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

A 15-year-old girl is described with encephalopathy associated with Hashimoto thyroiditis which subsequently developed into autoimmune thyrotoxicosis (hashitoxicosis) and distal renal tubular acidosis at age 5 years, pernicious anemia at age 9, and encephalopathy at age 12. Thyroid hormone levels were within the normal ranges at the time of neurologic presentation. Serum IgG concentration and oligoclonal IgG bands in cerebrospinal fluid closely correlated with the severity of neurologic symptoms. Treatment with glucocorticoids improved the level of consciousness but her mental state continued to deteriorate. Repeated cranial computed tomographic scans revealed progressive symmetric atrophy in the gray matter without infarction. These findings suggest that encephalopathy associated with Hashimoto thyroiditis is caused by an antineuronal antibody-mediated reaction.
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PMID:Hashimoto encephalopathy: etiologic considerations. 770 95

The aetiology of Hashimoto's encephalopathy is still unknown. A 52-year-old woman with so far unspecific thyroid disorder presented with acute onset of right-sided sensory loss and visual disturbances. The neurological examination revealed a right upper quadrant anopsia and subtle right-sided sensory loss and weakness. The cranial MRI showed a left-sided cerebral infarction. MR angiography demonstrated a stenosis of the proximal segment of the left posterior cerebral artery, which was confirmed by conventional catheter angiography. The patient had no cardiovascular risk factors, no signs of systemic vasculitis, and no thromboembolic disorder. Thyroid function tests showed a subclinical hypothyroidism with plasma TSH level of 12.0 mU/ml, and thyroid antibodies were markedly elevated (hTG-AB 3390 U/ml, TPO-AB > 8000 U/ml). Typical features of Hashimoto's disease were demonstrated by ultrasound and scintigraphic examination of the thyroid gland. To the best of our knowledge, this is the first description of Hashimoto encephalopathy with localised vasculitis of the posterior cerebral arteries and left posterior infarction. It could be shown that the MR angiogram is a feasible tool to demonstrate regression of the vasculitis under glucocorticoid therapy.
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PMID:[Circumscribed vasculitis with posterior infarct in Hashimoto encephalopathy]. 1259 32

Corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called Hashimoto's encephalopathy) is a rare, life-threatening, treatable, and possibly autoimmune condition. We identified nine patients (with the diagnosis made after 1979) who had relapsing encephalopathy compatible with previous reports of Hashimoto's encephalopathy and no other identifiable cause of encephalopathy at Mayo Clinic Rochester. Of these nine patients, three were clinically hypothyroid, four were subclinically hypothyroid, and two were euthyroid. Thyroid antibodies were positive in eight of eight patients in whom these measurements were made. Electroencephalographic abnormalities were identified in eight of the nine patients (89%). Magnetic resonance imaging (MRI) abnormalities considered etiologically related to encephalopathy were present in three patients (33%). An increased protein concentration was noted on cerebrospinal fluid examination in seven patients (78%). Of the six patients who received high-dose glucocorticoid therapy, 5 (83%) had improvement of neurologic symptoms. In conclusion, encephalopathy associated with autoimmune thyroiditis is rare but important to recognize because it may be responsive to high-dose glucocorticoid therapy. We believe that this condition is not caused by thyroid dysfunction or antithyroid antibodies but represents an association of an uncommon autoimmune encephalopathy with a common autoimmune thyroid disease. The term Hashimoto's encephalopathy is a misnomer and should not be used.
Thyroid 2002 May
PMID:Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996. 1269 1

Thyroid related autoantibodies have been related to the development of encephalopathy, known as Hashimoto's encephalopathy. However, their relation with the encephalopathy occurring in patients with Graves' disease has not been well established. The case is reported of a 51 year old woman presenting with subacute progressive dementia with evidence of hyperthyroidism. She had Graves' disease associated with high titres of thyroid related autoantibodies. Her encephalopathy was not improved by antithyroid drugs, but promptly responded to corticosteroid treatment, and stabilised with a gradual reduction of thyroid related autoantibody titres. Brain positron emission tomography initially showed a diffuse and multifocal cerebral hypometabolism with subsequent normalisation on her clinical recovery, which was consistent with the acute and reversible cerebral inflammation probably mediated by autoimmune mechanisms.
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PMID:Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study. 1264 74

A 55-year-old woman presented first episode of paraplegia at D6 level with a marked improvement of neurological signs, but relapse occurred five months later. Hashimoto's thyroiditis with euthyroidism was found. Long-term 9 months treatment with oral corticosteroids was applied. Also she was able to walk with help. Two years after the second palsy she developed a new paraplegia at D6 level with a severe residual deficit five months later in spite of oral corticosteroids. Thyroid antibodies remained positive. Devic's disease without optica neuritis associated with thyroiditis or spinal cord localisation of Hashimoto's encephalopathy was discussed.
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PMID:[Hashimoto's thyroiditis associated with recurrent transverse myelopathy]. 1291 77

