the indocyanine green (ICG) hepatic clearance, a very sensitive marker of liver function. The multivariate analysis disclosed that the ICG hepatic clearance and previous episodes of variceal bleeding were independently associated with the signal intensity in the globus pallidus. MRI repeated in 21 patients 10 to 20 months after transplant showed a disappearance of the lesion in all cases. We conclude that the hyperintense globus pallidus is secondary to the severity of the liver disease, and is reversible when liver function returns to normal.
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PMID:Hyperintense globus pallidus on T1-weighted MRI in cirrhotic patients is associated with severity of liver failure. 842 13


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