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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two women (aged 12 and 32 years) and a man aged 40 years presented with fluctuating confusional states, depressed level of consciousness, seizures and tremor or myoclonic activity; hallucinations occurred in two of them. Laboratory examinations showed antibodies to thyroid peroxidase in serum, elevated protein levels in CSF and normal cerebral MRI. The EEG findings were indicative of diffuse encephalopathy. Hashimoto's encephalopathy was diagnosed. One patient improved dramatically on prednisone therapy, the other two patients recovered spontaneously. Hashimoto's encephalopathy is a subacute, fluctuating encephalopathy with combinations of impaired level of consciousness, involuntary movements, and epileptic or psychiatric symptoms. It occurs in patients with antithyroid antibodies. It is important to consider this diagnosis, since some patients may benefit from treatment with corticosteroids.
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PMID:[Variable manifestations of Hashimoto's encephalopathy]. 1041 87

Hashimoto's encephalitis was first described more than 30 years ago. The clinical picture is typically that of a subacute encephalopathy with a moderate to marked alteration of consciousness, seizures, myoclonus or tremulousness. Additional stroke-like episodes can occur along the course of the disease which may be monophasic or relapsing. The diagnosis of Hashimoto's encephalitis requires the presence of an elevated titer of antithyroid antibodies (mainly anti-thyroperoxidase and also anti-thyroglobulin) which is not necessarily associated with obvious thyroid dysfunction. The results of neurologic investigations are not specific and show typically a global slowing of the EEG, a moderately high CSF protein content and a normal or near normal imaging except in rare cases. The disorder is considered autoimmune and is remarkably responsive to corticosteroids which must be started as soon as possible after the diagnosis has been confirmed biologically. The long-term prognosis is usually good but some patients may die or present major neurologic sequelae.
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PMID:[Hashimoto's encephalitis]. 1240 5

Characteristic clinical findings of Hashimoto's encephalopathy (HE) are stroke-like episodes, epileptic seizures, myoclonus, psychosis, and progressive cognitive impairment. Diagnosis of HE is supported by elevated antithyroid antibodies, an abnormal EEG, and by good response to steroids. We report on a 74-year-old female patient with a severe depressive episode who showed no treatment response to citalopram 40 mg/day and venlafaxine 150 mg/day. Diagnostic examination revealed an abnormal EEG, elevated thyroid peroxidase antibodies (TPO-Ab), and older postinflammatory changes in thyroidal sonography. We diagnosed a depression in HE and began treatment with prednisolone 70 mg/day with stepwise dose reduction, continuing treatment with venlafaxine 150 mg/day. Within 4 weeks of treatment, the severe depressive episode disappeared as well as abnormal EEG. In addition, serum values of TPO-Ab decreased. In HE, depressive symptoms can possibly be seen in a subgroup of patients or in the early course of the disease. Diagnosis of HE should be included in diagnostic procedures in cases of therapy-refractory depression because of a good response of HE to steroids.
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PMID:[Depression in Hashimoto's encephalopathy. Successful treatment of a severe depressive episode with a glucocorticoid as an add-on therapy]. 1544 21

A 37-year-old woman developed memory disturbance and seizures preceded by headache and high fever. Physical examination revealed persistent high fever, confusion, neck stiffness, anterograde and retrograde amnesia and disorientation. CSF showed pleocytosis and normal glucose level, and negative herpes simplex virus DNA on PCR. The fluid attenuated inversion recovery (FLAIR) MRI of the brain demonstrated nearly symmetric high signal intensity areas in the bilateral mesial temporal lobes. The tentative clinical diagnosis was non-herpetic acute limbic encephalitis (NHALE), and administration of methylprednisolone improved her conditions. Laboratory tests revealed mild hypothyroidism with high titers of serum antibodies against TPO and thyroglobulin, consistent with Hashimoto's disease. In addition, antibodies against amino terminal of alpha-enolase in the serum and those against glutamate receptor (GluR) epsilon2 in the serum and CSF were positive. A final diagnosis of Hashimoto's encephalopathy associated with GluRepsilon2 antibody was made. The present case suggests that NHALE-like clinical manifestation can be produced by autoimmune-mediated encephalopathies.
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PMID:[Non-herpetic acute limbic encephalitis-like manifestation in a case of Hashimoto's encephalopathy with positive autoantibodies against ionotropic glutamate receptor epsilon2]. 1809 94

Hashimoto's encephalopathy (HE) is a controversial neurological disorder that comprises a heterogenous group of neurological symptoms that manifest in patients with high titers of antithyroid antibodies. Clinical manifestations of HE may include encephalopathic features such as seizures, behavioral and psychiatric manifestations, movement disorders, and coma. Although it has been linked to cases of Hashimoto's thyroiditis or thyroid dysfunction, the most common immunological feature of HE is the presence of high titers of antithyroglobulin or anti-TPO (antimicrosomal) antibodies. At present, it is unclear whether antithyroid antibodies represent an immune epiphenomenon in a subset of patients with encephalopathic processes or they are really associated with pathogenic mechanisms of the disorder. The significance of classifying encephalopathies under the term HE will be determined in the future once the relevance of the role of antithyroid antibodies is demonstrated or dismissed by more detailed experimental and immunopathological studies. The responsiveness of HE to steroids or other therapies such as plasmapheresis supports the hypothesis that this is a disorder that involves immune pathogenic mechanisms. Further controlled studies of the use of steroids, plasmapheresis, or immunosuppressant medications are needed in the future to prove the concept of the pathogenic role of antithyroid antibodies in HE.
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PMID:Hashimoto's encephalopathy. 1899 Jan 31

