Hashimoto encephalopathy is an autoimmune encephalopathy predominantly diagnosed in the adult population. In the pediatric population, the mean age is determined to be 14 years, with the majority of adolescent females. The diagnosis of Hashimoto encephalopathy is clinical and based on the highly variable neuropsychiatric conditions, often affecting more than one area of the central nervous system, the detection of antiperoxidase or antithyroglobulin antibodies in serum, and on the elimination of other potential etiologies. The pathophysiology of Hashimoto encephalopathy is still unclear; autoimmune and vasculitic mechanisms can play an active role. A clinical response to corticosteroid therapy is supportive of the diagnosis. The occurrence of Hashimoto encephalopathy is unrelated to the patient's thyroid function status. Neuroimaging studies, EEG, and cerebrospinal fluid analysis can be supportive, although they are not diagnostic. MRI studies in children have mostly shown prolonged T2-weighted signals of the subcortical white matter, suggesting inflammation or demyelination.
The current standard treatment is the use of corticosteroids in addition to the treatment of any concurrent dysthyroidism. Other options are immunoglobulins and plasmapheresis.
We describe the case of a 17-year-old girl who admitted due to headache followed by hearing loss and disorentiation in depression status and anorexia nervosa with significant weight loss. She had a 8-year history of celiac disease. She had normal general and neurological examination, height 160 cm (25-50 pc), weight 37 kg (<3 pc), and body mass index 14.5 kg/m2 (< 3 pc). She had normal transglutaminase IgA antibody, significantly elevated thyroperoxidase (TPO) antibody titers 5.674U/mL in euthyroid function, consistent with the diagnosis of Hashimoto's thyroiditis. The analysis of cerebrospinal fluid showed high protein level and TPO antibodies. Cerebral MRI showed T2 hyperintensity in the periventricular white matter. Electroencephalography was normal. Steroid therapy was initiated with methylprednisolone intravenously for 3 days, followed by prednisone orally.
Encephalopathy as a complication of Hashimoto thyroiditis was first described by Brain and Coworkers in 1966 in the adult patient. In recent years have been increasingly recognized in both adult and pediatric patients. Particularly, the association between neuropsychiatric symptoms and Hashimoto thyroiditis should lead to early suspicion of this disorder. Prodromal headache and sensori-neural hearing loss have been reported in the autoimmune encephalitis. Therefore, this case report aims to raise awareness about the extreme variability of the clinical spectrum of the Hashimoto encephalopathy especially in the presence of a known autoimmune disease and the importance of multidisciplinary approach for a early recognition.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology