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1/14. Pediatric manifestations of Hashimoto's encephalopathy.

    Hashimoto's encephalopathy is a steroid-responsive encephalopathy associated with elevated blood concentrations of antithyroid antibodies. The patients are usually euthyroid or mildly hypothyroid. The authors report two pediatric patients with Hashimoto's encephalopathy and review the literature. The clinical picture in adolescents, as with adults, is pleomorphic but frequently associated with seizures, confusion, and hallucinations. Alternatively, progressive cognitive decline manifested by a drop in school performance can be observed. The diagnosis of Hashimoto's thyroiditis is often overlooked at presentation and a high degree of suspicion is necessary for proper diagnosis.
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ranking = 1
keywords = thyroiditis
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2/14. Hashimoto's encephalopathy.

    Hashimoto's encephalopathy is a subacute condition associated with autoimmune thyroiditis. Its presentation varies from focal neurologic deficits to global confusion. Unlike encephalopathy associated with hypothyroidism, Hashimoto's encephalopathy responds to steroid therapy and not thyroxine replacement.
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ranking = 8.2568880262272
keywords = autoimmune thyroiditis, thyroiditis
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3/14. Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?

    Hashimoto's encephalopathy is a severe and rather infrequent clinical condition initially described in patients suffering from chronic lymphocytic thyroiditis. Its origin is still controversial but it can be agreed to have an autoimmune etiology. In fact, its most characteristic finding is the high titre of antithyroid antibodies, especially antimicrosomal. We describe three cases of Hashimoto's encephalopathy and establish a relationship between the clinical status, the antithyroid antibody levels and its response to corticosteroid treatment. There was an excellent response to corticosteroid treatment in all three cases. Interestingly, one case was associated with Graves' disease. Given this, and after the review of the literature, we believe that the term 'encephalopathy associated to autoimmune thyroid disease' could be more appropriate to define this entity. Finally, we suggest that autoimmune thyroid encephalopathy must be suspected in the face of unaccounted acute or subacute encephalopathy with high levels of antithyroid antibodies.
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ranking = 1
keywords = thyroiditis
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4/14. tremor and myoclonus heralding Hashimoto's encephalopathy.

    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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ranking = 8.2568880262272
keywords = autoimmune thyroiditis, thyroiditis
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5/14. Hashimoto's thyroiditis--a rare but treatable cause of encephalopathy in children.

    Hashimoto's encephalopathy is a very rare complication of Hashimoto's thyroiditis. It is a progressive or relapsing encephalopathy associated with elevation of thyroid specific autoantibodies. patients usually present when euthyroid and this diagnosis should be considered in any unexplained encephalopathy or progressive cognitive decline in the euthyroid patient.
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ranking = 5
keywords = thyroiditis
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6/14. A case of kearns-sayre syndrome with autoimmune thyroiditis and possible Hashimoto encephalopathy.

    The kearns-sayre syndrome (characterized by onset before 20, chronic ophthalmoplegia, pigmentary retinal degeneration and at least 1 of the following symptoms: ataxia, heart block and high protein content in the cerebrospinal fluid) is a severe variant of chronic progressive external ophthalmoplegia (CPEO) with frequent re-arrangements of the mitochondrial dna (mtDNA). The aim of this paper is to report a sporadic paediatric case of kearns-sayre syndrome with mtDNA heteroplasmic deletion, absence of cytochrome c oxidase in many muscle fibers, autoimmune thyroiditis followed by depressive phobic disturbances, slowing EEG, hyperreflexia, tremor and visual hallucinations, in which the diagnosis of possible encephalopathy associated with autoimmune thyroid disease (Hashimoto encephalopathy) was made. We speculated that in this patient, predisposed by mitochondrial deletion, anti-thyroid antibodies may have interfered with mitochondrial cerebral function, causing Hashimoto encephalopathy and facilitating ophthalmoplegia. It seems important to study anti-thyroid antibodies in every case of kearns-sayre syndrome, specially if ophthalmoplegia is recent, even in order to the therapy.
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ranking = 41.284440131136
keywords = autoimmune thyroiditis, thyroiditis
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7/14. Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996.

