Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the clinical laboratory, electroencephalography (EEG), magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT) findings in a 15 year-old euthyroid girl with autoimmune thyroiditis and encephalopathy. She had stupor, coma and generalized tonic clonic seizure preceded by tremor and myoclonus with a previous misdiagnosis of epilepsy and encephalitis. Response to steroid after the 3rd relapse was excellent. Another four children in the literature are also discussed.
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PMID:Tremor and myoclonus heralding Hashimoto's encephalopathy. 1108 93

Hashimoto's encephalopathy (HE) is an acute or subacute relapsing disorder usually affecting euthyroid patients with evidence of autoimmune thyroiditis. The neurological manifestations are non-specific, with subacute cognitive impairment, movement disorders, generalized seizures, focal neurological symptoms such as stroke-like episodes, or psychiatric disturbances. Autoimmune phenomena are likely to play an etiological role. Magnetic resonance imaging (MRI) findings are usually normal or show non-specific changes. We report the case of an 11-year-old girl with autoimmune thyroiditis who presented acutely with a complex neuropsychiatric disorder in association with MRI evidence of focal involvement of the nucleus accumbens (NA). The NA, a ventral striate nucleus, is part of a complex dopaminergic network. Lesions to the NA result in several psychiatric symptoms, such as attention-deficit hyperactivity disorders. In this patient, we observed alternating phases of stupor and hyperkinetic-anxious behavior, with marked instability. The pathogenetic mechanism and the anatomic and functional correlations are briefly discussed.
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PMID:Hashimoto's encephalopathy with selective involvement of the nucleus accumbens: a case report. 1594 10

We report the first case of an autoimmune thyroid encephalopathy presenting with multifocal motor status epilepticus. A 37-year-old female patient was admitted with multifocal motor seizures intractable to intravenous status epilepticus treatments, asymmetrical quadriparesis, truncal ataxia and continuous semi-rhythmical jerks. Pathological signal alterations were detected in both precentral cortices in MRI examination. Autoimmune thyroiditis was diagnosed after radiological examinations of the thyroid gland and thyroid function tests. Seizures promptly ceased following intravenous steroid treatment. Immunohistochemistry studies showed mild to moderate neuronal staining with the plasma and CSF samples. Remarkably, autoimmune thyroiditis may present with migrating focal motor status epilepticus. We recommend anti-thyroid antibody screening for multifocal motor status epilepticus cases of unspecified cause.
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PMID:Autoimmune thyroid encephalopathy presenting with epilepsia partialis continua. 1692 5

Hashimoto's encephalopathy (HE) is not an often complication of autoimmune thyroiditis, usually at the time the patient is already euthyroid i.e. with normal TSH-value but positive antibodies to thyroid hormones. Signs and symptoms are acute or sub-acute, such as cerebrovascular accident, epileptic seizures generalised, myoclonic or partial, cognitive function disorder with dementia and psychical disorders. Pathological findings, even non-specifical, can be found in cerebrospinal fluid, by electroencefalography (EEG) or by brain scan with magnetic resonance (MR). Pathogenesy of the disease is unclear; however, corticoid treatment is usually succesful. Therefore, HE should be taken into consideration after elimination of all other causes of encephalopathy, with thyroiditis in family history in particular.
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PMID:[Hashimoto's encephalopathy]. 1706 9

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

Hashimoto's encephalopathy (HE) is a relapsing, but exquisitely corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis. Although a rare disease, with just over 100 cases reported, it may be under-recognised. Its presentation can be protean with prominent neuropsychiatric features, stroke-like episodes, seizures and myoclonic jerks. Prompt corticosteroid treatment usually leads to rapid recovery. Here we report a patient with HE, initially presenting with florid neuropsychiatric symptoms. Recent developments in the understanding of this condition are discussed.
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PMID:Hashimoto's encephalopathy masquerading as acute psychosis. 1831 25

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. It may have vascular, immune-mediated, neoplastic or metabolic-toxic causes. The origin of EPC has been linked with the motor cortex. This has been solidly supported by sophisticated electrophysiological studies. Here, a series of video sequences from patients with EPC (due to Rasmussen encephalitis, early-stage multiple sclerosis, and steroid responsive encephalopathy with autoimmune thyroiditis), and other cases with repetitive myoclonic jerks or movement disorders (myoclonic epilepsy associated with ragged-red fibers, Jacksonian march, myoclonic seizures in other types of frontal lobe or idiopathic generalized epilepsies, and different types of tremor) is presented. [Published with video sequences].
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PMID:Epilepsia partialis continua: semiology and differential diagnoses. 1836 24

Steroid responsive encephalopathy associated withSteroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), (also known as Hashimoto's encephalopathy) is a rare autoimmune encephalitis associated with high antithyroid antibodies, and presents with a relapsing-remitting or monophasic course, consisting of confusion, seizures, psychosis, dementia, or stroke-like episodes. In this report, a late onset depression was the initial presentation of SREAT in a middle aged patient, something rarely described before, indicating the need to suspect SREAT as a possible etiology for depression in this age group.
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PMID:Steroid responsive encephalopathy associated with autoimmune thyroiditis presenting with late onset depression. 2083 Oct 30

Steroid-responsive encephalopathy with autoimmune thyroiditis is a relatively uncommon entity in the pediatric population. Although the pathogenesis of steroid-responsive encephalopathy with autoimmune thyroiditis is uncertain, an autoimmune mechanism is suspected to be the most likely cause. Seizures of unknown etiology are a common presenting sign in the pediatric intensive care unit, and steroid-responsive encephalopathy with autoimmune thyroiditis should be considered as a possible cause of recurrent seizures. The outcome of steroid-responsive encephalopathy with autoimmune thyroiditis is variable in children, and is partly dependent on early diagnosis and the administration of intravenous steroids. Only 31 pediatric cases of steroid-responsive encephalopathy with autoimmune thyroiditis were described in the English-language literature since 1966. Very few were reported in the United States or among males. We describe a 13-year-old boy presenting with a new onset of seizures as a manifestation of steroid-responsive encephalopathy with autoimmune thyroiditis. This report presents, to the best of our knowledge, the youngest male with steroid-responsive encephalopathy with autoimmune thyroiditis, and the only reported child with other autoimmune manifestations in addition to encephalopathy.
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PMID:Steroid-responsive encephalopathy and autoimmune thyroiditis in a young boy. 2176 57

Hashimoto encephalopathy (HE) is a rare and often reversible neurological syndrome associated with autoimmune thyroiditis and steroid-responsiveness. This syndrome includes behavioral symptoms like delusions and delirium, mood disturbances, epilepsy, progressive cognitive impairment and alteration of vigilance and consciousness with confused state until coma. Two subtypes of clinical presentation are described: ictal onset with seizures and stroke like episode and insidious onset with progressive dementia. The pathogenesis is uncertain; several theories have been proposed: autoimmune, vasculitic and demyelinating. Here, we report the case of a patient with HE who was submitted to exhaustive neuropsychological exams in the premorbid and the acute phase, and following resolution of the acute phase. After the initial confusional state resolved, results of the neuropsychological exams revealed a diffuse pattern of cognitive impairment that eventually evolved toward a selective deficit in executive functions. This pattern of cognitive impairment suggests that, after an initial phase characterized by diffuse brain involvement, our patient was primarily affected by frontal lobe sufferance.
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PMID:Hashimoto's encephalopathy: neuropsychological findings. 2199 32


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