![]() ![]() Overall, the weaknesses of the criteria and their misuse characterizing as HE any neurological syndrome with positive TPO antibodies that responds to steroids, raise doubts about the clinical usefulness of the term HE. Finally, most patients reported with HE are not investigated for neuronal surface antibodies. However, the clinical criteria and paraclinical findings do not identify the patients that will respond to steroids (<30%). Another limitation is that the diagnostic confirmation of HE depends on steroid-responsiveness. The significance of antibodies against the amino (NH2)-terminal domain of α-enolase, considered a potential biomarker of HE, is also unclear. Moreover, the specificity of TPO antibodies is poor as they are also found in 13% of healthy persons. One is that there are no specific biomarkers of the disease. However, the diagnosis of HE has several important limitations. The beneficial effect of steroids was emphasized by renaming the disease ‘steroid-responsive encephalopathy associated with autoimmune thyroiditis’ (SREAT). HE is usually considered in patients with subacute cognitive deterioration, myoclonus, change in behaviour, or seizures, accompanied by normal or non-specific MRI and CSF findings, normal thyroid function or mild hypothyroidism, increased serum levels of thyroid peroxidase (TPO) antibodies, and clinical response to steroids. This chapter critically reviews the diagnostic criteria of Hashimoto encephalopathy (HE) and the misuse of this diagnosis. This case illustrates that physicians should be aware of the treatable condition of HE as a possible diagnosis in patients with bilateral pinpoint pupils and drowsiness but without corresponding MRI lesions. Her symptoms improved spontaneously including gradually normalization of her pupil size in 3 days and clear consciousness in 6 days therefore, corticosteroid was not administered. Of note, she had elevated serum levels of anti-TG antibody (282 IU/mL) and anti-TPO antibody (117 IU/mL). She had an elevated protein level (123 mg/dl) in cerebrospinal fluid without pleocytosis. Electroencephalography showed intermittent diffuse slowing. Brain MRI showed two tiny infarctions at the left midbrain and left thalamus, but did not show a pontine lesion, and demonstrated patent vertebral/basilar arteries. Neurologically, she was drowsy and had bilateral pinpoint-sized pupils without a light reflex, which probably indicated a bilateral pontine lesion. Pupil size change has never been previously reported as a presentation of HE.Ī 65-year-old woman without previous known thyroid disease presented with acute onset of drowsiness and blurred vision. Hashimoto's encephalopathy (HE) is an autoimmune-mediated encephalopathy with various manifestations. ![]()
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