functional neurology, rehabilitation, and ergonomics

FUNCTIONAL NEUROLOGY, REHABILITATION, AND ERGONOMICS Volume 3, Number 4, 2013 TABLE OF CONTENTS Editorial Collegiality Affects Output Gerry Leisman

413

Clinical Papers Unusual Brainstem Twisting Revealed by MRI Tractography in a Patient Who Survived a Severe Traumatic Upper Spinal Dislocation Calixto Machado, Jesús Pérez-Nellar, Rafael Rodríguez, Mario Estévez, Phillip A. DeFina, Adam Schiavi, Gerry Leisman, Frederick R. Carrick, Robert Melillo, and Mauricio Chinchilla The Treatment of Persistent Imbalance in a Patient with Traumatic Brain Injury Using a Functional Neurological Approach Susan E. Esposito, Linda E. Mullin, and Frederick R. Carrick

421

423

Unusual Presentation of Gluten Sensitivity in a Child: Symmetrical Distal Myopathy David B. Sullivan

431

Ear Insufflation Produces Rapid and Significant Relief of Trigeminal Neuralgia David B. Sullivan

439

Scientific Paper Megacity Air Pollution and the Impact on the Central Nervous System Lilian Calderón-Garcidueñas, Ana Calderón-Garcidueñas, and Ricardo Torres-Jardón

447

IAFNR News and Events

457

Literature Calling

479

New York

Journal of

Functional Neurology, Rehabilitation, and Ergonomics The Official Journal of the International Association of Functional Neurology and Rehabilitation The aim of this interdisciplinary journal is to provide a forum for the fields of Biomedical and Rehabilitation Engineering, Neuropsychology, Clinical Neurology, Human Factors and Ergonomics, and vocational assessment and training to present critical ideas, theories, proof-of-concept for technology solutions, and data-based evaluative research to facilitate return to work or more effective functional development in children and adults.

Functional Neurology, Rehabilitation, and Ergonomics is published quarterly by Nova Science Publishers, Inc. 400 Oser Avenue, Suite 1600 Hauppauge, New York 11788, USA Phone: (631) 231-7269 Fax: (631) 231-8175 E-mail: [email protected] Web: www.novapublishers.com ISSN: 2156-941X Institutional Subscription Rates per Volume Print: $295

Electronic: $295

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Additional color graphics might be available in the e-version of this journal. Copyright © 2014 by Nova Science Publishers, Inc. All rights reserved. Printed in the United States of America. No part of this Journal may be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, electrostatic, magnetic, tape, mechanical, photocopying, recording, or otherwise without permission from the Publisher. The Publisher assumes no responsibility for any statements of fact or opinion expressed in the published papers.

Editor-In-Chief Gerry Leisman Garden City, NY USA Nazareth, Israel Co-Editor-In-Chief Robert Melillo Garden City, NY USA Assistant Editor Production Janet Groschel Gilbert, AZ USA

Assistant Editor Production Alicia M. Zelsdorf Charlotte, NC USA

Assistant Editor News and Events Tricia Merlin Cape Canaveral, FL USA

Sergio Azzolino San Francisco, CA USA

Editorial Board Members Barbara Hicks Kingsford, MI USA

Jackie Oldham Manchester, UK

Randy Beck Perth, Australia

Newton Howard Cambridge, MA USA

Chandler Phillips Dayton, OH USA

Paul Berger-Gross Bayside, NY USA

Megan L. Hudson West Springfield, MA USA

Anthony L. Rosner Boston, MA USA

Eti Ben-Simon Tel-Aviv, Israel

Efraim Jaul Jerusalem, Israel

Peter Scire Peachtree City, GA USA

John A. Brabyn San Francisco, CA USA

Datis Kharrazian Carlspoor, CA USA

Fredric Schiffer Boston, MA USA

Orit Braun-Benjamin Karmiel, Israel

Samuel Landsberger Los Angeles, CA USA

Suryakumar Shah Pomona, NJ USA

Lynn M. Carlson West Springfield, MA USA

Calixto Machado Havana, Cuba

Maria E. Stalias Manhasset, NY USA, Athens, Greece

Ted Carrick Cape Canaveral, FL USA

Joy MacDermid Hamilton, Ontario Canada

Emmanuel Donchin Tampa, FL USA

Joav Merrick Jerusalem, Israel

Andrew L. Egel College Park, MD USA

Raed Mualem Nazareth, Israel

Khosrow Eghtesadi West Palm Beach, FL USA

Paul Noone Hampton E. Victoria, Australia

Joseph Weisberg Great Neck, NY USA Leslie Weiser Boston, MA USA Seung Won Lee Seoul, S. Korea

EDITORIAL

Funct Neurol Rehabil Ergon 2013;3(4):413-418

ISSN: 2156-941X © Nova Science Publishers, Inc.

COLLEGIALITY AFFECTS OUTPUT

Gerry Leisman Editor-in-Chief, FNRE Scientific Director F. R. Carrick Institute for Clinical Ergonomics, Rehabilitation, and Applied Neuroscience, USA The National Institute for Brain and Rehabilitation Sciences, Nazareth, Israel ORT-Braude College of Engineering, Karmiel, Israel Universidad de Ciencias Médicas de la Habana, Facultad Manuel Fajardo I know that I am going out on a limb on this but I believe that very little that is earth-shattering and useful has come from the “ivory tower.” If one were to examine the list of Nobel Prize winners and examine their affiliations [1], one would find that of the 320 freestanding research institutes, universities, corporations, and hospitals to which the Nobelists were affiliated, 203 (63 percent) of the Nobels were awarded to researchers whose full-time positions were not university-based. Other than corporations where success is measured in financial terms and researchers are beholden to shareholders, the majority of Nobel Prize winners in the sciences and economics worked in environments in which collegiality was a mainstay of day-to-day life, and interdisciplinary cooperation was de regueur and required by good intellectual etiquette. For the forty years that I have been in academia, I have had my laboratories, my students, my visitors, my courses, but precious little cross-fertilization of scientific ideas because each member of my faculty sat behind his or her own closed door with his or her own “family,” as if living in a high-rise apartment building in Manhattan where one, with difficulty, knows one’s neighbors. Do you really think that such an environment facilitates progress? It does, if one models family life as consisting of one child and no parents or siblings. I know how the science game is played. Academia is a pyramid with a very big base of PhDs and postdocs doing the heavy work. There are very few spots that exist on the top of the pyramid. These spots, taken by Assistant professors, Associate, and then Full Professors have very slow rates of rotation. One can think that one can get some crumbs from people who one thinks are important and who will have an impact on one’s career. If you really believe that, you are psychotic!!!!!!!!!!!! I had thought in the past that academia was a place where noble people would collaborate for a greater good. Smart people and good ideas would prevail. NONSENSE!!!!!!!!!! Academic life, like in any other field of life, is run by humans. As such, humans are capable of the best, and the worst. Welcome to the real academia. It is necessary to suck up to progress. Don’t get too original, too smart, or too effective. It is not enough to be good, you need to be political. Without “friends” you go nowhere. Without a “godfather” you go nowhere. Corporations are not much different. In academia, when there is cooperation there is progress; when there is not, there exists old, tenured, superannuated hags, who largely rest on their laurels and the little that they have produced since their earlier years. I look around for academic groups at levels of excellence in any particular sub-discipline with the result being the existence of 5 or 6 groups worldwide in each area of focus, quite a niche market. How’s that for overspecialization?

