H-Index
79
Scimago Lab
powered by Scopus
JCR
Clarivate
Analytics
15%
Acceptance
Rate
call: +1.631.470.9640
Mon-Fri 10 am - 2 pm EST

Logo



eISSN: 1643-3750

Get your full text copy in PDF

Atypical features of dementia in a patient with Creutzfeldt-Jakob disease

Maria Pąchalska, Henryk Kurzbauer, Maria Formińska-Kapuścik, Andrzej Urbanik, Grażyna Bierzyńska-Macyszyn, Paweł Właszczuk

Med Sci Monit 2007; 13(1): CS9-19

ID: 470152


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.

This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
I agree