Creutzfeldt-Jakob Disease and Hashimotos Thyroiditis: A Case Report Illustrating Prion-Induced Encephalitis

Abstract

Sporadic creutzfelt-Jakob disease (sCJD) is a rare neurodegenerative disorder and the most common type of the prion diseases that affects humans. Its clinical manifestations are progressive dementia, myoclonus, visual or cerebellar disturbances, pyramidal dysfunction, and akinetic mutism. Laboratory findings include high levels of the 14-3-3 protein in cerebrospinal fluid and Electroencephalogram with periodic spikes at ≈ 1 Hz. We present a 51- year-old Hispanic woman with Hashimoto’s thyroiditis and a 1-year history of depression who developed rapidly progressive dementia. She required extensive diagnostic examinations and eventually was diagnosed with sCJD based on the World Health Organization diagnostic criteria. Real-time quaking-induced conversion (RT-QuIC) was positive, and thyroid peroxidase antibodies (TPO-AB) (2391 IU/ml) and 14-3-3 protein (3.8 ng/ml) were elevated. Her key findings included rapidly progressive dementia, ataxia, akinetic mutism, myoclonus, rigidity, visual disturbance, EEG (Electroencephalogram) with periodic sharp wave complexes at 1 Hz, and normal head CT scans. She progressively worsened and died 3 months after the onset. In sum, patients with CJD and Hashimoto’s thyroiditis should be (i) evaluated for autoimmune encephalitis, (ii) screened for a rise in TPO antibodies, and (iii) steroids administration should be considered as adjuvant therapy. This case illustrates a prion disease mediated immune system modulation which we believe is a potential underlying mechanism by which the 14-3-3 protein induces a steroid non-responsive autoimmune encephalitis.