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Gerry Leisman

If one wants to survive in academia, one should best update one’s skills regularly and jump to the next hyped field. Adapt, but not intellectually. Returning to the Nobel model, much of the work in the sciences for which the Prize has been awarded was for work performed by individuals while largely in their 20’s and 30’s. Einstein once commented, "A person who has not made his great contribution to science before the age of thirty will never do so." [2] This may have been an accurate reflection of physics around the quantum mechanics revolution of the 1920s, but it is no longer the case for every field, according to an analysis of the age of Nobel laureates when they performed their prizewinning work. Now, older scientists are making the great discoveries. "Einstein, on this point, does not appear to be correct," says Benjamin Jones, an expert in innovation at the Kellogg School of Management at Northwestern University in Evanston, Illinois, who co-authored the study. "Scientists are typically getting older and the probability of making a discovery before age 30 has gone way down." Jones and Weinberg [3] analyzed 525 Nobel prizes awarded in physics, chemistry and medicine between 1900 and 2008. The pair used historical and biographical information to work out how old each laureate was when he or she performed the prizewinning work. They found that with a few exceptions — notably for the quantum mechanics discoveries of the 1920s and 1930s, which were often made by scientists under 30 — the trend across all fields is towards researchers being older when they produce their greatest work. In chemistry the frequency of discoveries made over 30 and over 40 has increased significantly, whereas medicine has seen an increase in frequency of discoveries made over 30 but less so over 40. In physics, discoveries in the early twentieth century were made very young, before this field too joined the ageing trend. The data on which the analysis is based may be readily found on the Nobel website [4] To explain the aging effect, Jones and Weinberg suggest a shift from theoretical work, in which younger people do better, towards experimental work, which requires experience, aggregation of knowledge, and most importantly, big expensive equipment that typically younger scientists do not have. By the way, Jones and Weinberg argue for the age of contribution getting older. Older than what though? With discoveries being made over 30 and 40 it is still not over 50 and 60. If one does not want to argue about Nobel Prizes, also making the case for principal work among younger individuals is the case of the Fields Medal for Mathematics. The prize is given every four years with a hiatus during WWII and started in 1936. The mean age of the recipients is 34.68 years and with little variability with a 0.7. The distribution of the prize and the age of the recipients at the time of the prize are found in Table 1 and are worth a moment of scanning. Table 1. Recipients of the Fields Medal for Mathematics and age at receipt of the award Year

Name

Gender

Citizenship

Born

Affiliation at the time of the award

2010

Ngô Bao

Châu

Male

Vietnam

1972

Université Paris-Sud & Instit. for Advanced Study

2010

Elon

Lindenstrauss Male

Israel

1970

Hebrew University of Jerusalem

2010 2010

Stanislav K.

Smirnov

Male

Russia

1970

University of Geneva

Cédric

Villani

Male

France

1973

École Normale Supérieure & Institut Henri Poincaré

2006

Andrei

Okounkov

Male

Russia

1969

Princeton University

2006

Grigori

Perelman

Male

Russia

1966

Declined Prize

2006

Terence

Tao

Male

Australia

1975

University of California

Collegiality Affects Output Year

Name

2006

Wendelin

2002

415

Gender

Citizenship

Born


Werner

Male

France

1968

Université Paris-Sud

Laurent

Lafforgue

Male

France

1966

Institut des Hautes Études Scientifiques

2002

Vladimir

Voevodsky

Male

Russia

1966

Institute for Advanced Study

1998

Richard E.

Borcherds

Male

UK

1959

University of Cambridge

1998

W. Timothy

Gowers

Male

UK

1963


1998

Maxim

Kontsevich

Male

Russia

1964


1998

Curtis T.

McMullen

Male

USA

1958

Harvard University

1994

Jean

Bourgain

Male

Belgium

1954


1994

Pierre-Louis

Lions

Male

France

1956

Université de Paris- Dauphine, CEREMADE

1994

Jean-Christophe Yoccoz

Male

France

1957

Université de Paris-Sud (Orsay)

1994

Efim I.

Zelmanov

Male

Russia

1955

University Wisconsin

1990

Vladimir

Drinfeld

Male

Soviet Union 1954

Steklov Mathematical Institute

1990

Vaughan F.R.

Jones

Male

New Zealand 1952

Columbia University

1990

Shigefumi

Mori

Male

Japan

1951

Harvard University

1990

Edward

Witten

Male

USA

1951


1986

Simon K

Donaldson

Male

UK

1957

University of Oxford

1986

Gerd

Faltings

Male

Germany

1954


1986

Michael H.

Freedman

Male

USA

1951


1982

Alain

Connes

Male

France

1947


1982

William P.

Thurston

Male

USA

1946


1982

Shing-Tung

Yau

Male

China

1949


1978

Pierre René

Deligne

Male

Belgium

1944


1978

Charles Louis

Fefferman

Male

USA

1949


1978

Margulis Gregori Aleksandrovitch

Male

Soviet Union 1946

University of Moscow

1978

Daniel G.

Quillen

Male

USA

1940

Massachusetts Institute of Technology (MIT)

1974

Enrico

E Bombieri

Male

Italy

1946

University of Pisa

1974

David Bryant

Mumford

Male

USA

1937

Harvard University

1970

Alan

Baker

Male

UK

1939


1970

Heisuke

Hironaka

Male

Japan

1931

Harvard University

1970

Serge P.

Novikov

Male

Soviet Union 1938

Belorusskii University

1970

John Griggs

Thompson

Male

USA

1932

University of Chicago

1966

Michael Francis Atiyah

Male

UK

1929


1966

Paul Joseph

Cohen

Male

USA

1934

Stanford University

1966

Alexander

Grothendieck Male

France

1928

University of Paris

416

Gerry Leisman Table 1. (Continued)

Year

Name

Gender

Citizenship

Born


1966

Stephen

Smale

Male

USA

1930


1962

Lars

Hörmander

Male

Sweden

1931

Stockholm University

1962

John Willard

Milnor

Male

USA

1931


1958

Klaus Friedrich Roth

Male

UK

1925

London University

1958

René

Thom

Male

France

1923

University of Strasbourg

1954

Kunihiko

Kodaira

Male

Japan

1915


1954

Jean-Pierre

Serre

Male

France

1926

Collège de France

1950

Laurent

Schwartz

Male

France

1915

Nancy University

1950

Atle

Selberg

Male

Norway

1917


1936

Lars Valerian

Ahlfors

Male

Finland

1907

Harvard University

1936

Jesse

Douglas

Male

USA

1897

MIT

We can also see a similar effect for the recipients of the $250,000 Turing Prize in Computer Sciences with the list of recipients presented in Table II. The average age at the time of the receipt of the prize was 54.29 and a but for work that was performed earlier in their academic lives Table 2. List of recipients of the Turing Prize [5] Year

Name

Gender

Citizenship

Born


2012

Shafi

Goldwasser

Female

Israel

1958

Weizmann Institute of Science

2012

Silvio

Micali

Male

Italy

1954

2011

Judea

Pearl

Male

Israel

1936

MIT (Massachusetts Institute of Technology) UCLA (University of California Los Angeles)

2010

Leslie Gabriel

Valiant

Male

UK

1949

Harvard University

2009

Charles P.

