Abstract

Abstract Objective: Hashimoto encephalopathy (HE) is a rare neurological condition with pathophysiology associated with elevated autoimmune anti-thyroid antibodies. The condition, associated with Hashimoto’s thyroiditis, is found more commonly in women. Clinical presentations of HE vary significantly, with cognitive deficit and generalized seizures as the most prominent manifestations. While there is robust literature illustrating HE in female populations, literature on HE in male patients, particularly with dominant motoric features, remains limited. Methods: A 52-year-old, right-handed, Caucasian male presented for evaluation reporting difficulties with short-term memory, word-finding, disorientation, and motor deficits. The patient exhibited significant motor symptoms including myotonia, micrographia, fasciculations and paresthesias, phantosmia, and myoclonic jerking, beginning in 2017 with progressive decline. Relevant medical history includes Hashimoto thyroiditis (2021), focal temporal EEG abnormalities (2021), dystonia (2021), and migraines. Prior to presenting concerns, the patient was otherwise generally healthy. Neuroimaging (MRI) revealed increased signal intensity in the subcortical white matter of the right frontal lobe. He was dependent in several instrumental activities of daily living. Results: His premorbid baseline was estimated to be in the average range. His neurocognitive profile was marked by mild impairments in immediate and delayed verbal memory and recognition paradigms, whereas he demonstrated moderate impairments in processing speed and motor functioning. Conculsion: Taken together, his neurocognitive profile, clinical history, and functional independence are consistent with the clinical presentation of HE. His cognitive deficits and epileptiform activity are consistent with prominent manifestations of HE, whereas his pronounced motoric features are seemingly aberrant in HE. Furthermore, post-steroidal treatment revealed increased cognitive proficiency on a single mental status examination.

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