During experimental hypertensive cardiac hypertrophy, the heart energy metabolism reverts from the normal adult type that obtains the majority of its requirement for adenosine triphosphate (ATP) from metabolism of fatty acids and oxidative phosphorylation (OXPHOS), to the fetal form, which metabolizes glucose and lactate. Mitochondrial synthesis and function require an estimated 1000 polypeptides, 37 of which are encoded by mitochondrial (mt) DNA, the rest by nuclear (n) DNA. Inherited or acquired aberrations of either mtDNA or nDNA mitochondrial genes cause mitochondrial dysfunction. Tissue expression of OXPHOS enzyme defects is often heterogeneous. As a result, cardiomyopathy and cardiac failure are frequent but unpredictable complications of mitochondrial encephalopathy, neuropathy, and myopathy. Several nuclear genes that encode mitochondrial proteins have been sequenced and specific defects associated with nuclear genes that affect mitochondrial structure and function have been linked to hypertrophic and dilated cardiomyopathies and to cardiac conduction defects. Thyroid hormone and exercise stimulate expression of a nuclear respiratory factor (NRF) that induces the nuclear gene TFAM, which encodes the mitochondrial transcription factor A that controls mitochondrial replication and transcription. TFAM-null mouse embryos lack mitochondria and fail to develop a heart. Mitochondrial dysfunction enhances the generation of radical oxygen species (ROS), which damage mtDNA, nDNA, proteins, and lipid membranes. Mice lacking the mitochondrial antioxidant enzyme manganese-superoxide dismutase (SOD) develop dilated cardiomyopathy. Palliative mitochondrial therapy with L-acetyl-carnitine and coenzyme Q10 improves cardiac function in patients with cardiomyopathy. Cure is only achievable by mitochondrial gene therapy. Experimental direct gene therapy uses vectors or targeting signal sequences to insert genes into mtDNA; indirect gene therapy employs viral or non-viral vectors to introduce genes into nDNA. Clinical repair of damaged somatic and germline genes that encode mitochondrial proteins may soon be within reach.
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PMID:Review: Mitochondrial medicine--cardiomyopathy caused by defective oxidative phosphorylation. 1458 51

Thyroid hormones are of primary importance for the perinatal development of the central nervous system, and for normal function of the adult brain. These hormones primarily regulate the transcription of specific target genes. They increase the cortical serotonergic neurotransmission, and play an important role in regulating central noradrenergic and GABA function. Thyroid deficiency during the perinatal period results in mental retardation. Hypothyroidism of the adults causes most frequently dementia and depression. Other less common clinical pictures include myxoedema coma, dysfunction of cerebellum and cranial nerves. Hypothyroidism also increases predisposition of stroke. Peripheral diseases frequently include polyneuropathy, carpal tunnel syndrome, myalgic state, and rarely myokymia. Nearly all the hyperthyroid patients show minor psychiatric signs, and infrequently psychosis, dementia, confusion state, depression, apathetic thyrotoxicosis, thyrotoxic crisis, seizures, pyramidal signs, or chorea occur. The peripheral complications may be indicated by chronic thyrotoxic myopathy, infiltrative ophthalmopathy, myasthenia gravis, periodic hypokalemic paralysis and polyneuropathy. Generalized resistance to thyroid hormone was confirmed in a number of patients with attention deficit-hyperactivity disorder. Significantly elevated antithyroid antibody titers characterize Hashimoto's encephalopathy. This condition is a rare, acute - subacute, serious, life threatening, but steroid-responsive, relapsing-remitting, autoimmune disease.
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PMID:[Some neurologic and psychiatric complications in endocrine disorders: the thyroid gland]. 1734 50