Thacker

Male

USA

1943

Microsoft Research

2008

Barbara

Liskov

Female

USA

1939

MIT (Massachusetts Institute of Technology)

2007

Joseph

Sifakis

Male

France

1946

CNRS (Centre national de la recherche scientifique)

2007

Ernest Allen

Emerson

Male

USA

1954

University of Texas

2007

Edmund Melson

Clarke

Male

USA

1945

Carnegie Mellon University

2006

Frances E. "Fran" Allen

Female

USA

1933

IBM Fellow Emerita

2005

Peter

Naur

Male

Denmark

1928

Copenhagen University

2004

Robert E.

Kahn

Male

USA

1938

CNRI (Corporation for National Research Initiatives)

2004

Vinton Gray

Cerf

Male

USA

1943

MCI Communications Corp

2003

Alan Curtis

Kay

Male

USA

1940

Hewlett Packard Senior Fellow

Collegiality Affects Output

417

Year

Name

Gender

Citizenship

Born


2002

Ronald Linn

Rivest

Male

USA

1947

MIT (Massachusetts Institute of Technology)

2002

Leonard M.

Adleman

Male

USA

1945

University of Southern California

2002

Adi

Shamir

Male

Israel

1952


2001

Kristen

Nygaard

Male

Norway

1926

University of Oslo

2001

Ole-Johan

Dahl

Male

Norway

1931

University of Oslo

2000

Andrew Chi-Chih Yao

Male

China

1946


1999

Frederick P.

Brooks

Male

USA

1931

University of North Carolina

1998

James Nicholas "Jim"

Gray

Male

USA

1944

Microsoft Research

1997

Douglas C.

Engelbart

Male

USA

1925

SRI International

1996

Amir

Pnueli

Male

Israel

1941


1995

Manuel

Blum

Male

Venezuela

1938

Carnegie-Mellon University

1994

Raj

Reddy

Male

India

1937


1994

Edward Albert

Feigenbaum

Male

USA

1936

Stanford University

1993

Richard Edwin

Stearns

Male

USA

1936

University at Albany

1993

Juris

Hartmanis

Male

USA

1928

Cornell University

1992

Butler W.

Lampson

Male

USA

1943

Digital Equipment Corporation

1991

Robin

Milner

Male

UK

1934

Edinburgh University

1990

Fernando José

Corbató

Male

USA

1926

1989

William Morton

Kahan

Male

Canada

1933

MIT (Massachusetts Institute of T h l of) California University

1988

Ivan Edward

Sutherland

Male

USA

1938

Sutherland, Sproull

1987

John

Cocke

Male

USA

1925

IBM

1986

John E.

Hopcroft

Male

USA

1939

Cornell University

1986

Robert Endre

Tarjan

Male

USA

1948


1985

Richard M.

Karp

Male

USA

1935


1984

Niklaus

Wirth

male

Switzerland

1934

Swiss Federal Institute of Technology

1983

Dennis MacAlistair Ritchie

Male

USA

1941

Bell Telephone Laboratories

1983

Kenneth Lane

Male

USA

1943

Bell Telephone Laboratories

1982

Stephen Arthur

Cook

Male

USA

1939

University of Toronto

1981

Edgar F. "Ted"

Codd

Male

UK

1923

IBM Almaden Research Center

1980

Charles Antony Richard Kenneth E.

Hoare

Male

UK

1934


Iverson

Male

Canada

1920

IBM Research

1979

Thompson

1978

Robert W

Floyd

Male

USA

1936

Stanford University

1977

John Warner

Backus

Male

USA

1924

IBM Research

1976

Michael Oser

Rabin

Male

Israel

1931

Hebrew University

1976

Dana Stewart

Scott

Male

USA

1932


1975

Allen

Newell

Male

USA

1927


1975

Herbert Alexander Simon

Male

USA

1916


1974

Donald Ervin

Knuth

Male

USA

1938

Stanford University

1973

Charles W.

Bachman

Male

USA

1924

General Electric

1972

Edsger Wybe

Dijkstra

Male

Netherl.

1930

Eindhoven University of Technology

418

Gerry Leisman Table 2. (Continued)

Year

Name

1971

John

Gender

Citizenship

Born

McC arthy

Male

USA

1927


1970

James Hardy

Wilkinson

Male

UK

MIT (Massachusetts Institute of Technology) 1919 1919 National Physical Laboratory

1969

Marvin Lee

Minsky

Male

USA

1927

1968

Richard Wesley

Hamming

Male

USA

1915

MIT (Massachusetts Institute of Technology) Bell Telephone Laboratories

1967

Maurice Vincent

Wilkes

Male

UK

1913


1966

Alan Jay

Perlis

Male

USA

1922

Yale University

So what is going on with us older people that were so productive during their early years and less so later? Does it depend on how one defines productive? Is it only the older people who have insights? Do younger people have much to offer? Is burn-out in the picture? How can you beat getting to the top without pain, humiliation, and prostitution? Is there anything after a Nobel or a Fields? I do not think it is largely burn-out as older research physicians, psychologists, chemists, physicists and others still produce – but not at the level of sustained highest level effort that once was. Now they are invited to speak at conferences, sit on government panels and review bodies, vote on tenure and appointment of others, attend conferences in exotic places, and even occasionally sabbaticals on some Mediterranean island for six months. Our computer science colleagues, on the other hand, find it necessary for technical reasons, to work in groups. Software engineers do not usually handle architecture and hardware problems and the projects will not get completed at the highest level without the intense collegiality of others in related disciplines. It is my editorial contention (not well researched) that the young scientists succeeded because of the collegiality that existed before fame blinded their ability to listen to others. Success in academic terms was found because they did not have their own office and laboratory behind “closed doors” coming out to attend department meeting every so often. They worked hard and with others. Do not even think that you can advance your careers in the vacuum of private practice. See you at IAFNR-2014 in Las Vegas.

REFERENCES [1] [2] [3] [4] [5]

Nobel Prize Organization. http://www.nobelprize.org/nobel_prizes/lists/universities.html (Downloaded 8 November 2013). Brodetsky S. The Jews in the post-war settlement. The Lucien Wolf Memorial Lecture.1942, London, UK: Jewish Historical Society of London. Jones BF, Weinberg BA. Age dynamics in scientific creativity. Proc Natl Acad Sci USA. 2011, 108(47):18910-18914. Nobel Prize Organization. http://www.nobelprize.org/nobel_prizes/. (Downloaded 8 November, 2013). Association of Computing Machinery. http://awards.acm.org/awards-search.cfm (Downloaded 8 November, 2013).