    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.
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ranking = 49.541328157363
keywords = autoimmune thyroiditis, thyroiditis
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8/14. Thyroid storm with encephalopathic symptoms due to Graves' disease and inappropriate secretion of thyrotropin.

    A 41-year-old woman is described, first hospitalized in the neurosurgical department for a transient ischemic attack with left hemiparesis followed after 6 hours by tonic-clonic seizures starting from the left hemiface and quickly generalized. brain computed tomography (CT) scan and magnetic resonance imaging were normal. Clinically the patient presented tremor, tachycardia, generalized muscle weakness, and profuse diaphoresis. T4 and T3 were elevated. The patient was transferred from the neurosurgical to the medical department where a thyroid storm due to autoimmune Graves' disease with normal thyrotropin (TSH) values responsive to thyrotropin-releasing hormone (TRH) stimulation was diagnosed. A syndrome of inappropriate secretion of TSH was suspected in an unusual presentation as autoimmune Graves' hyperthyroidism. The TSH alpha-subunit and alpha-subunit/TSH molar ratio were normal, which supported the diagnosis of non-neoplastic inappropriate secretion of TSH. However, severe autoimmune Graves' hyperthyroidism is very rare indeed because autoantibodies to thyroid antigens are generally non-detectable in such patients. Our patient was treated initially with barbiturates, then with dexamethasone, Lugol's solution, methimazole and propranolol. Treatment of this patient proved difficult, and definitive improvement was obtained only after triiodothyroacetic acid administration, but methimazole and propranolol administration could not be discontinued. Fine needle aspiration biopsies of the thyroid in 2 occasions showed follicular or follicular-papillary proliferation with lymphocytic infiltration as in chronic thyroiditis. The patient is now in good clinical conditions and is followed up regularly. Autoimmune Graves' hyperthyroidism may be associated in extremely rare instances with non neoplastic inappropriate secretion of TSH.
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ranking = 1
keywords = thyroiditis
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9/14. Cerebellar subacute syndrome due to corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called "Hashimoto's encephalopathy").

    The corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (the so-called "Hashimoto's Encephalopathy") is a rare disorder with multiple symptomatology, breaking out with an acute or subacute onset and having a relapsing course, not correlated to thyroid hormone levels, with autoimmune pathogenesis, and usually associated with Hashimoto's thyroiditis. In this paper, we report on a case study regarding a 46 year-old woman showing a subacute course cerebellar syndrome, associated with Hashimoto's thyroiditis, diagnosed as "Hashimoto's encephalopathy". The possible pathogenesis and the major aspects of the differential diagnostic sector are discussed with particular reference to an ataxic syndrome caused by a progressive non-familial adult onset cerebellar degeneration (PNACD), associated with the thyroid disease itself.
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ranking = 43.284440131136
keywords = autoimmune thyroiditis, thyroiditis
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10/14. Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy.

    Hashimoto's encephalopathy (HE) is a severe but treatable condition that rarely complicates Hashimoto's thyroiditis. Clinically it is characterized by progressive or relapsing symptoms, including tremor, myoclonus, stroke-like episodes, seizures, impairment of consciousness, and dementia. We describe a patient presenting with recurrent generalized convulsive status epilepticus (GCSE), despite antiepileptic medications, who was successfully treated with methylprednisolone. Our observation confirms that the clinical spectrum of HE at presentation is heterogeneous and diagnosis is often difficult. This case highlights the crucial importance of antithyroid antibody measurement in patients presenting with otherwise unexplained episodes of GCSE with or without adjunctive signs of encephalopathy or thyroiditis.
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ranking = 2
keywords = thyroiditis
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