To evaluate the frequency and type of movement disorders (MD) in patients with CJD from at tertiary hospital in Peru.

Creutzfeldt-Jakob disease (CJD) is a rare, fatal, progressive neurodegenerative disease. It is characterized by cognitive impairment of subacute onset, rapid evolution, associated with behavioral symptoms, pyramidal signs, ataxia and movement disorders.

A review of cases of inpatients with a diagnosis of CJD at the Instituto Nacional de Ciencias Neurológicas from 2017-2022 was carried out. Fifteen patients were evaluated. Nine female and 6 males.  The average age at the onset of the disease was 64.2 ± 7.59 years. The average time of illness until discharge or death was 5.9 ± 3.11 months. Ten patients (66.6%) had electroencephalogram with the presence of typical waves, 9/1 (60%) patients had positive 14-3-3 protein while 6/15 could not perform it, 13/15 (86.6%) patients had MRI with characteristic findings of CJD, only 2 did not have this study. The most frequent initial symptoms were cognitive impairment (60%) followed by movement disorders (53.33%), with ataxia being the most common; The movement disorders present in the patients were myoclonus (93.33%), ataxia (80%), parkinsonism (66.67%), dystonia (53.33%), alien limb phenomenon (33.33%), tremor (26.67%), fasciculations (6.67%) and gaze paralysis (6.67%), no patient had chorea. The sporadic CJD was the most frequent form. Myoclonus was noted in 14/15 patientes throughout the disease course also parkinsonism in 10/15 (66.67%). With 6.67% of cases were recorded as having an ataxic gait. In addition, most of the patients showed more than one movement throughout the disease course.

Movement disorders are very common in CJD, with more than one type occurring during the course of the disease with, the myoclonus and ataxia as the most common.