CLINICAL PAPERS



UNUSUAL BRAINSTEM TWISTING REVEALED BY MRI TRACTOGRAPHY IN A PATIENT WHO SURVIVED A SEVERE TRAUMATIC UPPER SPINAL DISLOCATION Calixto Machado1,, Jesús Pérez-Nellar2, Rafael Rodríguez3, Mario Estévez1, Phillip A. DeFina4, Adam Schiavi5, Gerry Leisman6,7,8, Frederick R. Carrick7,9, Robert Melillo6,7, and Mauricio Chinchilla2 1

Institute of Neurology and Neurosurgery, Department of Clinical Neurophysiology, Havana, Cuba 2 Hermanos Ameijeiras Hospital, Service of Neurology, Havana, Cuba 3 International Center for Neurological Restoration Havana, Cuba 4 International Brain Research Foundation, Chief Executive Officer/Chief Scientific Officer 5 Anesthesiology and Critical Care Medicine, and Neurology Neurosciences Critical Care Division. The Johns Hopkins Medical Institutions, USA 6 The National Institute for Brain & Rehabilitation Sciences, Nazareth, Israel 7 F. R. Carrick Institute for Clinical Ergonomics, Rehabilitation, and Applied Neurosciences (CERAN), Gilbert, AZ, USA 8 O.R.T.-Braude College of Engineering, Karmiel, Israel 9 F.R. Carrick Institute for Graduate Studies, Cape Canaveral, Florida, USA We report unusual twisting of the brainstem in MRI tractography in a patient who survived a traumatic brain injury with upper spinal dislocation. A 39-year-old male patient involved in a highspeed car accident was admitted in coma on February 2003. He had a Glasgow Coma Scale of 4, respiratory arrhythmia and tetraplegia. Four weeks later he was diagnosed as being in a persistent vegetative state (PVS). Our group evaluated him for first time in 2010. The patient was then in a minimally conscious state (MCS), with a limited but clear evidence of awareness of the environment, based on a reproducible gestural response following simple commands and visual pursuit of relatives and other persons in his room. He maintained a severe tetraplegia, hyperreflexia, and bilateral Babinski sign. Neuroimaging studies (Figure 1) performed according to our protocol for the assessment of PVS/MCS,[1,2] demonstrated a severe atrophic and twisted brainstem. There was an MRI-T2 hyperintensity in the lower part of the medulla oblongata that suggested the presence of an old infarct, probably due to an ischemic and/or hemorrhagic insult because of the compression of the brainstem. MRI-Tractography revealed brainstem long tracts twisting. In order to have a better visualization of bone abnormalities, CT with 3D reconstruction was performed demonstrating a rotatory deformity of the upper spine. 

Address for Correspondence: Dr. Calixto Machado, Institute of Neurology and Neurosurgery, Department of Clinical Neurophysiology, 29 y D, Vedado, La Habana 10400 Cuba. E-mail: [email protected]

422

Calixto Machado, Jesús Pérez-Nellar, Rafael Rodríguez et al.

Figure 1. A-B: MRI (T2, coronal view), shows brainstem deformity and T2 hyperintensity in the lower part of the medulla oblongata suggesting the presence of an old infarct. C: Tractography reveals twisting of long tracts along the brainstem. D: MRI (T1, sagittal view), showed severe atrophy of the brainstem. E-F: CT-3D showing a rotatory upper spine dislocation.

In our patient hypoxic encephalopathy secondary to acute respiratory insufficiency was surely the cause of his chronic consciousness disorder.[1,2] Probably his critical condition, and the needs of life support protocols upon arrival to the intensive care, hampered the with upper spinal dislocation diagnosis,[3-5] leading to a lack of radiological evidence, or due to the presence of additional injuries where a clinical examination is impossible. The demonstration of brainstem twisting instead of section in MRI-Tractography has is an unusual neuroimaging finding, and scientifically highlights the neuroimaging findings in this patient who survived severe upper spinal dislocation.

REFERENCES [1] [2]

[3]

[4] [5]

Machado C. Persistent vegetative and minimally conscious states. Rev Neurosci. 2009;20: 203-20. Machado C, Estévez M, Rodríguez R, Pérez-Nellar J, Gutiérrez J, Carballo M, Olivares A, Fleitas F, Pando A, Beltrán C. A Cuban Perspective on Management of Persistent Vegetative State. MEDICC Rev. 2012;14:44-8. Ehlinger M, Charles YP, Adam P, Bierry G, Dosch JC, Steib JP, Bonnomet F. Survivor of a traumatic atlanto-occipital dislocation. Orthopaedics & Traumatology: Surgery & Research. 2011;97:335-40. Hamai S, Harimaya K, Maeda T, Hosokawa A, Shida J, Iwamoto Y. Traumatic AtlantoOccipital Dislocation With Atlantoaxial Subluxation. Spine. 2006; 31:E421-E24. Munoz-Mahamud E, Combalia A, Bori G. Traumatic atlanto-occipital dislocation: a case report. Journal of Orthopaedic Surgery. 2012;20:391-4.



THE TREATMENT OF PERSISTENT IMBALANCE IN A PATIENT WITH TRAUMATIC BRAIN INJURY USING A FUNCTIONAL NEUROLOGICAL APPROACH Susan E. Esposito1, Linda E. Mullin1, and Frederick R. Carrick2 1

Life University, Department of Chiropractic Sciences, Marietta, GA USA 2 Carrick Institute of Graduate Studies, Cape Canaveral, Florida USA

ABSTRACT Background: We describe a case of a 39 year-old male, who presented to a university-based functional neurology clinic for impaired balance following a Traumatic Brain Injury (TBI). After 4 years of neuropsychiatric and neurobehavioral treatment, he remained completely disabled with symptoms of persistent and severe balance issues, anxiety, depression with suicidal thought, decreased attention and concentration, short and long term memory loss, difficulty following spoken directions, perseveration of thought, as well as debilitating physical complaints. Methods: Diagnostic inventories of brain function revealed a frontal lobe syndrome with a central vestibular axis and a postural instability confirmed by computerized dynamic posturography (CDP). The patient underwent ten weeks of vestibular rehabilitation strategies including frontal eye movements and multi-axis labyrinthine and otolithic stimulation. Results: Improved stability confirmed by computerized dynamic posturography. There was a significant improvement in cognitive abilities, a reduction in anxiety and stability of mood. The patient reported increased energy, clarity of thought and memory. There was also a progressive return of normal speech patterns. Processing speed and executive functions were observed to be appropriate. The number and severity of physical complaints decreased and he was able to regain independence and resume regular social activities. Conclusion: Post-TBI patients commonly complain of imbalance while standing or walking. This is a case of a patient, 4-years post TBI with persistent balance issues who demonstrated significant recovery of functions after treatment with vestibular rehabilitation strategies.

Keywords: traumatic brain injury, post-traumatic stress disorder, vestibular therapy, vestibular function tests, posture/physiology, chiropractic

INTRODUCTION Traumatic brain injury is defined as damage to the brain resulting from external mechanical force, such as rapid acceleration or deceleration, impact, blast waves, or penetration by a projectile. [1] 

Correspondence: Dr. Susan E. Esposito, Life University, Department of Chiropractic Sciences, 1269 Barclay Circle, Marietta, GA 30060 USA. Email: [email protected]

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Susan E. Esposito, Linda E. Mullin, and Frederick R. Carrick

Temporary or permanent impairment of brain function is the sequella of TBI. [2] It is estimated by the National Institute of Health that 503 million Americans currently live with disabilities resulting from TBI. The costs associated with TBI are about $60 billion annually in the US and it contributes to 30% of all injury-related deaths. [3-5] Problems with cognition and behavior or mental health are among the most frequent sequelae after TBI. Persistent issues with balance are also noted in the literature. [68] Effects of brain injury can cause potentially permanent changes and may even lead to posttraumatic stress disorder (PTSD). [9] Hillier et al. interviewed 67 subjects with TBI of mixed severity (mostly severe) at 5-years post injury and found that, after headache, balance deficit was the secondmost frequent self-reported physical symptom. [10] This case report describes a patient presenting with disturbance of balance, cognition and behavior of 4 years duration after TBI, successfully managed with a chiropractic functional neurological approach.

