Data regarding the course of HE are scarce and rely on clinical follow-ups which usually lasted from 6 to 24 months. However, cases with partial remission or progression even up to death were described. HE is characterized by a remission of NPS, followed by normalization of neuroimaging and EEG after corticosteroid (glucocorticoid) therapy (steroid-responsiveness) or, if resistant, escalating immunosuppression. Abnormalities in neuroimaging, electroencephalogram (EEG), and cerebrospinal fluid (CSF) are not required to diagnose HE although being present in more than 50% of the cases (usually elevated CSF protein, generalized slowing of EEG-waves, and T2-hyperintense foci on brain MR imaging). HE’s clinical onset is usually subacute and its clinical presentation is multifaceted including neuropsychiatric symptoms (NPS), such as cognitive decline, behavioral symptoms, depression, psychosis, cerebral ischemia, seizure, myoclonus, tremors, and fluctuating levels of consciousness. Most patients with HE are euthyroid or have subclinical hypothyroidism, and 20% of them have overt hypothyroidism while hyperthyroidism is rare. These antibodies are a hallmark of Hashimoto thyroiditis that occurs in 1–5% of the general population. Hashimoto encephalopathy (HE) is a rare pathological condition with an estimated prevalence of 2/100,000 and various neuropsychiatric symptoms (NPS) combined with positive serum antithyroid-peroxidase autoantibodies (TPO-Abs, usually >200 U/mL). Another special feature of this case report is the linkage of HE to an autoimmune polyendocrine syndrome (type 3B) affecting the gastroduodenum in addition to the thyroid gland. A potential contribution of bipolar disorder and metabolic encephalopathies due to severe hypothyroidism, glucocorticoid treatment, accelerated thyroid hormone replacement therapy, or vitamin B deficiency is critically discussed. Diagnostic analyses found no evidence of limbic encephalopathies characterized by serum antibodies against intracellular, synaptic, or further cell surface antigenic targets, neoplasm, and connective tissue or vasculitis diseases. Antithyroid-peroxidase autoantibodies, the hallmark of autoimmune thyroiditis, were found in the serum and also in the cerebrospinal fluid. This presentation includes one of the longest lasting follow-up studies of HE considering the neuropsychiatric symptoms (here delirium, mania, and EEG-slowing) and their relation to serum autoantibody levels. We report the case study of a 57-year-old Caucasian female with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), commonly termed Hashimoto encephalopathy (HE).
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