EEG normalized, and the anti-TPO antibody level came down, though still elevated

EEG normalized, and the anti-TPO antibody level came down, though still elevated. == Case 2 == A 65-year-old woman was first seen in October 2019. elevated serum IgG4 levels in younger males with very high levels of thyroid antibodies. The role of the thyroid autoantibodies in THIQ the central nervous system (CNS) tissue damage remains unclear and these can act only as markers for diagnosis. Conversely, they have a role to play in determining the thyroid pathology more glandular fibrosis associated with thyro-peroxidase antibody than with the thyroglobulin antibody. == Summary == HE is a syndrome characterized by altered mental status, confusion, hallucinations, delusions, and sometimes seizures, in association with high serum anti-thyroid antibody concentration that is usually THIQ responsive to glucocorticoid therapy. Diagnosis requires the exclusion of other causes of encephalopathies and encephalitis including autoimmune encephalitis associated with neuronal surface antibodies and paraneoplastic ones. Diagnosis also is dependent on the demonstration of thyroid autoantibodies in serum. Since there is no direct pathophysiologic link between antithyroid antibodies, Hashimoto thyroiditis and the cerebral syndrome, the nomenclature HE could be misleading. The response to steroids led to a renaming of the syndrome to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), though some cases do not respond to steroids. In recent years, attention has turned to an aggressive form of Hashimotos thyroiditis accompanied by elevated serum IgG4 levels (IgG4-related disease). This is characterized by a higher incidence in men (5:1) than in women, onset at a younger age, more intense thyroid inflammation and higher antithyroid antibody titters. Such patients have excessive production of IgG4 + plasmacytes, which infiltrate various organs leading to their fibrosis and sclerosis, sometimes resulting in inflammatory tumors. HE is treated with corticosteroids along with treatment of the dysthyroid condition, if any. There are yet no guidelines regarding steroid dose and/or duration. Keywords:Hashimotos thyroiditis; Hashimotos encephalopathy, Anti-thyro-peroxidase antibody; Anti-thyroglobulin antibody; Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT); IgG4 related disorders == THIQ Introduction == Hashimotos Encephalopathy (HE) is a syndrome characterized by altered mental status, hallucinations, delusions, and sometimes seizures, in association with high serum anti-thyroid antibody concentration that is usually responsive to glucocorticoid therapy [1]. Diagnosis requires exclusion of other identifiable causes of encephalopathy [2,3]. Brain MRI and cerebrospinal fluid (CSF) studies are normal or non-specific [4]. HE is believed to be immune-mediated [5]. Three cases of HE are described herein followed by remarks and a review of the currently available literature. THIQ == Case Studies == == Case 1 == A 43-year-old man was admitted to a different institution in 2017 for evaluation of 2-month history of progressive behavioral changes characterized by being withdrawn gradually, getting irritable over trivial matters, progressively forgetful for recent events, inability to recognize family members and having complex visual hallucinations. In addition, he had five to six generalized tonicclonic seizures and had been having jerky movements of his arms mostly. He also had some degree of truncal and appendicular ataxia. After admission, his Mini Mental Status Examination (MMSE) score was 20/30. No focal neurological or meningeal signs were detected. Visual acuity and eye movements were normal. He was afebrile. MRI brain with and without contrast was essentially normal. Routine hematological and biochemical parameters were within normal limits. TSH was mildly elevated. HIV status was negative. CSF study showed a raised Eng protein content to 90 mg% and a mild lymphocytic pleocytosis (10 cells/cmm). CSF study for HSV1 was negative. Serum for anti-NMDAR, anti-VGKC and anti-LGI1 autoantibodies, were negative. EEG showed marked generalized slowing without any epileptiform features, periodic features, triphasic waves or FIRDA. The corresponding author was consulted for opinion regarding the high suspicion of a prion disease like Creutzfeldt Jakob disease (CJD). The only previously overlooked finding had been a goiter. This prompted checking on thyroid auto-antibodies. The THIQ anti-thyroglobulin antibody was undetected but the anti-TPO antibody level was raised to 300 mUnits/dL. This confirmed the diagnosis HE. He was given methylprednisolone 250 mg intravenously three times daily for 10 days and then switched to oral prednisolone 60 mg daily. He was reviewed after about 1 month when there had been no recurrence of seizures, no myoclonic jerks were seen and his cognition improved. EEG normalized, and the anti-TPO antibody level came down, though still elevated. == Case 2 == A 65-year-old woman was first seen in October 2019. She complained of gradually progressive difficulty in walking for the past 1 year. Later, her family members noticed that she was not speaking as clearly as.