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 case of reversible encephalopathy after one dose of ifosfamide/mesna in an epileptic 15-year-old girl is reported. No other pathology could be responsible for the symptoms. An epilepsy or a toxicity induced by vincristine were discussed. Nevertheless, the possible role of phenobarbital, known to induce hepatic microsomal activity, seems the more probable mechanism.
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PMID:Ifosfamide/mesna related encephalopathy: a case report with a possible role of phenobarbital in enhancing neurotoxicity. 313 98

An animal model of central distal axonopathy following chronic administration of phenytoin is described. Male C57/BL6J mice received diphenylhydantoin (DPH) in the daily diet (liquid diet 'Stardit', supplemented with vitamins) over a period of 8 weeks. Control and experimental animals were pair-fed. Twelve mice of both groups were perfused via the left ventricle with glutaraldehyde. Representative samples of the cerebral cortex (area 3), cerebellum (vermis and deep cerebellar nuclei), thalamus, hypothalamus, and liver were embedded in araldite. Semi-thin sections and electron microscopy of the cerebellar vermis revealed marked dystrophic changes in the Purkinje cell axons. The presynaptic segments of Purkinje cell axons in the deep cerebellar nuclei showed massive enlargement and swelling due to accumulation of spherical particles and tubular structures in the axoplasm. These structures represent a proliferation of the smooth endoplasmic reticulum. Identical changes were found in hepatocytes of treated animals. Because phenytoin induces hepatic microsomal enzymes, we suggest that phenytoin-related Purkinje cell damage may be produced by an induction of Purkinje cell microsomes with proliferation of the smooth endoplasmic reticulum which causes a swelling and enlargement of presynaptic segments of Purkinje cell axons in deep cerebellar nuclei. Chronic phenytoin administration to mice is a new model of phenytoin-induced encephalopathy and of distal axonopathy of cerebellar neurons.
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PMID:Damage of Purkinje cell axons following chronic phenytoin administration: an animal model of distal axonopathy. 402 72

A case of a 12-year-old girl with a multiple auto-immune disorder is reported. She showed Hashimoto thyroiditis which subsequently developed to hashitoxicosis and distal renal tubular acidosis at 5 years of age, pernicious anaemia at the age of 9 and severe encephalopathy at the age of 12. Laboratory studies revealed very high titres of anti-microsomal and anti-thyroglobulin antibodies and positive gastric parietal cell antibody. As to the encephalopathy, positive oligoclonal IgG bands and high values of IgG index and IgG synthesis ratio in CSF were observed with aggravation of her neurological symptoms. High-dose steroid therapy was effective toward the encephalopathy. Paediatricians should pay careful attention to patients with Hashimoto thyroiditis for association with other autoimmune disorders.
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PMID:Hashimoto thyroiditis, distal renal tubular acidosis, pernicious anaemia and encephalopathy: a rare combination of auto-immune disorders in a 12-year-old girl. 815 29

A case of 43-year-old woman with Hashimoto's encephalopathy who experienced three relapses closely associated with the menstrual cycle is reported. In April 1992, she began to experience occasional tremors in her arms. Three months later, she experienced a generalized seizure and was transferred to our hospital. Hashimoto's thyroiditis was diagnosed on the basis of high thyroid microsomal titer and mild hypothyroidism. Neurological findings in admission included action tremor in both hands, myoclonus in all extremities, cerebellar ataxia, confusion, and hyperreflexia. Cerebrospinal fluid showed elevated protein level without pleocytosis. Electroencephalogram showed diffuse slowing and magnetic resonance imaging of brain was normal. Hashimoto's encephalopathy was diagnosed from these findings. These episodes of remission and exacerbation were observed during the admission. Her symptoms started at ovulation, worsened during the luteal phase, and improved when menstruation started. After the third relapse, she was treated with oral thyroxine for hypothyroidism and with an estrogen and progesterone combination to regulate the menstrual cycle. Her thyroid function gradually became euthyroid and she did not experience any subsequent relapses. The relation between the relapsing course and menstrual cycle suggests that the periodic alteration of gonadotrophic and/or gonadal hormones or the menstrual regulating center itself in the brain may be an important factor of pathogenetic mechanism of the disorder.
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PMID:[A case of Hashimoto's encephalopathy with a relapsing course related to menstrual cycle]. 829 82

Diffuse subcortical MRI signal abnormalities were seen during a subacute exacerbation in a patient with Hashimoto's encephalopathy. The patient had an excellent clinical response to corticosteroids. Clinical recovery paralleled normalization of MRI abnormalities and lowering of thyroid microsomal antibody titer.
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PMID:Reversible MRI findings in a patient with Hashimoto's encephalopathy. 922 97

Patients with Hashimoto's encephalopathy (HE), a steroid-responsive disorder, associated with Hashimoto's disease and high levels of thyroid-related autoantibodies usually present with a subacute onset of confusion, focal or generalized seizures. Frequent EEG abnormalities include generalized, rhythmic bifrontal or temporal slowing. Elevated protein levels or an intrathecal IgG synthesis may be present in cerebrospinal fluid (CSF). A 39-year-old woman underwent a relapsing course of myocloni and generalized seizures. Initially, thyroid function, thyroid-related autoantibody screening and cerebral MRI were unrevealing. CSF showed oligoclonal bands. Short-term treatment with high doses of prednisolone resolved the myocloni. During the 5th episode of myocloni, signs of hyperthyroidism and elevation of thyroid microsomal antibody titer developed. Hashimoto's thyroiditis and HE were diagnosed. After subtotal thyroidectomy the patient remained asymptomatic.
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PMID:Manifestation of Hashimoto's encephalopathy years before onset of thyroid disease. 1002 9

