alexa Brainstem Sparing in Human Prion Disease: Sleep and Autonomic Function in a Long Survival Case Report | OMICS International | Abstract
ISSN: 2167-0277

Journal of Sleep Disorders & Therapy
Open Access

OMICS International organises 3000+ Global Conferenceseries Events every year across USA, Europe & Asia with support from 1000 more scientific Societies and Publishes 700+ Open Access Journals which contains over 50000 eminent personalities, reputed scientists as editorial board members.

Open Access Journals gaining more Readers and Citations
700 Journals and 15,000,000 Readers Each Journal is getting 25,000+ Readers

This Readership is 10 times more when compared to other Subscription Journals (Source: Google Analytics)

Case Report

Brainstem Sparing in Human Prion Disease: Sleep and Autonomic Function in a Long Survival Case Report

Alessandro Pincherle1,2*, Eleonora Tobaldini3, Lucio Mos3, Ambra Dominese2, Vincenzo Patruno4, Nicola Montano3, Flavio Villani2, Fabrizio Tagliavini2, Giorgio Giaccone2 and Gabriella Marcon2-6
1Neurology Unit, Spitalzentrum Biel, Biel, Switzerland
2Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milan, Italy
3Department of Biomedical and Clinical Sciences “L. Sacco”, Medicine and Physiopathology Unit, L. Sacco Hospital, University of Milan, Italy
4Department of Cardiology, San Daniele del Friuli Hospital, Udine, Italy
5Pneumologia Riabilitativa, Istituto di Medicina Fisica e Riabilitazione “Gervasutta”, Udine, Italy
6Department of Biological and Medical Sciences (DSMB), University of Udine, Italy
*Corresponding Author : Alessandro Pincherle
Neurology Unit
Spitalzentrum Biel Biel, Switzerland
Tel: 0041323444763
E-mail: [email protected]
Received: February 05, 2016 Accepted: February 18, 2016 Published: February 26, 2016
Citation: Pincherle A, Tobaldini E, Mos L, Dominese A, Patruno V, et al. (2016) Brainstem Sparing in Human Prion Disease: Sleep and Autonomic Function in a Long Survival Case Report. J Sleep Disord Ther 5:236. doi:10.4172/2167-0277.1000236
Copyright: © 2016 Pincherle A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Background: The prion diseases are characterized by sleep disruption, with FFI typically characterized also by severe autonomic dysfunction and sympathetic hyperactivity. We report the results of an extensive neurophysiological and autonomic assessment in a CJD patient carrying the D178 mutation with the uncommon homozygosity for valine at codon 129, mutation with long disease duration.

Results: A 47years old female presented with a memory impairment followed by progressive cognitive deficits and ataxia. The clinical picture slowly worsened to a state of akinetic mutism in about 2 years, and the patient died six years after the onset of symptoms. Repeated PSG and long-term actigraphic recordings, showed a peculiar, previously undescribed, pattern characterized by conservation of a rudimental circadian and ultradian rhythm, despite dramatic sleep micro-structure deterioration. We also observed a normal autonomic physiological response to orthostatic challenge and normal dynamic autonomic modulation during wake and sleep. The post-mortem brain pathology study, showed that neuronal loss was substantial in the cerebral cortex, diencephalon and thalami, but not in brainstem nuclei.

Conclusions: We hypothesize that, despite a dramatic neurological picture (i.e. akinetic mutism) and a severe sleep micro-structural alteration, the persistence of an autonomic modulation and the persistence of a rudimental circadian and ultradian oscillation, are related to the relatively conserved anatomo-functional integrity of foundamental neuronal systems in the brainstem.

Keywords

Peer Reviewed Journals
 
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2018-19
 
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri & Aquaculture Journals

Dr. Krish

[email protected]

1-702-714-7001Extn: 9040

Biochemistry Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Business & Management Journals

Ronald

[email protected]

1-702-714-7001Extn: 9042

Chemistry Journals

Gabriel Shaw

[email protected]

1-702-714-7001Extn: 9040

Clinical Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Engineering Journals

James Franklin

[email protected]

1-702-714-7001Extn: 9042

Food & Nutrition Journals

Katie Wilson

[email protected]

1-702-714-7001Extn: 9042

General Science

Andrea Jason

[email protected]

1-702-714-7001Extn: 9043

Genetics & Molecular Biology Journals

Anna Melissa

[email protected]

1-702-714-7001Extn: 9006

Immunology & Microbiology Journals

David Gorantl

[email protected]

1-702-714-7001Extn: 9014

Materials Science Journals

Rachle Green

[email protected]

1-702-714-7001Extn: 9039

Nursing & Health Care Journals

Stephanie Skinner

[email protected]

1-702-714-7001Extn: 9039

Medical Journals

Nimmi Anna

[email protected]

1-702-714-7001Extn: 9038

Neuroscience & Psychology Journals

Nathan T

[email protected]

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

Ann Jose

[email protected]

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

[email protected]

1-702-714-7001Extn: 9042

 
© 2008- 2018 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version
Leave Your Message 24x7