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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nitrogen balance at three levels of protein intake was measured in eight patients with cirrhosis of the liver; moreover, at each level of protein intake, the effects on nitrogen balance of branched-chain amino-acid enriched protein and natural protein were compared. From these nitrogen balance data, minimum protein requirements were calculated by linear regression analysis. The patients were in a negative nitrogen balance on a 40 g protein diet (-0.75 +/- 0.15 gN.), and in positive nitrogen balance on 60 g (+1.23 +/- 0.22 gN.) or 80 g of protein per day (+2.77 +/- 0.20 g N.). Their mean minimum protein requirement (48 +/- 5 g of protein/day or 0.75 g/kg/day) is higher than expected in healthy people; the safe level of protein intake (mean + 2 sd) is 58 g per day or 1.2 g/kg/day. Nitrogen balances and protein requirements were not different on branched-chain amino-acid enriched diets. The physical condition of the patients improved when they came into positive nitrogen balance; the higher rates of protein intake were well tolerated without onset of encephalopathy. We conclude that protein requirements are elevated in cirrhosis of the liver; diets supplying less than 60 g of protein per day should not be prescribed in long term treatment of cirrhotic patients.
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PMID:Minimum protein requirements in liver cirrhosis determined by nitrogen balance measurements at three levels of protein intake. 1683 9

A 6-year-old Quarter Horse stallion was referred to Oklahoma State University Veterinary Medical Teaching Hospital for evaluation of abdominal pain that developed after breeding activity earlier in the day. The horse developed diarrhea and progressively worsening neurologic signs (circling, ataxia, head pressing) within 22 hours of presentation and was subsequently euthanized due to severe self-destructive behavior. Antemortem biochemical and hematologic abnormalities included hypocalcemia but no evidence of hepatic disease. Idiopathic hyperammonemia and encephalopathy were suspected; cerebrospinal fluid (CSF) and aqueous humor were collected 10 hours postmortem for ammonia analysis using a colorimetric assay. Results were compared with those of 6 horses that also had been euthanized, for diseases unrelated to encephalopathy. Ammonia also was measured in plasma samples obtained antemortem. Ammonia concentrations in plasma (958 micromol/L), CSF (1566 micromol/L) and aqueous humor (1018 micromol/L) samples from the stallion were markedly increased compared to those in the 6 unaffected horses (plasma, 9-43 micromol/L; CSF, 370-532 micromol/L; aqueous humor, 70-483 micromol/L). Since the acute nature of hyperammonemic encephalopathy often does not provide sufficient time for an antemortem diagnosis, postmortem analysis of CSF and aqueous humor ammonia concentrations may be a useful alternative for documenting hyperammonemia in horses.
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PMID:Postmortem diagnosis of idiopathic hyperammonemia in a horse. 1752 96

Hyperammonemia (HA) commonly occurs with the use of valproic acid (VPA); while it has no clinical significance in most cases, the Physician Desk Reference recommends its discontinuation in the presence of HA. The purpose of this study is to review the literature in order to estimate the prevalence and magnitude of HA in VPA treated patients, to establish any association with hepatotoxicity and encephalopathy and to identify any factors associated with its occurrence. A search of MEDLINE and Cochrane Database of Systematic Reviews, between 1980 and 2005 was performed. Out of 183 studies, 24 met our inclusion criteria. The prevalence of HA in the prospective studies ranged between 70% and 100%, while in cross-sectional studies it varied between 16% and 100%. Ammonia (NH(3)) blood levels increased by a two-fold average relative to the baseline levels. There was no association between HA and clinical symptoms. Concomitant administration of other antiepileptic drugs (AEDs) was the factor most frequently associated with HA.
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PMID:The measurement of ammonia blood levels in patients taking valproic acid: looking for problems where they do not exist? 1816 42

We report an 85-year-old man with Hashimoto's encephalopathy (HE) who showed spontaneous complete remission. The autoantibody against the amino (NH2) terminal region of alpha-enolase was positive in our patient. Neuropsychological manifestations, such as personality change and progressive cognitive impairment, gradually improved over approximately 6 weeks after onset of disease without corticosteroid treatment in parallel with a decrease in the anti-thyroglobulin antibody in the cerebrospinal fluid. HE should be considered as a possible diagnosis even in elderly patients with neuropsychiatric symptoms, particularly when a previous history of thyroid disease is present.
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PMID:An 85-year-old case with Hashimoto's encephalopathy, showing spontaneous complete remission. 1787 54

A 79-year-old female was admitted to our hospital because of unconsciousness and convulsion following mental deterioration. On admission, she exhibited myoclonic movement of the right side of the face and right fingers in addition to rigospasticity and tremors in the right arm and leg. Laboratory tests revealed hyperthyroidism with an increased anti-TSH-R antibody titer. In addition, an echogram indicated excessive blood flow at the thyroid; hence, the patient was diagnosed with Basedow's disease. Interestingly, the tests also revealed increased titer of anti-TPO antibody, anti-Tg antibody, and anti-NH2 terminal of alpha-enolase (NAE) antibody; in addition, an EEG showed abnormal findings potentially indicating periodic synchronous discharge. Brain MRI showed cerebral atrophy, and brain 99mTc-ECD-SPECT images demonstrated an overall decrease in the accumulation of 99mTc in the cerebrum. The abovementioned findings are common to patients with Creutzfeldt-Jakob disease (CJD). We initiated treatment for hyperthyroidism with thiamazole and lugol, but this did not regain consciousness. Because she had anti-thyroid antibody was observed, we considered a differential diagnosis of Hashimoto's encephalopathy and, in fact, methylprednisolone pulse therapy alleviated her symptoms and normalized the EEG findings. The condition in this case clinically mimicked CJD; therefore, the differentiated diagnosis is important because Hashimoto's encephalopathy is treatable disease.
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PMID:[Case report of a patient with Hashimoto's encephalopathy associated with Basedow's disease mimicking Creutzfeldt-Jakob disease]. 1851 79

Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that may occur in people with normal liver-associated enzyme levels, despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl who had absence seizure and complained about progressive dizziness and general malaise several days after restarting valproate. Then, she presented vomiting and decreased consciousness three weeks after valproate use. Notably, her serum ammonia level was five times the upper limit of normal (184 micrommol/L), with normal liver-associated enzyme and supra-therapeutic valproate level. EEG showed continuous generalized slowing. The tandem mass analysis revealed carnitine deficiency. Consciousness improved after emergent hemodialysis. Ammonia level and EEG also returned to normal. Possible mechanisms, risk factors and the treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.
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PMID:Valproate-induced hyperammonemic encephalopathy treated by hemodialysis. 1879 59

Elevated concentrations of ammonia in the brain as a result of hyperammonemia leads to cerebral dysfunction involving a spectrum of neuropsychiatric and neurological symptoms (impaired memory, shortened attention span, sleep-wake inversions, brain edema, intracranial hypertension, seizures, ataxia and coma). Many studies have demonstrated ammonia as a major player involved in the neuropathophysiology associated with liver failure and inherited urea cycle enzyme disorders. Ammonia in solution is composed of a gas (NH(3)) and an ionic (NH(4) (+)) component which are both capable of crossing plasma membranes through diffusion, channels and transport mechanisms and as a result have a direct effect on pH. Furthermore, NH(4) (+) has similar properties as K(+) and, therefore, competes with K(+) on K(+) transporters and channels resulting in a direct effect on membrane potential. Ammonia is also a product as well as a substrate for many different biochemical reactions and consequently, an increase in brain ammonia accompanies disturbances in cerebral metabolism. These direct effects of elevated ammonia concentrations on the brain will lead to a cascade of secondary effects and encephalopathy.
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PMID:Identifying the direct effects of ammonia on the brain. 1910 24

