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Hashimoto encephalopathy is characterized by severe neuropsychiatric findings, including psychosis, confusion, seizures, stupor, stroke-like episodes, tremor, and myoclonus. The combination of findings is variable. Hashimoto encephalopathy constitutes an important differential diagnosis in patients with encephalopathy. The triad of encephalopathy, corresponding electroencephalographic slowing, and increased protein content in cerebrospinal fluid should prompt testing of anti-thyroid antibodies in blood and cerebrospinal fluid. Elevated antibody levels support the diagnosis. We describe a 15-year-old girl with a fluctuating course of Hashimoto encephalopathy. Electroencephalograms revealed no specific alterations, but widespread slowing of the background activity occurred during two episodes of fluctuating encephalopathy. Cortical edema was indicated by cranial magnetic resonance imaging during the first episode of encephalopathy, in the context of cerebral seizures. Laboratory findings were in accordance with Hashimoto encephalopathy, which was steroid-responsive.
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PMID:Hashimoto encephalopathy in a 15-year-old-girl: EEG findings and follow-up. 1974 53

Hashimoto's encephalopathy (HE) is a rare, still not well understood, autoimmune disease with neurological and psychiatric manifestations. and elevated titers of antithyroid antibodies in serum and cerebrospinal fluid (CSF) as a hallmark of the disease. Patients are mostly women. Current diagnostic criteria include corticosteroide responsiveness, but it is the case in only 50% of patients with HE. In steroid non-responders other immunomodulatory therapies or plasmapheresis could be applied. Disease course can be acute, subacute, chronic or relapsing-remitting. Two distinct forms emerged from the reported cases: a vasculitic type characterized by multiple relapsing-remitting stroke-like episodes and mild cognitive impairment and a diffuse progressive type characterized by dementia and psychiatric symptoms. Both forms may be accompanied by depressed level of consciousness (stupor or coma), tremor, seizures, or myoclonus. We present two patients with two distinct forms of HE who had different clinical manifestations and response to therapy.
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PMID:Clinical manifestations, diagnostic criteria and therapy of Hashimoto's encephalopathy: report of two cases. 1984 19

The literature is reviewed. Hashimoto encephalopathy is a steroid-responsive encephalopathy associated with Hashimoto thyroiditis (SREHT). The incidence of SREHT in childhood is underestimated. Two subtypes have been described. The diffuse progressive type is associated with insidious onset and progressive impairment of mental status, such as confusion, somnolence, and psychosis. The vasculitic type is characterized by acute stroke-like episodes associated with focal neurologic features and seizures. Hashimoto encephalopathy (SREHT) is a clinical condition with elevated thyroid antibody titers encompassing persistent or relapsing seizures, myoclonus, focal neurologic deficits and neuropsychiatric disorders such as psychosis, delusions and hallucinations, and affective disorders. By far, the vast majority of reports on paediatric and adult patients describe a dramatic clinical response to either methylprednisolone pulse therapy or daily oral prednisone or prednisolone.
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PMID:[Undetected Hashimoto encephalopathy--a diagnostic challenge in child psychiatry and child neurology]. 2004 73

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

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

Hashimoto's encephalopathy (H.E.) is probably of autoimmune etiology, and manifests with seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, myoclonus. It is presumed to be autoimmune in origin with high serum titers of antithyroid peroxidase antibodies (anti-TPA). Thyroid function might often be normal. The diagnosis is arrived at by excluding other toxic, metabolic and infectious causes of encephalopathies, supportive clinical profile, elevated thyroid antibodies and optimum steroid response. We present the characteristic phenotypic manifestations, magnetic resonance imaging and electroechography observations and response to immunomodulation with follow-up in three cases of H.E. All the three cases manifested with subacute to chronic progressive encephalopathy, cerebellar dysfunction, seizures, behavioral abnormalities and oculomotor disturbances and had evidence of hypothyroidism, elevated titers of anti-TPA and positive thyroid anti-microsomal antibodies. Atypical and uncommon presentations are known. This report emphasizes that a high index of suspicion is often required in cases with "investigation negative encephalopathy" for early diagnosis of H.E.
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PMID:Steroid-responsive encephalopathy in autoimmune thyroiditis: Clinical spectrum and MRI observations in three cases. 2202 37