CASE PRESENTATION A 39 year-old right handed male, presented with symptoms of unsteadiness and balance disturbance. He was well developed and well nourished. His affect was blunt. He appeared to be sleepy, confused, forgetful and disorganized. His summary of injuries included a depressed skull fracture of the left frontal bone, facial fractures, complex laceration of the right forehead and open comminuted fracture of the right frontal sinus as well as right retinal tear four years earlier. He underwent a bi-frontal craniotomy. His eight-point Extended Glasgow Outcome Scale (GOS-E) was rated as a 3 indicating severe disability (cannot live independently and requires daily assistance due to physical or mental impairment). In addition to his balance issues, he had difficulty with concentration, persistent severe headaches with scintillating scotoma, irritability, depression, difficulty sleeping, lack of energy, dizziness, and both long and short term memory loss. He complained of diffuse ongoing left sided body pain in both upper and lower extremities as well as his torso. He used stability devices around the home including a tub bench for showering because of his impaired balance. Four years after sustaining his TBI by being bludgeoned with a gun handle in an attack, he maintained considerable fear and anxiety surrounding his attack as well as a hyper-vigilance to other possible health issues. He was easily frustrated due to his cognizance of his cognitive impairments, which included short and long term memory deficits, decreased attention and difficulty finding words. He struggled to follow conversations. This patient had sufficient functional cognitive impairment to require a daily residential program to manage his activities of daily living (ADL). He necessitated a checklist for his morning and evening routine which included showering, setting alarms, taking medications, locking and unlocking the doors, loading the pillbox weekly, organizing a grocery list, money management and doctor’s appointments. He was unable to independently care for himself, drive a car or ride a bicycle and he used pre-programmed audio and visual alarms for management of his medications. His medications included Aricept, Naprosyn, Paxil, and Tylenol. He was diagnosed by his attending neurologist with traumatic brain injury and post-traumatic stress disorder (PTSD). His debilitating symptoms persisted despite 4 years of rehabilitation therapies including eye exercises, balance exercises and music therapy.

EXAM FINDINGS The patient was measured at 71” and 175 pounds. His BP, pulse, temperature and respiration rate were within normal limits. On inspection the patient displayed normal, symmetrical facial tone at rest and with volitional movements; however, on spontaneous smiling (emotionality) left sided facial paresis was noted. Light reflex exam revealed blepheroclonus with aversive reaction bilaterally.

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Table 1. Patient complaints and exam findings Pre-treatment Daily chronic depression and anxiety with suicidal thoughts Chronic, sharp frontal and midline head pain Disturbed sleep - waking unrested Yellow light bursts in right eye - up to 100 per day Photophobia to direct light (sunlight and room light) CAPS testing - stability score on flat surface of 0 CAPS testing - stability score on perturbed surface of 0 Cautious, slow gait with veering to right Decreased arm swing during straight line ambulation right more than left - worsened by dual tasking Olfaction detection 7" right and 2" left Anisocoria A/V ratios 1:3 bilaterally Direct light reflex - Hippus of right pupil Convergence test failed at 3" in left eye Pursuits caused diplopia in all angles of right gaze Saccades - increased latency and dysmetric to the right. Leftward saccades were dysmetric to closely approximated targets OPK- Increased latency of fast phase refixations in horizontal directions - Lateral pulsion in vertical directions Hyperesthesia- right CN V and T1 dermatome Left ptosis Left facial paresis with emotionality Palatal paresis on left Tongue myokymia on protrusion Vibration sense- absent on great toe on right and diminished on left Synkinesis of trapezius and upper extremity flexors were elicited on biceps reflex bilaterally. Percussion myoclonus was noted upon striking the thenar eminence bilaterally. Finger to nose inaccurate on right Heel to shin inaccurate on the right Finger thumb tapping test - Grade 3 right, Grade 2 left Performed 2 of 3 commands in a series Recalled 1 of 3 words in a 5 minute delayed recall test

Post-treatment Resolved Resolved Sleeping through the night 2-3 occurances per week No evidence of photophobia Stability score of 84.2 (eyes open) and 70.2 (eyes closed) Stability score of 68.7 (eyes open) and 60.6 (eyes closed) Appropriate cadance, stride length and speed Equal arm swing Olfaction detection 10" bilaterally Symmetrical pupil diameter A/V ratios 1:2 bilaterally Resolved Improved to 1" in left eye Diplopia only at right end-gaze angle Increased accuracy with normal latency

Latency approximating normal limits. Lateral pulsion was resolved. Resolved Resolved Resolved Resolved Resolved Improved on right and WNL on left Resolved Resolved Improved accuracy Resolved Grade 1 right, Grade 0 left Performed 3 of 3 commands in a series Recalled 3 of 3 words in a 5 minute delayed recall test

This aberrant response to light was diminished with lenses that were red-blue when red was placed in the left visual field and blue was in the right visual field. Pursuit, saccade and optokinetic (OPK) testing proved deficiencies in normal eye responses. Rightward pursuits evoked diploplia and nystagmus throughout all angles of right gaze. Rightward saccades had an increase in initiation latency and were dysmetric. Leftward saccades were dysmetric only to closely approximated targets. There was an increased latency of fast phase re-fixations during horizontal OPK stimulations and vertical OPK stimulations produced lateral pulsion. Synkinesis of trapezius and upper extremity flexors were elicited on biceps reflex bilaterally. Percussion myoclonus was noted upon striking the thenar eminence bilaterally. Fine motor testing revealed deficiencies as the finger to thumb tapping

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test was graded 3 on the right with concomitant facial activity and graded 2 on the left. Finger to nose testing was less accurate on the right and it induced a retropulsion. Gross motor coordination deficit was noted with a drift of the left arm on rebound testing of the shoulder flexors. Gait was slow and cautious with decreased arm swing bilaterally (right more than left). Dual mental tasking with gait produced marked hesitations of cadence with decreased speed and stride length. Computerized Dynamic Posturography (CDP) testing was done on a flat platform as well as a perturbation cushion. This revealed a severe impairment in balance as he was unable to maintain his position on the flat platform with eyes open or eyes closed during the test. The patient fell from the platform in a posterior direction. This produced a stability score of 0. (Table 1).

Treatment The patient underwent a series of vestibular and cortical rehabilitation treatments to affect the visual, cortical and proprioceptive centers that control balance over a 10-week period. Red-Blue lenses were employed in the first week of therapy to attenuate the stimulatory effects of white light to the superior colliculi. This markedly reduced his photophobia and headaches. To activate proprioceptive pathways, complex movement therapies were simultaneously and passively delivered in opposing direction to proximal joints of the right upper and lower extremity. To increase the efficacy of the visual neuronal pathways that contributes to balance, eye exercises including micro saccades in a direction up and to the right with gaze holding of inter-saccadic targets were prescribed. Following the micro-saccades, the patient executed pursuits in the direction of upper right to lower left. Vestibulo-cortical pathway activation was achieved through whole body off-axis vestibular rotation (OAVR) in a gyroscope. The direction of rotation utilized was a right yaw with negative pitch axis intended to stimulate the right lower brain stem and left upper brainstem and cortices. Fast stretch adjustments were employed to the right upper limb and right cervical areas.

