Neuropsychological features of rapidly progressive dementia in a patient with an atypical presentation of Creutzfeldt-Jakob DiseaseMaria Pąchalska, Henryk Kurzbauer, Bruce D. MacQueen, Maria Formińska-Kapuścik, Irena Herman-Sucharska
Med Sci Monit 2001; 7(6): CS1307-1315 :: ID: 508365
Background. Creutzfeldt-Jakob Disease (CJD) is a degenerative disease of the brain, characterized by rapid and irreversible decline, with dementia, ataxia, myoclonus, and other neurological and neurobehavioral disorders associated with rapidly progressive spongiform encephalopathy. The mode of transmission and basic pathomechanism remain unclear. The clinical picture of CJD is highly diverse, producing a number of variants.
Material and methods. The patient to be described is a 68-year-old Polish female, JR, clinically diagnosed with CJD. The article presents the case history in detail, with particular emphasis on neuropsychological testing, which was initiated when the patient was still lucid and capable of cooperation. The first presenting symptom was agraphia, followed by hemianopsia and other vision disorders, culminating in visual hallucinations. As the progress of the disease accelerated there was rapidly progressive dementia, aphasia developing to organic mutism, myoclonus, hyperkinesia, ultimately loss of all verbal contact or voluntary movement.
Results. JR’s neuropsychological parameters declined in a period of less than 3 months from near normal to levels characteristic of severe dementia.
Conclusions. The clinical picture here presented is consistent with that of the Heidenhain variant of CJD, with spongiform encephalopathy beginning in the right occipital lobe. Several features of the case remain atypical, however, including the absence of the most common genetic mutation and the patient’s long survival after onset.
Keywords: spongiform encephalopathy, Creutzfeldt-Jakob Disease - Heidenhain variant, Dementia, Hallucinations, Agraphia
01 May 2023 : EditorialEditorial: Twenty Years On from Sequencing the Human Genome, Personalized/Precision Oncology Prepares to Meet the Challenges of Checkpoint Inhibitor Therapy
Med Sci Monit 2023; 29:e940911
28 May 2023 : Database AnalysisScientometric and Visualized Analysis of Intravoxel Incoherent Motion Magnetic Resonance Imaging: 1988-2021
Med Sci Monit In Press; DOI: 10.12659/MSM.938715
25 May 2023 : Database AnalysisThe COVID-19 Crisis and the Incidence of Alcohol-Related Deaths in Poland
Med Sci Monit In Press; DOI: 10.12659/MSM.940904
25 May 2023 : Review articleA Review of Preclinical and Clinical Studies in Support of the Role of Non-Steroidal Anti-Inflammatory Drug...
Med Sci Monit In Press; DOI: 10.12659/MSM.940635
25 May 2023 : Clinical ResearchPeriapical Lesions and Missed Canals in Endodontically Treated Teeth: A Cone-Beam Computed Tomographic Stud...
Med Sci Monit In Press; DOI: 10.12659/MSM.940533
Most Viewed Current Articles
13 Nov 2021 : Clinical ResearchAcceptance of COVID-19 Vaccination and Its Associated Factors Among Cancer Patients Attending the Oncology ...
Med Sci Monit 2021; 27:e932788
30 Dec 2021 : Clinical ResearchRetrospective Study of Outcomes and Hospitalization Rates of Patients in Italy with a Confirmed Diagnosis o...
Med Sci Monit 2021; 27:e935379
08 Mar 2022 : Review articleA Review of the Potential Roles of Antioxidant and Anti-Inflammatory Pharmacological Approaches for the Man...
Med Sci Monit 2022; 28:e936292
01 Jan 2022 : EditorialEditorial: Current Status of Oral Antiviral Drug Treatments for SARS-CoV-2 Infection in Non-Hospitalized Pa...
Med Sci Monit 2022; 28:e935952