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

Objective. To describe the clinical characteristics and radiological findings in two patients with subacute encephalitis associated with elevated serum voltage-gated potassium channel antibody (VGKCAb) and antithyroperoxidase (TPO) antibody. Case Reports. Case 1: 63-year-old woman was admitted for altered mental status and possible seizure activity. MRI brain showed hyperintensity in the bilateral hippocampal areas. She was positive for VGKCAb and anti-TPO antibodies. She was treated with steroids, IVIG, plasma exchange and azathioprine. After 8 months, she had marked improvement in her memory and seizures. Case 2: 61-year-old woman was admitted for video EEG monitoring of unclassified seizure and cognitive function decline. MRI of the brain showed mild hyperintensity in bilateral hippocampal areas and significant atrophy in the frontotemporal lesion. Anti-TPO antibody and VGKCAb were positive. She was treated with steroids, plasma exchange and azathioprine. After 9 months, she had marked improvement in her memory and seizures. Conclusion. Autoimmune subacute encephalitis appears to be an underdiagnosed entity. It is important to screen patients with subacute encephalitis for anti-TPO antibody and VGKCAb, particularly in the presence of seizures. Immunosuppressive therapy appears to be effective in treating this entity.
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PMID:Immunotherapy responsive autoimmune subacute encephalitis: a report of two cases. 2033 72

Hashimoto's encephalopathy, a steroid-responsive encephalopathy, presents with cognitive deterioration, psychiatric symptoms, and seizures and is often associated with elevation of anti-thyroid peroxidase antibodies or thyroid dysfunction. Because of the absence of a gold-standard diagnostic test and serological marker of disease, improvement in cognitive impairment is an important sign when assessing response to corticosteroid treatment. The purpose of this case study is to present a methodology for tracking neurocognitive functioning using computerized testing (CNS Vital Signs) in a 14-year-old girl with Hashimoto's encephalopathy. Computerized neuropsychological testing was done prior to commencing corticosteroid treatment, as well as at 7 follow-up assessments over 1 year. Improvement in cognitive, psychological, and behavioral functioning heralded a response to treatment, which was sustained during tapering of medication. Computerized neuropsychological testing seems to be a viable tool for tracking cognitive functioning (when rapid assessments are desired) and assessing response to corticosteroid treatment in Hashimoto's encephalopathy.
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PMID:A methodology for assessing treatment response in Hashimoto's encephalopathy: a case study demonstrating repeated computerized neuropsychological testing. 2131 Oct 82

Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare condition whose pathogenesis is unknown, though autoimmune-mediated mechanisms are thought to be involved. The prevalent neurological manifestations of this disorder are epileptic seizures and psychocognitive disorders associated with EEG alterations. High anti-thyroid antibody titers (particularly in cerebrospinal fluid) and the effectiveness of steroid therapy are usually considered to be crucial elements in the diagnostic process. We describe a 19-year-old female patient who had been referred to the psychiatric unit because of behavioral disorders characterized predominantly by delirium with sexual content. She developed recurrent focal seizures characterized by atypical ictal semiology (repetitive forceful yawning) and a rare EEG pattern (recurrent seizures arising from the left temporal region without evident "encephalopathic" activity). The presence of anti-thyroperoxidase antibodies in her cerebrospinal fluid and a good response to steroids confirmed the diagnosis of HE. The atypical presentation in the case we describe appears to widen the electroclinical spectrum of HE and highlights its importance for differential diagnosis purposes in the neuropsychiatric setting.
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PMID:Recurrent partial seizures with ictal yawning as atypical presentation of Hashimoto's encephalopathy (steroid-responsive encephalopathy associated with autoimmune thyroiditis). 2203 May 35

A 16-year-old girl with a history of Graves' disease presented with two episodes of generalised tonic-clonic seizures, necessitating intensive care admission. Laboratory examination demonstrated a suppressed thyroid-stimulating hormone level with dramatically elevated free triiodothyronine, free thyroxine and thyroid-stimulating immunoglobulins. Cerebrospinal fluid analysis showed oligoclonal banding in the absence of pleocytosis, thyroid peroxidase antibodies or infection. Neuroimaging revealed the presence of a congenital arachnoid cyst in the right temporal lobe. Despite restoration of euthyroidism and administration of antiepileptic and antiviral drugs, neurological features persisted. Subsequently, intravenous corticoids were administered to exclude the contribution of an underlying autoimmune encephalopathy. The patient gradually recovered and, in retrospect, elevated serum N-methyl-D-aspartic acid-receptor (NMDA-R) antibodies were detected. Although this patient presented with an intracerebral arachnoid cyst that can act epileptogenic per se, the combination of prolonged postictal encephalopathy with unresponsiveness to antiepileptic measures, absence of focal epileptiform activity on EEG, response to corticoids and serum NMDA-R antibody positivity favours the diagnosis of autoimmune NMDA-R encephalitis in this case.
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PMID:Seizures and postictal stupor in a patient with uncontrolled Graves' hyperthyroidism. 2266 27


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