Hashimoto's encephalopathy is a term used to describe an encephalopathy of presumed autoimmune origin characterised by high titres of antithyroid peroxidase antibodies. In a similar fashion to autoimmune thyroid disease, Hashimoto's encephalopathy is more common in women than in men. It has been reported in paediatric, adult and elderly populations throughout the world. The clinical presentation may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms and myoclonus. Thyroid function is usually clinically and biochemically normal.Hashimoto's encephalopathy appears to be a rare disorder, but, as it is responsive to treatment with corticosteroids, it must be considered in cases of 'investigation negative encephalopathies'. Diagnosis is made in the first instance by excluding other toxic, metabolic and infectious causes of encephalopathy with neuroimaging and CSF examination. Neuroimaging findings are often not helpful in clarifying the diagnosis. Common differential diagnoses when these conditions are excluded are Creutzfeldt-Jakob disease, rapidly progressive dementias, and paraneoplastic and nonparaneoplastic limbic encephalitis. In the context of the typical clinical picture, high titres of antithyroid antibodies, in particular antithyroid peroxidase antibodies, are diagnostic. These antibodies, however, can be detected in elevated titres in the healthy general population. Treatment with corticosteroids is almost always successful, although relapse may occur if this treatment is ceased abruptly. Other forms of immunomodulation, such as intravenous immune-globulin and plasma exchange, may also be effective. Despite the link to autoimmune thyroid disease, the aetiology of Hashimoto's encephalopathy is unknown. It is likely that antithyroid antibodies are not pathogenic, but titres can be a marker of treatment response. Pathological findings can suggest an inflammatory process, but features of a severe vasculitis are often absent. The links between the clinical pictures, thyroid disease, auto-antibody pattern and brain pathology await further clarification through research. It may be that Hashimoto's encephalopathy will be subsumed into a group of nonvasculitic autoimmune inflammatory meningoencephalopathies. This group may include disorders such as limbic encephalitis associated with voltage-gated potassium channel antibodies. Some authors have suggested abandoning any link to Hashimoto and renaming the condition 'steroid responsive encephalopathy associated with autoimmune thyroiditis' to better reflect current, if limited, understanding of this condition.
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PMID:Hashimoto's encephalopathy : epidemiology, pathogenesis and management. 1785 Jan 70

Thyroid dysfunctions may be accompanied by numerous neurological and psychiatric disorders. The most known is cognitive impairment and depression in hypothyroid patients, as well as an increased risk of cerebrovascular accidents. A separate, although a rare entity, is Hashimoto's encephalopathy. In hyperthyroidism there is an increased incidence of psychiatric disorders, including apathetic hyperthyroidism and hyperthyroid dementia. Functional imaging of cerebral blood flow and metabolism helped establish both global and/or regional decrease of both cerebral blood flow and metabolism in hypothyroidism, particularly in regions mediating attention, motor speed and visuospatial processing. Hypothyroid dementia may be mediated by neurocircuitry different from that in major depression. Less is known on flow/metabolism changes in hyperthyroidism. Global blood flow may be slightly increased, with regional deficits of blood flow, particular in hyperthyroid dementia. As presented above radionuclide functional imaging showed some metabolic patterns in thyroid dysfunctions, but still many issues remain unresolved. In particular little is known about the underlying pathology of cognitive impairment and depression in hypothyroidism, which may differ from ones in euthyroid patients. Also little is known about the reversibility of changes in cerebral blood flow following thyroid replacement therapy. In hyperthyroid patients functional imaging might contribute to elucidate the background of apathetic hyperthyroidism and potential different background of psychiatric complications.
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PMID:Neurological and psychiatric disorders in thyroid dysfunctions. The role of nuclear medicine: SPECT and PET imaging. 1838 53

A 19-year-old female diagnosed with Graves' disease had treatment initiated with propylthiouracil (PTU). Pretreatment complete blood count and liver-associated enzymes (LAEs) were normal, but no further LAEs were obtained, reflecting U.S. guidelines written in 1995. Three months later, she presented with nausea, vomiting, abdominal pain, and jaundice. LAEs were markedly elevated with: total bilirubin, 6.5 mg/dl; aspartate aminotransferase (AST), 1747 IU/L; and alanine aminotransferase (ALT) 1589 UL/L. After 6 days at an outside hospital, she was transferred to our tertiary care center in acute liver failure with coagulopathy and stage II encephalopathy. Liver transplant evaluation was promptly initiated and she was listed as status 1. PTU was the only medication she had taken; and all serologic, autoimmune, and metabolic studies were negative. She demonstrated rapid clinical deterioration, and on hospital day 7 she underwent orthotopic liver transplant but succumbed to tonsillar herniation immediately after surgery. Pathology from her explanted liver revealed marked necrosis and collapse, consistent with her acute liver failure. PTU-associated hepatotoxicity and myelotoxicity have been well-recognized serious adverse effects for more than 50 years. However, as deaths related to hepatic injury from PTU are rare, American Thyroid Association guidelines do not call for routine monitoring of LAEs, although monitoring of white blood cell count levels is advised. Given the wide spectrum of PTU-related liver injury, ranging from asymptomatic elevations in ALT to fatal acute liver failure, we urge consideration of an LAE monitoring program to prevent irreversible liver damage and call for a reappraisal of monitoring guidelines in the United States.
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PMID:Gone (from the Physicians' desk reference) but not forgotten: propylthiouracil-associated hepatic failure: a call for liver test monitoring. 2057 20


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