22 December 2006
Atypical features of dementia in a patient with Creutzfeldt-Jakob disease
Maria Pąchalska, Henryk Kurzbauer, Maria Formińska-Kapuścik, Andrzej Urbanik, Grażyna Bierzyńska-Macyszyn, Paweł WłaszczukMed Sci Monit 2007; 13(1): CS9-19 :: ID: 470152
Abstract
Background: This article describes a Polish patient (female, right-handed, age 68 at onset) diagnosed with the Heidenhain variant of Creutzfeldt-Jakob Disease (HvCJD), characterized clinically by isolated visual
disturbances with no ocular dysfunction prior to the development of myoclonus and other symptoms of CJD.
Case Report: Nothing in the history pointed to iatrogenic or acquired CJD, and genetic testing ruled out familial CJD. The neuroradiological picture (MRI) showed non-specifi c features of cerebral atrophy (cortical and subcortical). An EEG revealed periodic triphasic sharp waves, particularly in the occipital
lobes, and myoclonus occurring synchronically with generalized periodic epileptiform discharges. Comprehensive neuropsychological testing documented rapidly progressive dementia, with dysgraphia and aphasia deteriorating to organic mutism. Post-mortem neuropathological examination
confi rmed spongiform encephalopathy, especially in occipital cortex, with amyloid plaques but without neurofi brillary tangles.
Conclusions: Over the crucial 6-week period the patient went from “Mild Cognitive Impairment” to a status resembling the fi nal stages of Alzheimer’s disease, without any evidence of a CVA. The only aspect of this case that does not fi t the usual criteria for the Heidenhain variant is the fact that the patient survived over a year in a persistent vegetative state. Ophthalmologists and family physicians should be aware of the possibility of HvCJD in any patient over 60 presenting with otherwise inexplicable visual disturbances in the absence of signifi cant ocular pathology, even when other symptoms
of dementia may not be immediately noticeable.
Keywords: Brain - radiography, Creutzfeldt-Jakob Syndrome - complications, Dementia - pathology, Diagnostic Techniques, Ophthalmological, Fatal Outcome, Hemianopsia - diagnosis, Magnetic Resonance Imaging, Neuropsychological Tests
517 4
Editorial
01 May 2023 : Editorial
Editorial: Twenty Years On from Sequencing the Human Genome, Personalized/Precision Oncology Prepares to Meet the Challenges of Checkpoint Inhibitor TherapyDOI: 10.12659/MSM.940911
Med Sci Monit 2023; 29:e940911
In Press
31 May 2023 : Review article
Prevalence, Diagnosis, and Management of Eclampsia and the Need for Improved Maternal Care: A ReviewMed Sci Monit In Press; DOI: 10.12659/MSM.939919
30 May 2023 : Clinical Research
A Case-Control Study of Cognitive Function in Patients with End-Stage Renal Disease Before and After Hemodi...Med Sci Monit In Press; DOI: 10.12659/MSM.940409
30 May 2023 : Review article
A Review of the Role of the Zebrafish (Danio reiro) in Preclinical and Clinical Models of Biomarker Identif...Med Sci Monit In Press; DOI: 10.12659/MSM.940550
30 May 2023 : Database Analysis
The COVID-19 Crisis and the Incidence of Alcohol-Related Deaths in PolandMed Sci Monit In Press; DOI: 10.12659/MSM.940904
Most Viewed Current Articles
13 Nov 2021 : Clinical Research
Acceptance of COVID-19 Vaccination and Its Associated Factors Among Cancer Patients Attending the Oncology ...DOI :10.12659/MSM.932788
Med Sci Monit 2021; 27:e932788
30 Dec 2021 : Clinical Research
Retrospective Study of Outcomes and Hospitalization Rates of Patients in Italy with a Confirmed Diagnosis o...DOI :10.12659/MSM.935379
Med Sci Monit 2021; 27:e935379
08 Mar 2022 : Review article
A Review of the Potential Roles of Antioxidant and Anti-Inflammatory Pharmacological Approaches for the Man...DOI :10.12659/MSM.936292
Med Sci Monit 2022; 28:e936292
01 Jan 2022 : Editorial
Editorial: Current Status of Oral Antiviral Drug Treatments for SARS-CoV-2 Infection in Non-Hospitalized Pa...DOI :10.12659/MSM.935952
Med Sci Monit 2022; 28:e935952