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Título

Creutzfeld Jakob disease and COVID 19: a case report

RESUMO

CASE REPORT: We describe a case of a male patient, 48-years-old, whose started 20 days after the diagnosis of coronavirus disease 2019 (COVID 19) with progressive fatigue, binocular diplopia, headache and slowing of thought. After a few weeks, he began to experience difficulties in walking, with ataxia and incoordination. The symptoms has a dramatic evolution over three months with onset of diffuse myoclonus, significant language and movement impairment and finally evolved to akinetic mutism. Complementary investigation whit brain magnetic resonance imaging (MRI) showed restricted diffusion in the cortical and subcortical posterior regions and deep nuclei gray matter (left caudade nuclei). The electroencephalogram (EEG) revealed intermittent generalized periodic discharges (GPD) and diffuse-symmetric slowing. The cerebrospinal fluid (CSF) analysis and the 14-3-3 research were negative. In view of the findings, we agree with the Crutzfeld-Jacob disease hypothesis, supported by University of Edinburgh diagnostic criteria (2017) to probable sporadic Jakob-Creutzfeldt disease. Patient complicated with bronchoaspiration pneumonia with needing of mechanical ventilation. Nowadays he persist in akinetic mutism with tracheostomy and gastrostomy.
DISCUSSION: Prion diseases are a heterogeneous group of diseases caused by prions –abnormally shaped proteins, and it can be acquired, hereditary or sporadic form. CJD is a more common form of prion disease that affects humans and courses with rapidly progressive dementia associated with behavioral changes, ataxia, extrapyramidal features and myoclonus. Its diagnosis is based on the clinical history and interpretation of the clinical features, CSF, EEG and MRI, in addition to the genetic tests supported by polymerase chain reaction (PCR-RT). There are some reports of patients who developed CJD with a temporal relationship to COVID 19 that were initially interpreted as a coincidence because the rare incidence of CJD and the COVID 19 pandemic that we have faced in recent years. Although, there are descriptions of simultaneous clinical presentations of COVID-19 and CJD, that can indicate some correlated mechanism between them.
FINAL COMMENTS: There is still no definitive explanation for the relationship between COVID 19 and CJD, but it is questioned whether the inflammatory cascade caused by the coronavirus infection could accelerate or even precipitate the neurodegeneration characteristic of CJD.

Palavras Chave

PRION DISEASE; CREUTZFELD JAKOB DISEASE; COVID-19