RESULTS Progressive improvement was measured in the parameters of sway, balance and center of pressure as assessed by CDP over the 10 weeks of treatment (Table 1). The last CDP measure showed the patient could maintain balance for 20 seconds and was rated with a stability score of 84.2 with eyes open and 70.2 with eyes closed. On a perturbation cushion his scores were 68.7 and 60.6 respectively. Pursuit, saccade and optokinetic eye movements showed significant improvement. Diplopia was confined to his extreme right gaze angle. Saccades showed increased accuracy and normal latency in all directions. Latency approximated normal limits with OPK testing in all directions with resolution of lateral pulsion on vertical stimulation. There was no evidence of photophobia, blepheroclonus, facial paresis, percussion myoclonus or synkinesis. Finger to nose testing normalized. Fine motor movements improved with finger to thumb tapping test graded 1 on the right with no concomitant facial activity and graded 0 on the left. No gross motor deficits were observed. Gait was appropriate for cadence, speed and stride length. There was resolution of dizziness, headaches and left sided body pain. Significant improvements were evidenced in cognitive abilities, reduction in anxiety and stability of mood. The patient reported increased energy, clarity of thought and memory. There was also a progressive return of normal speech patterns. He was able to follow and contribute to conversations in a socially acceptable manner. Processing speed and executive functions were observed to be appropriate. As the number and severity of physical complaints decreased he was able to resume regular social activities. He no longer required a residential program and was able to care for himself independently without


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checklists or direction from an outside source. He is able to ride his bicycle, drive a car, live independently, and he even resumed playing the guitar. He had gradually weaned himself off of his medications.

DISCUSSION According to the CDC (2010), the most common cause of death and disability in children and young adults in the US is TBI. The severity of a TBI may range from mild, which is a brief change in mental status or consciousness to severe, characterized by an extended period of unconsciousness or amnesia after the injury. Problems with cognition and behavior or mental health are amongst the most frequent sequelae after TBI. In 2009, Dikmen et al. performed a systematic review to examine the relation between TBI and cognitive impairments 6 months or longer post injury. There was a connection between penetrating head injury and long-term cognitive impairments as seen in this case. [11] Another frequent complaint of patients who have sustained a TBI is problems with balance and instability that may not be apparent on standard clinical examination. These balance studies have shown that subjects, who are placed in a static posture, reveal that persons with TBI exhibit increased sway while standing over neuro-typical subjects. [12-14] Pickett et al., provided evidence that computerized posturography testing can be used in the diagnosis of balance impairments and “provides quantitative data to track changes over time and/or assess the efficacy of treatment interventions.”[15] In this case a CDP was utilized as a measure of balance and an indicator of patient progress. The CDP test is divided into three sensory systems involved in maintaining balance effectively. These systems are visual, proprioceptive and vestibular. The fourth system, which is auditory, is eliminated by the use of noise cancelation headphones during the testing. The patient stands on the force plate facing a blank wall. The feet are placed in a position shoulder width apart. Pressure transducers under the footplate, with calculations for height and weight of the subject, allow measurement of the degree of postural sway relative to the 12.5-degree cone of stability and center of pressure. The patient is asked to maintain balance for 20 seconds. Normal parameters of balance have been established through the study of healthy individuals for each age decade and sex. A score of 0 represents instability to the point of falling or having to step off the platform. A score of 100 represents no sway. According to Pagnacco et al., the stability score from the CDP is the most repeatable on this instrument. [16] This patient showed an improvement in stability with eyes open from a grade of 0 on initial measurement, to a score of 84.2, categorized as between mildly and moderately reduced. For this patient’s age and sex a normal CDP score with eyes open is 90.3. [17] Normal scores for eyes closed are valued at 89.2, which categorizes this patient as severely reduced at 70.2, however he has gained ample stability to be self-sufficient and regain independence. One of the treatment modalities chosen for utilization in this case was a full-body gyroscope. It is classified as a balance training system that is a fully automated, computer-controlled multi-axis rotating chair. This chair can rotate 360o in both pitch and yaw axes simultaneously or individually at velocities to 25 RPM. The rotating chair allows the doctor to activate specific semicircular canals of the vestibular system, which in turn activate corresponding areas of the brainstem and brain. This specific application of stimulation in combination with the other therapeutic exercises and strategies were utilized to promote synchronization of hemispheric activity, enhance proficient vestibular/cerebellar - frontal lobe connectivity, and to enhance neuro-protective plasticity.

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CONCLUSION Many patients with TBI complain of imbalance. This is a case of a patient, 4-years post TBI with persistent balance issues, who demonstrated significant recovery of functions after treatment with a functional neurological approach of specific eye movement, extremity movement and vestibular rehabilitation strategies. Further research into the effects of this method of brain based treatment on unresolved balance issues following TBI is warranted.

REFERENCES [1]

[2] [3]

[4]

[5]

[6] [7]

[8]

[9] [10] [11] [12] [13] [14] [15]

Faul M, Xu L, Wald MM, Coronado VG. Traumatic brain injury in the United States: emergency department visits, hospitalizations, and deaths. Atlanta (GA): Centers for Disease Control and Prevention, National Center for Injury Prevention and Control; 2010. Rutherford, GW; Corrigan, JD (2009). "Long-term Consequences of Traumatic Brain Injury". J Head Trauma Rehab 24 (6): 421–423. Ettenhofer ML, Abeles N: The significance of mild traumatic brain injury to cognition and selfreported symptoms in long-term recovery from injury. J Clin Exp Neuropsychol 2009, 31:363– 372. Ettenhofer ML, Barry DM: A comparison of long-term postconcussive symptoms between university students with and without a history of mild traumatic brain injury or orthopedic injury. J Int Neuropsychol Soc 2012, 18:451–460. Halldorsson JG, Flekkoy KM, Arnkelsson GB, Tomasson K, Magnadottir HB, Arnarson EO: The scope of early traumatic brain injury as a long-term health concern in two nationwide samples: prevalence and prognostic factors. Brain Inj 2012, 26:1–13. Dikmen S, Machamer J, Fann JR, Temkin NR. Rates of symptom reporting following traumatic brain injury. J Int Neuropsychol Soc. 2010 May;16(3):401-11. Epub 2010 Mar 1. Chou, L.-S., Kaufman, K.R., Walker-Rabatin, A.E., Brey, R.H., Basford, J.R., 2004. Dynamic instability during obstacle crossing following traumatic brain injury. Gait and Posture 20, 245– 254. Basford, J.R., Chou, L.-S., Kaufman, K.R., Brey, R.H., Walker, A., Malec, J.F., 2003. An assessment of gait and balance deficits after traumatic brain injury. Archives of Physical Medicine and Rehabilitation 84, 343–349. Ozen LJ, Fernandes MA: Slowing down after a mild traumatic brain injury: a strategy to improve cognitive task performance? Arch Clin Neuropsychol 2012, 27:85–100. Hillier SL, Sharpe MH, Metzer J. Outcomes 5 years post-traumatic brain injury (with further reference to neurophysical impairment and disability). Brain Inj. 1997;11(9):661-75. Dikmen S, Machamer J, Fann JR, Temkin NR. Rates of symptom reporting following traumatic brain injury. J Int Neuropsychol Soc. 2010 May;16(3):401-11. Epub 2010 Mar 1. Newton RA. Balance abilities in individuals with moderate to severe brain injury. Brian Inj 1995;9:445-51. Lehmann JF, Boswell S, Price R,. Quantitiative evaluation of sway as an indicator of functional balance in post-traumatic brain injury. Arch Phys Med Rehabil 1990;71:955-62. Ingersoll CD, Armstrong CW. The effects of closed head injury on postural sway. Med Sci Sports Exerc 1992;24:739-43. Pickett TC, Radfar-Baublitz LS, McDonald SD, Walker WC, Cifu DX. Objectively assessing balance deficits after TBI: Role of computerized Posturography. J Rehabil Res Dev. 2007;44(7):983-90.


