Journal of Neurology Research, Vol. 1, No. 3, Aug 2011
Vol. 1, No. 3, Aug 2011
Journal of Neurology Research, ISSN 1923-2845 print, 1923-2853 online
Article copyright, the authors; Journal compilation copyright, J Neurol Res and Elmer PressTM
Journal website
http://www.neurores.org
Case Report
Creutzfeldt-Jakob Disease: An Atypical Case With Acute Onset and Quick Progression
Adriana Moroa, b, Alisson Pittol Bresciania, Marcus Victor de Oliveiraa, Pedro Henrique de Campos Albinoa, Melina More Bertottia, Rafael Martins Ferreiraa, Luiz Paulo Queiroza, Paulo Mattosinho Filhoa
aNeurology Department, Hospital Governador Celso Ramos, Florianopolis, Santa Catarina, Brazil
bCorresponding author: Adriana Moro, Prefeito Antenor Mesquita 98/101, Centro Florianopolis SC, CEP: 88015 - 150.
Manuscript accepted for publication August 8, 2011
Short title: Creutzfeldt-Jakob Disease
doi:10.4021/jnr33w
Abstract
Creutzfeldt-Jakob disease (CJD) is a prionic neurodegenerative disease, which leads to patients death about 12 months after the onset of symptoms.We report a case of CJD with atypical evolution and unusual findings in MRI.
Introduction
Creutzfeldt-Jakob disease (CJD) is a prionic neurodegenerative disease, which leads to patients death about 12 months after the onset of symptoms [1, 2]. Patients develop progressive dementia with beginning in the 4th - 5th decade of life, associated with myoclonic jerks, cerebellar syndrome, visual deficits, pyramidal dysfunction or extra-pyramidal acinetic mutism [1]. Additional tests that may aid in diagnosis are brain magnetic resonance imaging (MRI) [3], electroencephalogram (EEG) [4] and protein 14 - 3 - 3 in the cerebrospinal fluid (CSF) [3].
We report a case of CJD with atypical evolution and unusual findings in MRI.
At day 21 patient developed akinetic mutism. Progressive worsening of right hemiparesis and myoclonus were observed.
The patient died at day 68 owing to acute respiratory failure...
