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Título

Report of three cases of patients with probable Creutzfeldt-Jakob disease in a period of six months in the same region of Brazil

RESUMO

Case Presentation: A 54-year-old woman presented with psychiatric disorder with persecutory delusions, evolving with focal epileptic seizures for 20 days. On admission, myoclonus was evident in the upper and lower limbs and spasticity in the lower limbs. LCR cultures were negative, VDRL unreactive, oligogloconal bands absent and 14-3-3 protein search present. EEG unchanged. MRI of the skull, signs of restriction and sparse cortical diffusion in the frontal and parietal lobes, and in the striatum on the right. A 57-year-old man presented with progressive cognitive impairment associated with depression, two months ago. On admission, hypovigil, temporo-spatial disorientation, psychomotor retardation, impaired short-term memory, myoclonus in upper and lower limbs and face, spasticity of the limbs. CSF negative for encephalitis. EEG with diffuse slowing of background activity. Cranial MRI with bilateral striated cortex and bilateral lentiform nucleus. A 68-year-old man presented with episodes of spatial disorientation for a month and a half, evolving with mental confusion associated with visual hallucinations, being admitted to psychiatry with a hypothesis of delirium. Assessed by Neurology due to spontaneous myoclonus in the face and upper limbs. The EEG marked disorganized brain electrical activity for age with generalized periodic discharges. CSF with 14-3-3 protein search present. MRI of the skull signs of diffusion restriction involving the caudate and putamen bilaterally and symmetrically. The three cases evolved in less than 30 days of hospitalization with akinetic mutism, and death from bronchoaspiration pneumonia. Discussion: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disease, whose diagnosis is based on clinical, radiological, electroencephalographic or cerebrospinal fluid findings, which makes its diagnosis a challenge due to the availability of exams and the low rate of clinical suspicion. Final comments: Despite the rarity, the three cases were identified with a hypothesis of probable CJD, in a period of only six months in the same region of Brazil, with no relationship of kinship and no report of possible acquisition of an iatrogenic form.

Palavras Chave

myoclonus; Creutzfelt-Jakob disease; , rapidly progressive neurodegenerative disease;