Moléstia de Creutzfeldt-Jakob: estudo clínico, histopatológico e eletromicroscópico de um caso

A case of Creutzfeldt-Jakob disease (CJD) in a 76 year-old man is presented. The clinical picture included a rapid progressive dementia associated with ataxia, global aphasia, myoclonus and pyramidal signs; death occurred after about 4 months. There was an antecedent of chemical trauma caused by plant liquid on right eye 12 to 18 months before. The electroencephalogram showed diffuse slow activity and the neuropathological findings were typical. The detection of a protein called "prion" or PrP27-30 in the scrapie and the finding that some proteins isolated from brain of patients with CJD have reacted with antibodies raised against it have improved the knowledge about the infectious agent. The recent reports of young patients with CJD after human growth hormone therapy prepared from pools of pituitary glands obtained at autopsy are alarming and probably new cases will be described.

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Doença de Creutzfeldt-Jakob: registro de caso

Kouyoumdjian

Meneghette

Tognola

et al. 1987

Arq. Neuro-Psiquiatr.

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Creutzfeldt-Jakob disease in Venezuela a case report

Caraballo¹

1991

Arq. Neuro-Psiquiatr.

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SUMMARY -A case of Creutzfeldt-Jakob disease (CJD) in a 32 year old man is presented. The clinical picture included a rapid progressive dementia associated with ataxia, global aphasia, myoclonus and pyramidal signs, death ocurred after 13 months. The diagnosis of CJD was confirmed by CT and neuropathological studies. This is the first report of CJD occurring in Venezuela. Doença deCreutzfeldt-Jakob na Venezuela: registro de caso.RESUMO -Registro de caso de doença de Creutzfeldt-Jakob (DCJ) em um paciente com 32 anos de idade. O quadro clínico abrangia demência rapidamente progressiva associada a ataxia, afasia global, miocloniais e sinais piramidais. A morte ocorreu após 13 meses. O diagnóstico foi confirmado por dados dia TC e do estudo neurcpatológico. Este é o primeiro relato de DCJ na Venezuela.Creutzfeld-Jakob disease (CJD), a rare disorder, is attributed to a slow virus infection. Clinico-pathological aspects of the first case of CJD observed in Venezuela aie reported in this paper. CASE REPORTNAM, a 32-year-old heavy machinery operator, according to references from relatives, presents since 2 months ago a frame characterized by: gradual loss of recent memory, absent-mindedness concentration, irritability and incompetence to carry out everyday actions. One month later, he is admitted into Caracas University Hospital where a cardiopulmonary evaluation and laboratory routine were performed, with results within normal values. A CT scan of the brain was performed showing a moderate increase of the silvian fissures (Fig. 1*). He is discharged against medical opinion. Two months later, he starts to show changes in his behavior, developing paranoid and delirious ideas, with visual and auditory hallucinations, lacking introspection. As the disease progresses, he loses all his faculties, not being able to recognize the members of his family. He presents dysphagia, aphasia and incontinence of urine, being admitted at the Maracaibo Psychiatric Hospital where he stayed for 15 days showing no general changes. Seven months after onset of the disease he presents progressive and insidious ataxic march, spasticity and generalized myoclonia. His physical condition deteriorates gradually until he loses his capacity to move by himself staying permanently in bed.Eleven months after onset of the disease, he is sent to the Southern General Hospital in Maracaibo. He was a non-drinker and non-smoker. There was no history of tuberculosis, hypertension, diabetes mellitus and no positive family-history of any neurological disease.

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Creutzfeldt-Jakob disease a case report, with special attention to the electroencephalogram in this disorder and to its possible relationships to kuru, scrapie and «mad cow disease»

Chapman¹,

Silva²

1993

Arq. Neuro-Psiquiatr.

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A case of Creutzfeldt-Jakob disease in a 58-year-old Brazilian cattle rancher and businessman is presented. The EEG was normal, which is consistent with the fact that it was made during the first half of his illness; in a later stage suppression of normal rhythms by slow moderate voltage waves would be expected. The resemblances of kuru, scrapie and "mad cow disease" to C-J disease are discussed. In each of these 4 illnesses the patient or affected animal (scrapie and "mad cow disease") (a) has a widespread spongiform encephalopathy and consequent dementia, myoclonic epilepsy and cerebellar and corticospinal symptoms. (b) Each illness is caused by a virus (or virus-like organism called a PrP or prion) which is unusually resistant to heat and entirely resistant to ultraviolet light and x-rays. (c) This causative agent can be transmitted to other mammals by intracerebral injection or, in the proved cases of 3 of them, by the oral route. Unresolved questions about C-J disease include the following: Are C-J disease, kuru, scrapie and "mad cow disease" essentially similar illnesses caused by the same virus or by subtle variants of it? What is the incubation period of C-J disease, and does its virus exist for long periods of time in some asymptomatic persons, some of whom may never become neurologically ill? How does this virus enter the bodies of most persons with C-J disease, and why does the clinical disease characteristically occur only in middle age?

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Moléstia de Creutzfeldt-Jakob: estudo clínico, histopatológico e eletromicroscópico de um caso

Cited by 3 publications

References 7 publications

Doença de Creutzfeldt-Jakob: registro de caso

Doença de Creutzfeldt-Jakob: registro de caso

Creutzfeldt-Jakob disease in Venezuela a case report

Creutzfeldt-Jakob disease a case report, with special attention to the electroencephalogram in this disorder and to its possible relationships to kuru, scrapie and «mad cow disease»

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