The authors describe the case of a 61-year-old woman who was admitted to our intensive care unit (ICU) due to impaired consciousness associated with generalised seizures. Her cerebrospinal fluid, electrolytes, acid-base analysis, and common laboratory and toxicology tests were normal. An MRI ruled out the presence of stroke or haemorrhage but showed severe leukoencephalopathy. Parkinson's disease, Creuzfeld-Jacob disease, vascular alterations, cancer, and rheumatological and metabolic diseases were evaluated and excluded. In view of her history of hypothyroidism despite adequate hormonal replacement and clinical behaviour, Hashimoto's encephalopathy was considered. Anti-thyroperoxidase levels were above 3000 IU/ml. The patient received 5 g of methylprednisolone followed by prednisone, but after a favourable initial response, returned to a comatose state. However, after administration of intravenous immunoglobulin (IVIG) 2 g/kg, the patient recovered with resolution of neurological symptoms and was discharged from the ICU 4 days after finishing IVIG treatment.
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PMID:Successful response to intravenous immunoglobulin as rescue therapy in a patient with Hashimoto's encephalopathy. 2280 79

We report a longstanding selective memory deficit in a euthyroid 45-year-old woman who was being treated with levothyroxine for Hashimoto thyroiditis. The patient had complained of memory problems and deterioration of her concentration skills for about 2 years. Her endocrinologist thought that she was depressed. The patient's physical examination was normal. She scored a full 30 points on the Mini-Mental State Examination, but neuropsychological evaluation showed a significant deficit in her verbal memory. Routine blood tests and cerebrospinal fluid analysis showed only antithyroid peroxidase antibodies. Brain magnetic resonance imaging was normal. Electroencephalogram showed scarce intermittent bilateral multifocal theta waves. We increased the patient's daily dose of levothyroxine and started her on dexamethasone therapy. Five months later, we repeated the entire evaluation and found both her cognitive function and her electroencephalogram to be normal. Autoimmune encephalopathy associated with Hashimoto thyroiditis is already known to present with either stroke-like episodes or diffuse progressive deterioration. Our patient shows that the encephalopathy can present as a chronic selective memory deficit that can spare executive functions and short-term memory. This presentation can be missed or mistaken for depression, but can be diagnosed with a detailed neuropsychological evaluation.
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PMID:A selective memory deficit caused by autoimmune encephalopathy associated with Hashimoto thyroiditis. 2296 Apr 39

Hashimoto's encephalopathy is an immune-mediated disorder characterized by acute or subacute encephalopathy related to increased anti-thyroid antibodies. Clinical manifestations of Hashimoto's encephalopathy may include stroke-like episodes, altered consciousness, psychosis, myoclonus, abnormal movements, seizures, and cognitive dysfunction. Acute cognitive dysfunction with convulsion as initial clinical manifestations of Hashimoto's encephalopathy is very rare. We report a 65-year-old man who developed acute onset of cognitive decline and convulsion due to Hashimoto's encephalopathy.
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PMID:Hashimoto's Encephalopathy Presenting with Acute Cognitive Dysfunction and Convulsion. 2464 76

Both severe thyrotoxicosis and hypothyroidism may affect brain function and cause a change in consciousness, as seen with a thyroid storm or myxedema coma. However, encephalopathy may also develop in patients with autoimmune thyroid diseases independent of actual thyroid function level, and this is known as Hashimoto's encephalopathy. Although most patients are found to have Hashimoto's thyroiditis, less frequently they have Graves' disease. Clinical manifestations include epilepsy, disturbance of consciousness, cognitive impairment, memory loss, myoclonus, hallucinations, stroke-like episodes, tremor, involuntary movements, language impairment, and gait impairment. Hashimoto's encephalopathy is a relatively rare disease. As a good response can be obtained with corticosteroid therapy, early diagnosis and treatment is very beneficial for patients. Here we report three patients with Hashimoto's encephalopathy with typical manifestations of hallucinations that were associated with hypothyroidism, hyperthyroidism, and euthyroid status, respectively. They all showed a dramatic response to methylprednisolone pulse therapy.
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PMID:Hashimoto's encephalopathy: report of three cases. 2544 53


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