Edorium Journal of

Neurology

 
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Case Report
 
Cognitive event-related potentials in patient with hereditary hemochromatosis: A case report
Nataša Klepac1, Magdalena Krbot Skorić1, Natalia Palac1, Petra Nimac Kozina1, Ivan Adamec1
1Department of Neurology, University Hospital Center Zagreb, Kišpaticeva, Zagreb, Croatia.

Article ID: 100003N06NK2015
doi:10.5348/n06-2015-3-CR-2

Address correspondence to:
Nataša Klepac
Department of Neurology, University Hospital Center Zagreb
Kišpaticeva 12, Zagreb
Croatia

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How to cite this article
Klepac N, Skorić MK, Palac N, Kozina PN, Adamec I. Cognitive event-related potentials in patient with hereditary hemochromatosis: A case report. Edorium J Neurol 2015;2:5–10.


Abstract
Introduction: Hereditary hemochromatosis is an autosomal recessive disorder, characterized by iron overload and accumulation in liver, pancreas, heart and brain with secondary tissue damage. Mutations in the hemochromatosis gene are commonly associated with hereditary hemochromatosis and consequently iron overload. The 2 most common hemochromatosis gene variants are C282Y and H63D. Hemochromatosis gene mutations are being investigated as genetic risks for neurodegenerative disorders since iron accumulation in the brain are consistent observations in many neurodegenerative diseases. Multiple studies discussed the association between hereditary hemochromatosis and Alzheimer's disease. Although cognitive impairment is well known feature of hereditary hemochromatosis the exact cause is unknown. Cognitive event-related potentials are method for testing higher cognitive functions. It allows completely non-invasive insight into cognitive processes, in particular through the display of the timeliness of the cognitive process, because the method has a very high temporal resolution. The changes in cognitive event-related potentials are characteristics for Alzheimer's disease.
Case Report: We present for the first time results of cognitive event-related potentials in case of a patient with hereditary hemochromatosis. Patient is homozygous for the C282Y variant of hemochromatosis protein mutation gene and has positive family history for dementia. Cognitive event-related potentials showed reduced amplitude and prolonged latency of response which was similar to changes observed in Alzheimer's disease patients.
Conclusion: Neuro-electrophysiological changes observed in our patient with hereditary hemochromatosis and cognitive impairment were similar to that observed Alzheimer's disease patients. Based on our findings hereditary hemochromatosis and Alzheimer's disease share the same neurophysiological characteristics indicating that impairment of iron homeostasis may be underlining cognitive impairment in both disorders.

Keywords: Alzheimer's disease, Cognitive event-related potentials, Hereditary hemochromatosis, Hemochromatosis gene


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Author Contributions
Nataša Klepac – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Magdalena Krbot Skorić – Acquisition of data, Drafting the article, Final approval of the version to be published
Natalia Palac – Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published
Petra Nimac Kozina – Acquisition of data, Drafting the article, Final approval of the version to be published
Ivan Adamec – Substantial contributions to conception and design, Drafting the article, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2015 Nataša Klepac et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



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