A 15-year-old girl is described with encephalopathy associated with Ha
shimoto thyroiditis which subsequently developed into autoimmune thyro
toxicosis (hashitoxicosis) and distal renal tubular acidosis at age 5
years, pernicious anemia at age 9, and encephalopathy at age 12. Thyro
id hormone levels were within the normal ranges at the time of neurolo
gic presentation, Serum IgG concentration and oligoclonal IgG bands in
cerebrospinal fluid closely correlated with the severity of neurologi
c symptoms. Treatment with glucocorticoids improved the level of consc
iousness but her mental state continued to deteriorate. Repeated crani
al computed tomographic scans revealed progressive symmetric atrophy i
n the gray matter without infarction. These findings suggest that ence
phalopathy associated with Hashimoto thyroiditis is caused by an antin
euronal antibody-mediated reaction.