Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A psychiatric disorder well known for more than fifty years in children and adolescents has changed several times its name. Mild child encephalopathy, minimal (mild) cerebral dysfunction, impaired attention, hyperkinetic disorder. The cause is not known, there is, however, evidence of familial disposition. Symptoms of this disorder were fully manifested in patients with generalized resistance to thyroid hormone. The disease is due to a genetic mutation of the beta-thyroid receptor and characterized by a reduced response of the pituitary and peripheral tissue to thyroid hormone. The authors refer to the work of authors, who discovered the association between the hyperkinetic disorder and generalized resistance to thyroid hormone.
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PMID:[Hyperkinetic disorder--a new view? (Generalized resistance to thyroid hormone)]. 798 30

Propylthiouracil (PTU), a thyroid hormone inhibitor, is widely used for the treatment of hyperthyroidism. Rarely, the drug has been associated with severe hepatotoxicity. We present the case of a 13-year-old girl who developed jaundice and profound liver dysfunction with rapid progression to metabolic encephalopathy while receiving PTU therapy. She died despite extensive therapeutic measures including orthotopic liver transplantation. Her rapid clinical course and fatal outcome show that in spite of regular monitoring, severe, rare, rapidly occurring complications of PTU therapy may still occur.
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PMID:Propylthiouracil-induced fulminant hepatitis: case report and review of the literature. 902 82

We report the clinical, laboratory, EEG, and SPECT findings in a 59-year-old euthyroid woman with previously undiagnosed autoimmune thyroiditis, subclinical hypothyroidism, and rapidly progressive dementia. We made a diagnosis of Hashimoto's encephalopathy based on elevated thyrotropin, abnormal EEG, and clinical improvement after thyroid hormone replacement. SPECT demonstrated global hypoperfusion with normalization on clinical recovery, suggesting a possible mechanism for the pathogenesis of Hashimoto's encephalopathy.
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PMID:Autoimmune thyroiditis and a rapidly progressive dementia: global hypoperfusion on SPECT scanning suggests a possible mechanism. 927 Jun 13

We recently showed that some decrease in thyroid hormone blood levels can effectively and significantly prevent the development of cirrhosis and fulminant hepatic failure and decrease portal pressure in three different rat models. This study was conducted to determine whether hypothyroidism has a beneficial effect over euthyroidism on patients with active liver cirrhosis of different etiologies. The medical files of hypothyroid patients with cirrhosis who were referred to the Tel-Aviv Medical Center between the years 1980 and 1995 were retrospectively evaluated. Of 3,528 patients with biopsy-proven cirrhosis and 4,738 hypothyroid patients who were identified, only 46 (25 female, 54%; mean age, 52.3 +/- 9.1) met the eligibility criteria. The patients suffered from cirrhosis (mean, 9.5 +/- 4.3 years; range, 4-23) and had hypothyroidism (mean, 12 +/- 6 years; range, 4-31). Most patients suffered from hypothyroidism of unknown etiology (85%), whereas the rest had hypothyroidism after surgical/iodine ablation of the gland. In the hypothyroid versus the euthyroid state, a significant negative correlation was found between thyroid-stimulating hormone blood levels and both functional and synthetic liver function tests (p < 0.001). A significant negative correlation was also found between thyroid-stimulating hormone blood levels and clinical deterioration manifested as bleeding varices, the development of ascites, and episodes of encephalopathy. We conclude that in patients with liver cirrhosis, the liver function in the hypothyroid state tend to be better than in the euthyroid state. A mild controlled decreased thyroid function may be beneficial for euthyroid cirrhotic patients.
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PMID:Liver function in cirrhotic patients in the euthyroid versus the hypothyroid state. 985 65

A 25-year-old woman presented with a subacute confusional state, headaches, unsteadiness, myoclonus, seizures, and an amnesic syndrome as a manifestation of Hashimoto's encephalopathy. Investigations showed biochemical hypothyroidism, raised thyroid microsomal antibodies, and weakly positive antineuronal antibodies. A T2-weighted MRI of the brain showed bilateral symmetric areas of increased signal in the mesial temporal lobes and hippocampi that had a low signal intensity on T1-weighted imaging. Despite clinical and radiologic improvement after steroid and thyroid hormone replacement therapy, a severe amnesic syndrome with associated localized MRI abnormalities persists.
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PMID:Amnesic syndrome with bilateral mesial temporal lobe involvement in Hashimoto's encephalopathy. 1068 Aug 14

The corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (the so-called "Hashimoto's Encephalopathy") is a rare disorder with multiple symptomatology, breaking out with an acute or subacute onset and having a relapsing course, not correlated to thyroid hormone levels, with autoimmune pathogenesis, and usually associated with Hashimoto's thyroiditis. In this paper, we report on a case study regarding a 46 year-old woman showing a subacute course cerebellar syndrome, associated with Hashimoto's thyroiditis, diagnosed as "Hashimoto's encephalopathy". The possible pathogenesis and the major aspects of the differential diagnostic sector are discussed with particular reference to an ataxic syndrome caused by a progressive non-familial adult onset cerebellar degeneration (PNACD), associated with the thyroid disease itself.
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PMID:Cerebellar subacute syndrome due to corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called "Hashimoto's encephalopathy"). 1608 Jun 55

Changes of the affective and cognitive function are usually associated with thyroid gland dysfunction. In autoimmune thyroid disease, these changes can be caused by thyroid dysfunction (hypo- or hyperthyroidism) or associated with the presence of antithyroid antibodies. Even a small change in thyroid hormone concentration is associated with change of cognitive function. In euthyroid older males, variation of total and free thyroxin accounts for about 10% of Wechsler adult intelligence test variance. In euthyroid females, lower cognitive function, measured by Mini Mental test, also correlates with blood thyroxin. Short-term (4 weeks) hypothyroidism induces clinically significant cognitivedysfunction, which is reversible by thyroid hormone substitution. Mild hypothyroidism (TSH less than 10) also induces reversible cognitive dysfunction. In hypothyroidism, PET scanning shows global reduction of brain blood flow and glucose metabolism. Hashimoto's encephalopathy is characterized by corticosteroid reversible encephalopathy associated with the presence of antithyroid antibodies. Encephalopathy can be manifested as multiple stroke-like episodes (vasculitis like), or as diffuse, progressive type characterized by dementia and psychiatric symptoms. In euthyroid patients with Hashimoto's thyroiditis and no evidence of neurological disease, SPECT showed brain perfusion abnormalities. Post mortem and brain biopsy findings can be normal or show perivascular lymphocytic infiltration. Recently, presence of antineuronal antibodies has been found in patients with Hashimoto's thyroiditis. Specific high reactivity against human alpha-enolase was high in patients with Hashimoto's encephalopathy, but absent in patients with other neurological disorders and healthy subjects. Specific antineural antibodies were found in another group of Hashimoto's encephalopathy patients. Furthermore, Ferracci et al, found antithyroid antibodies in the CSF of patients with Hashimoto's encephalopathy.
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PMID:[Autoimmune thyroid disease and brain]. 1640 64

We describe a patient with Hashimoto's encephalopathy presenting as long-standing episodes of aphasia associated with migraine-like headache. Repeated thyroid hormone levels were within normal values, but high titers of antithyroid antibodies in serum, and diffuse EEG slowing and CSF abnormalities during one episode led to the diagnosis.
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PMID:Hashimoto's encephalopathy mimicking migraine with aura. 1735 9

An 82-year-old Japanese man had consciousness disturbance due to severe hypothyroidism triggered by percutaneous absorption of iodine from an iodine-containing ointment used in diabetic gangrene treatment. Laboratory data revealed extremely high urinary iodine concentrations, and chronic thyroiditis-induced hypothyroidism. Excess iodine intake can also cause hypothyroidism. It was unlikely that iodine intoxication or Hashimoto's encephalopathy had caused the consciousness disturbance. The patient regained consciousness after discontinuing the use of the ointment and commencing thyroid hormone therapy. We conclude that consciousness disturbance resulted from severe hypothyroidism caused by chronic thyroiditis and excess iodine absorption.
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PMID:A case of consciousness disturbance resulting from severe hypothyroidism due to chronic thyroiditis and excess iodine absorption. 2204 70

To develop and function optimally, the brain requires a balanced environment of electrolytes, amino acids, neurotransmitters, and metabolic substrates. As a consequence, organ dysfunction has the potential to induce brain disorders and toxic-metabolic encephalopathies, particularly when occurring during early stages of cerebral maturation. Induced toxicity of three different organ systems that are commonly associated with brain complications are discussed. First, thyroid hormone deficiency caused by intrinsic or extrinsic factors (e.g., environmental toxins) may induce severe adverse effects on child neurological development from reversible impairments to permanent mental retardation. Second, inadequate removal of wastes due to chronic renal failure leads to the accumulation of endogenous toxins that are harmful to brain function. In uremic pediatric patients, the brain becomes more vulnerable to exogenous substances such as aluminum, which can induce aluminum encephalopathy. Following surgical procedures, neurological troubles including focal defects and severe epileptic seizures may result from hypertensive encephalopathy combined with toxicity of immunomodulating substances, or from the delayed consequences of cardiovascular defect. Taken together, this illustrates that organ disorders clearly have an impact on child brain function in various ways.
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PMID:Postnatal toxic and acquired disorders. 2362 16


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