Low-grade or minimal hepatic encephalopathy (MHE) is characterised by relatively mild neurocognitive impairments, and occurs in a substantial percentage of patients with liver disease. The presence of MHE is associated with a significant compromise of quality of life, is predictive of the onset of overt hepatic encephalopathy and is associated with a poorer prognosis for outcome. Early identification and treatment of MHE can improve quality of life and may prevent the onset of overt encephalopathy, but to date, there has been little agreement regarding the optimum method for detecting MHE. The International Society on Hepatic Encephalopathy and Nitrogen Metabolism convened a group of experts for the purpose of reviewing available data and making recommendations for a standardised approach for neuropsychological assessment of patients with liver disease who are at risk of MHE. Specific recommendations are presented, along with a proposed methodology for further refining these assessment procedures through prospective research.
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PMID:Neuropsychological assessment of hepatic encephalopathy: ISHEN practice guidelines. 1930 44

Hashimoto's thyroiditis (HT) is the most common disorder affecting the thyroid gland. Encephalopathy associated with abnormal thyroid functions, such as myxedema encephalopathy, is treatable. Hashimoto's encephalopathy (HE) was recognized as a new clinical disease based on an autoimmune mechanism associated with HT, and steroid treatment has been successfully administrated. Recently, we discovered serum autoantibodies against the NH2-terminal of a-enolase (NAE) as a specific diagnostic marker for HE. We analyzed these serum anti-NAE autoantibodies and the clinical features in 84 cases of HE. The 84 patients consisted of 26 men and 58 women, from many institutions throughout Japan and other countries. A total of 37 patients carried anti-NAE antibodies (44%). The age was widely distributed between 19 and 87 years old, with two peaks (around 20-30 and 50-70 years old). Most patients were in euthyroid states, and all patients had anti-thyroid (TG) and/or anti-thyroid peroxidase (TPO) antibodies, and anti-TSH receptor (TSHR) antibodies in some cases. Only 20% of patients had past histories of HT. The acute encephalopathy form was the most common clinical feature, and subacute or chronic psychiatric forms and other forms such as pure ataxia, limbic encephalopathy, and Creutzfeldt Jakob-like forms followed. The patients with anti NAE antibodies tended to exhibit acute encephalopathy. The most common neuropsychiatric features were consciousness disturbance, psychiatric symptoms, and seizures. Involuntary movements (tremor, myoclonus, or choreoathetosis) or ataxia occasionally occurred. Abnormalities, especially the slowing of background activities, on EEG and elevated levels of protein/IgG in cerebrospinal fluid (CSF) were common and useful laboratory findings for the diagnosis, while abnormalities on brain MRI were rare and non-specific in HE. Immunotherapies such as glucocorticoid, immunosuppressants, immunoglobulin, and plasma exchange, were recommended and effective for HE treatment. HE belongs to a part of a clinical spectrum consisting of individuals with anti-thyroid antibodies, overlapping the clinical spectrum of HT. Anti-NAE autoantibodies were positive in 44% of patients with HE. Considering the overall findings, we should be aware of the possibility of autoimmune encephalopathy associated with thyroid disorders (HE) in patients with an unknown etiology of neuronpsychiatric symptoms with/without a past history of HT.
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PMID:[Anti-NAE autoantibodies and clinical spectrum in Hashimoto's encephalopathy]. 1936 98

We present a 71-year-old woman with hereditary hemorrhagic telangiectasia (HHT) who at age 69, had undergone total gastrectomy because of repeated upper gastrointestinal bleeding. A day prior to admission she began to demonstrate abnormal behavior. Examination showed she was restless and had higher brain dysfunction. Triphasic waves were seen on EEG, and a high signal in the globus pallidus on T1-weighted MRI. Plasma NH3 level was increased after a meal. Abdominal CT scan showed vascular anomalies including a portohepatic vein shunt. She was diagnosed with portosystemic encephalopathy. After treatment with a low-protein diet, lactitol, and branched chain-amino acids, her clinical condition, plasma NH3 level after a meal, and EEG returned to normal. Because portosystemic shunt is rare in HHT, there have been few reports of portosystemic encephalopathy with this condition. However, with aging, the possibility of portosystemic encephalopathy increases because of age-related increases in portosystemic shunt volume.
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PMID:[Elderly hereditary hemorrhagic telangiectasia female with portosystemic encephalopathy]. 1959 5


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