Resumo

REYES H, Valentina; MUNOZ N, Teresa  e  HIDALGO A, Leonel. Creutzfeldt- Jakob Disease: Report Of One Case. SCIENTIFICA [online]. 2014, vol.12, n.1, pp. 125-128. ISSN 1813-0054.

ABSTRACT Introduction: Creutzfeldt-Jakob disease belongs to a group of diseases known as transmissible spongiform encephalopathies or prion diseases. Classified in sporadic, familial and iatrogenic, manifested by subacute dementing box, motor symptoms, visual, and myoclonus Case Presentation: male, 42 years without morbid history, presents picture of two months' duration characterized by dizziness, imbalance and difficulty in underway. In extra-sistema neurologist asked which brain MRI showed hyperintense lesions in both striated mainly cortical hyperintense to right and right frontal and occipital bilaterally. Physical Exam General: No changes. Neurological examina-tion: Vigil, aware, attentive, temporomandibu-lar spatially oriented, good-humored; long memory and short-term preserved; higher brain functions preserved; examination of cranial nerves, greater left diplopia without other alteration; Consideration motor normal tone, preserved trofismo, strength preserved; ataxic gait, dysmetria left; no sensory disturb-ances. Family Background: brother died from similar symptoms. Given the Creutzfeldt-Jakob disease clinic suspect a nonspecific metabolic or toxic encephalopathy, requested an Electro-encephalogram and 14-3-3 lumbar puncture awaiting results. Currently with Chlorproma-zine 25 mg c / vo night and Paracetamol 500 mg po SOS. Discussion: The diagnosis of Creutzfeldt-Jakob disease is suspected with the clinic, and is based with characteristic MRI findings, EEG and CSF analysis. Suspecting the familiar form genetic study suggests. As an invariably fatal disease without treatment, difficult decisión between studies and interven-tions with expectant management

Palavras-chave : Creutzfeldt-Jakob disease; transmissible spongiform encephalopathies; prion diseases.