A case report of sporadic Creutzfeldt-Jakob disease in an Asian origin coronavirus disease-19 patient: An enigma

Abstract

reutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder affecting about one in every 1 million persons/year worldwide [1]. It is the prototype of a family of rare and fatal human degenerative conditions characterized by progressive brain dysfunction. CJD falls into four categories: sporadic, familial, iatrogenic, and variant. Sporadic CJD (sCJD), thought to occur worldwide, is the most common form and represents about 85% of all CJD cases; the incidence ranges between 0.5 and 1.5 cases per million inhabitants per year [2]. An early diagnosis is important for counseling the patient and their family members, as also for optimal management of symptoms and to avoid any iatrogenic transmission. However, any delay in diagnosing the disease is due to a combination of its rarity as well as heterogeneity in the early stages of the disease [3].We report our coronavirus disease (COVID-19) intensive care unit experience in a patient of sCJD who presented with respiratory symptoms and seizures, which were different from the typical neuropsychiatric presentation of sCJD. Hence, we describe the challenges faced in diagnosis, management, and precautionary measurements taken for such a patient, whose condition got aggravated and worsened by the concurrent COVID-19. CASE REPORTA 61-year-old female presented with symptoms of heaviness over the base of the head which progressively worsened over 2 months, along with difficulty in walking, "tremulousness" and low-grade fever on and off. Gradually, she developed a few episodes of confusion with irrelevant and abnormal behavior which worsened over a week along with visual hallucinations.The clinical presentation of the patient suggested some neurodegenerative disease for which, she was referred to the Institute of Human Behavior and Allied Sciences, Delhi, India, where the diagnosis of CJD was made based on the clinical symptoms, typical electroencephalograph (EEG), and magnetic resonance imaging (MRI).The patient was admitted for the above complaints, where she was clinically suspected to be a case of autoimmune encephalitis. Contrast-enhanced MRI was done which revealed empty "sella turcica," and she was treated with methylprednisolone and intravenous immune globulin. The patient's condition showed little improvement for a while and later started to develop

Creutzfeldt–Jakob disease in a post-COVID-19 patient: did SARS-CoV-2 accelerate the neurodegeneration?

“…Two case reports recently concluded that there is a sudden surge in the systemic inflammatory response due to COVID-19 and concluded the same hypothesis of accelerated progression of sCJD cases [ 23 – 25 ].…”