Introduction:

Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder caused by prions in humans. It presents as a spongiform encephalopathy of worldwide distribution with a prevalence of 1 in 1,000,000 inhabitants.

Objective:

to characterize Creutzfeldt-Jakob disease at the National Institute of Neurology and Neurosurgery during the period from February 1981 to January 2019.

Methods:

an observational, descriptive cross-sectional study was carried out on patients diagnosed with Creutzfeldt-Jakob disease, of non-hereditary etiology, at the National Institute of Neurology and Neurosurgery of Cuba between 1981 and 2019. The universe was constituted by the 12 patients diagnosed in that period.

Results:

female sex predominated in 66,3 %. The mean hospital stay was 9,8 days with a minimum of 7 and a maximum of 46 days. The first symptom identified by the patient was difficulty walking (50 %), followed by loss of balance (33 %) and dizziness (17 %). The predominant radiological finding was frontoparietotemporal and cerebellar atrophy in 50 % of patients. The most frequent clinical manifestations were aphasia in 25 %, disorientation, dysarthria, dysphagia and urination disorders in the same proportion.

Conclusions:

In the investigation a greater number of cases were observed in the female sex. Difficulty walking followed by loss of balance and dizziness were the first symptoms identified. Frontoparietotemporal and cerebellar atrophy predominated as radiological findings. The most frequent clinical manifestations were aphasia, disorientation, dysarthria, dysphagia and urination disorders.

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