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

Thirty-five years since its introduction into clinical use, valproate (valproic acid) has become the most widely prescribed antiepileptic drug (AED) worldwide. Its pharmacological effects involve a variety of mechanisms, including increased gamma-aminobutyric acid (GABA)-ergic transmission, reduced release and/or effects of excitatory amino acids, blockade of voltage-gated sodium channels and modulation of dopaminergic and serotoninergic transmission. Valproate is available in different dosage forms for parenteral and oral use. All available oral formulations are almost completely bioavailable, but they differ in dissolution characteristics and absorption rates. In particular, sustained-release formulations are available that minimise fluctuations in serum drug concentrations during a dosing interval and can therefore be given once or twice daily. Valproic acid is about 90% bound to plasma proteins, and the degree of binding decreases with increasing drug concentration within the clinically occurring range. Valproic acid is extensively metabolised by microsomal glucuronide conjugation, mitochondrial beta-oxidation and cytochrome P450-dependent omega-, (omega-1)- and (omega-2)-oxidation. The elimination half-life is in the order of 9 to 18 hours, but shorter values (5 to 12 hours) are observed in patients comedicated with enzyme-inducing agents such as phenytoin, carbamazepine and barbiturates. Valproate itself is devoid of enzyme-inducing properties, but it has the potential of inhibiting drug metabolism and can increase by this mechanism the plasma concentrations of certain coadministered drugs, including phenobarbital (phenobarbitone), lamotrigine and zidovudine. Valproate is a broad spectrum AED, being effective against all seizure types. In patients with newly diagnosed partial seizures (with or without secondary generalisation) and/or primarily generalised tonic-clonic seizures, the efficacy of valproate is comparable to that of phenytoin, carbamazepine and phenobarbital, although in most comparative trials the tolerability of phenobarbital was inferior to that of the other drugs. Valproate is generally regarded as a first-choice agent for most forms of idiopathic and symptomatic generalised epilepsies. Many of these syndromes are associated with multiple seizure types, including tonic-clonic, myoclonic and absence seizures, and prescription of a broad-spectrum drug such as valproate has clear advantages in this situation. A number of reports have also suggested that intravenous valproate could be of value in the treatment of convulsive and nonconvulsive status epilepticus, but further studies are required to establish in more detail the role of the drug in this indication. The most commonly reported adverse effects of valproate include gastrointestinal disturbances, tremor and bodyweight gain. Other notable adverse effects include encephalopathy symptoms (at times associated with hyperammonaemia), platelet disorders, pancreatitis, liver toxicity (with an overall incidence of 1 in 20,000, but a frequency as high as 1 in 600 or 1 in 800 in high-risk groups such as infants below 2 years of age receiving anticonvulsant polytherapy) and teratogenicity, including a 1 to 3% risk of neural tube defects. Some studies have also suggested that menstrual disorders and certain clinical, ultrasound or endocrine manifestations of reproductive system disorders, including polycystic ovary syndrome, may be more common in women treated with valproate than in those treated with other AEDs. However, the precise relevance of the latter findings remains to be evaluated in large, prospective, randomised studies.
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PMID:Pharmacological and therapeutic properties of valproate: a summary after 35 years of clinical experience. 1226 62

We report a case of Hashimoto's encephalopathy with prominent unsteadiness of gait. Nerve conduction studies and electromyographic findings were consistent with a sensory ganglionopathy. Symptoms and clinical findings resolved with high-dose corticosteroid therapy and paralleled the levels of anti-microsomal antibodies. Sensory ganglionopathy seems to be another aspect of the broad clinical syndrome labeled Hashimoto's encephalopathy.
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PMID:A case of Hashimoto's encephalopathy: association with sensory ganglionopathy. 1613 8

Examination of 91 patients with diabetic retinopathy in the presence of type I diabetes mellitus (DM-1) showed that 56% of the patients had a great variety of organ-specific and organ-nonspecific autoantibodies (AABs), including those to the microsomal and cytoplasmic fractions of endocrine glands, such as pancreas, thyroid, and pituitary. AABs were most common to the pituitary (23.2%), total myelin protein (18.7%), and denatured DNA (17.6%). There were correlations between AAB and the duration of DM-1 more than 20 years (78.3%; p < 0.05), between the total myelin protein AAB and encephalopathy (31.3%; p < 0.05), between the denatured DNA AAB and the increased retinal vascular permeability (29.3%; p < 0.05), which suggests their implication in vascular wall disintegration. No correlations could be found between AAB to the pituitary, pancreas, and thyroid and obvious pathology of the glands. Combined therapy with the immunomodulators thymactid and lycopide yielded a total beneficial effect (AAB disappearance, decreased titers, and no changes) in 88.3% of case while beneficial effect of insulin therapy was obtained in 53.9% of cases, which suggests that it is expedient to include of currently available immunomodulators into traditional insulin therapy.
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PMID:[Systemic autoantibodies and their correction in patients with diabetic retinopathy in type 1 diabetes mellitus]. 1721 99


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