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[16] Pagnacco G, Oggero E, Carrick FR. Repeatability of posturographic measures of the mctsib static balance tests a preliminary investigation. Biomed Sci Instrum. 2008;44:41-6. [17] Amin, M., Girardi, M., Konrad, H.R., Hughes, L.F. (2000) "Normative Data for the BalanceTRAK 500". Otolaryngology Head and Neck Surgery, 123(2) P119-20.

Received: June 17 2013 Modified: January 11 2014 Accepted: January 23 2014.



UNUSUAL PRESENTATION OF GLUTEN SENSITIVITY IN A CHILD: SYMMETRICAL DISTAL MYOPATHY David B. Sullivan Private Practice - Mechanicsburg, PA USA

ABSTRACT A 16-year-old athletic female presented with the single complaint of bilateral leg pain in the lateral compartments. She complained of constant, severe burning pain with occasional sharp, stabbing pain of three years duration. Orthopedic diagnosis was bilateral compartment syndrome. She had undergone two separate bilateral fasciotomies which yielded no change in symptoms. She had also undergone multiple rounds of physical therapy which produced no symptomatic improvement. Clinical examination by the author was significant only for weakness of the tibialis anterior bilaterally, with pain on active and passive movement. A review of systems questionnaire revealed mild gastrointestinal symptoms alternating constipation and diarrhea, gas, bloating and fullness after meals. Laboratory parameters revealed the diagnosis of gluten-sensitivity with positive autoimmune markers. These findings suggested an etiology of an autoimmune polymyositis as the pathophysiology of her leg pain. She was recommended to follow a glutenfree diet, and within four months, nearly all of her leg pain and gastrointestinal symptoms had resolved. Her remaining discomfort was eliminated after a twelve-week course of vibration and muscle stimulation in the legs. This therapy is theorized to have reversed neuro-plastic changes, which favored increased efficiency of nociceptive afferent integration. This presentation is unique in the literature and considering that gluten-related myopathies can be present without significant enteropathy, gluten sensitivity should be considered in the differential diagnosis of patients presenting with unexplained and unresponsive musculoskeletal pain.

Keywords: gluten, sensitivity, autoimmune, myopathy, pediatric, neurorehabilitation, neuroplasticity

INTRODUCTION Immunologic sensitivity to the wheat protein gluten, as well as Celiac disease, is associated with many different neurological and musculoskeletal illnesses [1-9]. Most importantly, from a clinical perspective, is that the various neuropathies and myopathies stemming from gluten sensitivity can manifest in the absence of gastrointestinal symptoms. [3,10-13]. In 2010, Hadjivassiliou et al. stated that individuals “who present with neurological manifestations of gluten sensitivity have no gastrointestinal symptoms”. [3,14] The same author described in 2011 a patient with probable inclusion body myositis associated with celiac disease who had no gastrointestinal symptoms. [15]

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Correspondence: Dr. David B. Sullivan, 1001 S. Market St. Suite B, Mechanicsburg, PA, 17055 USA Email: [email protected]

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Hadjivassiliou further states that that although muscle involvement in gluten sensitive individuals is less common, it is a distinct and significant manifestation of the auto-immunologic assault. [3,16] This important feature, and the reality of autoimmune assault on various tissue sites in human disease, should be noted as clinicians develop differential diagnoses in patients with myopathies of unknown etiology, or those conditions which do not readily respond to typical interventions. [17] Thorough investigation and proper management of gluten-related autoimmune states can significantly, and positively, alter the course of treatment for many individuals. [18-20] Herein we present the case of a patient whose myopathy was associated with an autoimmune reactivity to gluten.

CASE REPORT A 16-year-old female presented in November 2011 with a three-year history of bilateral leg pain in the anterior and lateral compartments. She stated that the pain seemed to begin with shin-splint type pain which increased suddenly and severely in December 2008. The pain continued to increase steadily in the following years, finally reaching a daily average of 8-9/10 on a 10-point pain rating scale. She described the pain as a constant, severe burning with occasional sharp, stabbing episodes. She also described the pain as “rubbing metal along the bone.” Her leg pain would be exacerbated by activity and sports, but could also flare to a crippling level during short or extended periods of rest. She reported that ice was the only modality that afforded any symptomatic change. Ice would provide temporary relief while it was in contact with the leg, but the pain would return immediately upon removing the ice. She was not aware of any other patterns associated with her pain, nor did she recall any other specific provocative or palliative measures. The history of her present condition is as follows: In March 2008, she sustained a left ankle inversion sprain during a sporting event. Subsequent radiographic examination of the left leg and ankle revealed no abnormalities of bone or soft tissue. With conservative management, she made a full recovery. Approximately five months later, in September 2008, the patient noticed that she had increasing pain along the anterior aspect of her right leg. This was assumed to be due to her activity during sports. Radiographic examination of the right leg and ankle revealed no abnormalities of bone or soft tissue. By December 2008, the pain was present in both legs and progressively increasing in severity. Her family physician rendered a diagnosis of shin splints. She was instructed to decrease her level of activity in sports, rest her legs more frequently and use over-the-counter pain medication for relief. The patient complied with these orders, but found that the pain continued to intensify. Because her condition failed to resolve with conservative management, the patient was referred for orthopedic evaluation in October 2009 and was diagnosed with chronic bilateral compartment syndrome. A nuclear medicine study was performed in October 2009 to rule out any myeloproliferative involvement. The examination revealed no evidence of myeloproliferation or stress fracture, but a “questionable focus of very subtle” tracer uptake in the right tibia, possibly secondary to shin splints. On follow-up orthopedic evaluation, the patient was recommended to undergo surgical compartment release because of the unrelenting nature of her pain. Bilateral fasciotomy of the anterior and lateral compartments was performed in November 2009. Shortly after recovery, she was enrolled in a sixweek course of physical therapy, which failed to bring about symptomatic improvement. Eight-week postoperative MRI revealed only slight subcutaneous edema, presumed to be due to the surgery. She was referred for more physical therapy, which provided no symptomatic change after four weeks. Because there had been no symptomatic change at five months post-surgery, the patient sought a second opinion at a different orthopedic clinic in April 2010. Her attending physician was suspicious of dysfunctional foot mechanics as the source of her pain. She underwent gait analysis to test for dynamic abnormalities and the potential for improvement with cushioned foot orthotics. Video gait

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analysis revealed no significant abnormalities in her leg mechanics in both shoe-on and shoe-off testing. Despite this assessment, she was enrolled in a physical therapy program designed to optimize lower extremity mechanics and function during activities of daily living and sport. This trial of therapy was unsuccessful at providing lasting pain reduction after three months. Because of the lack of symptomatic change, advanced testing was recommended. The patient was referred for evaluation of leg compartment pressure elevation as an etiology of her pain. Pre- and post-exercise compartment pressure checks via needle manometry were performed. The results revealed post-exercise pressure elevations in the anterior and deep posterior compartments bilaterally, and the left superficial posterior compartment. Due to pressure elevations and increased pain with exercise, surgical corrective procedures were advised. The patient underwent bilateral fasciotomy, this time on all four compartments of both legs in August 2010. After recovery, she was enrolled in a physical therapy program which again failed to provide durable pain relief after four months. In the months that followed, the patient tried other types of therapy to bring about pain relief. A massage therapist and also a chiropractor treated her. She reported that neither was successful in inducing any significant or lasting pain relief. Through the course of her illness, the patients’ pain continued to increase in its severity. By late 2011, her average daily pain level was approximately 8/10. The author first evaluated the patient in November 2011, approximately three years after the onset of pain. Review of systems questionnaire parameters, including cardiovascular, respiratory, genitourinary, endocrine, skin and EENT were deemed to be within normal limits. She did however, report mild gastrointestinal symptomatology with alternating constipation and diarrhea, gas and bloating, and increased abdominal fullness and pressure after meals. On physical examination, her height was 155cm (5’1”) and weight was 56.25kg (124 lbs). She was well developed and well-nourished with an athletic physique. Auscultation of the heart and lungs revealed no abnormalities and cranial nerve testing was within normal limits. Extensive scarring on the skin of both legs was attributed to her previous surgeries. Musculoskeletal examination revealed weakness and pain in the tibilais anterior in both legs. The pain was present on active and passive movement. Muscle strength of the tibialis anterior was 4/5 bilaterally due to pain. She had slight instability on Sharpened Romberg test, and displayed mild dysdiodochokinesia in the left upper extremity with pronation/supination of the forearm. The suspicion of an immunological etiology as a driving factor in her condition was high because of the idiopathic nature of the pain and her non-responsiveness to previous therapies. Laboratory investigations were ordered to screen for food sensitivities, immunological activity and autoimmune markers.

RESULTS Laboratory studies indicated positive elevations in IgA to the following: Wheat, Wheat Germ Agglutinin, Alpha Gliadin 33-mer, Alpha Gliadin 17-mer, Alpha Gliadin 16-mer, Omega Gliadin, Glutenin, Gluteomorphin, Prodynorphin and Gliadin-Transglutaminase. IgG levels to all of the aforementioned compounds were normal. Results also indicated equivocal levels of Wheat IgG and Glutamic Acid Decarboxylase (GAD65) IgA. The results of a cross-reactive foods panel indicated equivocal levels of cow’s milk and polish wheat IgG+IgA combined, and elevated levels of Buckwheat IgG+IgA combined. The pattern of IgA elevations to each parameter in the gluten panels suggested possible celiac disease [21, 22]. The patient was advised as to this possibility and counseled on the importance of duodenal biopsy and genotyping. Ultimately, the patient and her parents elected not to undergo this

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diagnostic confirmation. Regardless, the presence of transglutaminase was also strongly suggestive of autoimmune myopathy. [23-31] Based on the results of her sensitivity panel, she was started on a gluten-free diet. Because a cross-reactive food sensitivity panel showed equivocal elevation to cow’s milk, it was decided to avoid cow’s milk and dairy products as well. The patient was counseled on foods to avoid and other aspects of implementing a gluten-free, dairy-free dietary regimen. She initially had some difficulty in meeting her daily caloric requirements and maintaining stable blood sugar levels. On day four of her diet, the patient complained of drowsiness and fatigue during her school day, and especially after physical education class. While she was reading in the library that afternoon, she became faint and lost consciousness. She was transported via ambulance to the local emergency department where she slowly revived and regained full consciousness. After this alarming incident, the patient and her family were counseled again on implementation of a gluten-free, dairy-free diet, with specific emphasis on meeting the caloric demands of her athletic lifestyle. There were no further incidents related to implementation of the recommended diet. Within three months of following this diet, the patient’s leg pain and gastrointestinal symptoms had resolved by approximately 60%, and had resolved to approximately 90% at four months postimplementation. On four-month follow-up evaluation, the patient rated her average daily pain as a 1/10, down from 8 - 9/10 upon initial presentation. The patient retained some degree of pain and discomfort in both legs, particularly during periods of rest. This was most notable upon arising from sleep or after periods of rest on a couch or reclining chair. The patient also reported that although she was able to resume sports at her previous level, she would notice pain in her legs during vigorous activities and when she would stop running. Because a slight amount of pain remained, and the presumptive cause of her immune-mediated pain had been removed from her diet, it was decided to investigate sensory-motor based therapeutics in an effort to bring forth a complete resolution of her condition. Bedside testing revealed that a 128Hz tuning fork applied to the anterior aspect of the tibia had a positive impact on pain in that area but did not alter pain in the lateral regions. The patient noted that after a brief period of vibratory stimulation, she was unable to elicit any pain on palpation of the anterior aspects of her legs. Instrument-assisted manipulation applied to the tibialis anterior elicited significant pain reduction in the lateral aspects of her legs. Based on her positive response to a trial of care, she was enrolled in a twice weekly course of rehabilitation consisting of vibration and muscle stimulation of the legs. For the first 4 treatments, the patient was placed in the supine position and a 128Hz tuning fork was applied along the length of the tibia. This produced durable pain relief in the anterior aspect of the legs and was discontinued because the anterior pain had completely resolved. In addition, instrumentassisted manipulation was applied along the length of the tibialis anterior. This application was intended to stimulate the muscle stretch reflex and facilitate activation in Aβ afferent nerve fibers. Both legs were treated multiple times at each session in 2-3 minutes increments with an increase in intensity each week. After 5 treatments, the patient noted her first completely pain-free period since the beginning of her ordeal, and after 7 sessions she was able to achieve approximately 7 hours of total and bilateral pain relief. After 3 weeks of care, the analgesic effect of this type of stimulation was complete and almost immediate upon application. At 7 weeks into therapy, the patient reported her average daily pain rating was approximately 0.2-0.5/10 with occasional periods of increased pain of approximately 2/10. At the end of 12 weeks of care, she reported being completely pain-free on a daily basis. Fundamental principles of neuro-rehabilitation were observed during her treatment, including monitoring for any autonomic or somatosensory concomitants that might indicate neuro-physiologic fatigue of the related neural-sensory pathways.


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DISCUSSION Gluten Sensitivity in the Etiology of Pain The presentation of gluten sensitivity is typically associated with various gastrointestinal manifestations including cramping, bloating, alternating constipation and diarrhea, and excessive gas. It is not uncommon though for individuals to present with dermatological or neuro-musculoskeletal complaints only and make no mention of enteric involvement. Cases in which gastrointestinal symptoms are either minimal or absent can lead to misdiagnosis or ineffective treatment if gluten sensitivity is not considered as part of the differential diagnosis. It has been well-documented that gluten sensitive individuals, and even celiac patients, frequently present only with nongastroenteropathic symptoms. [3,2] Furthermore, gluten sensitivity and Celiac disease are both associated with neurological and musculoskeletal dysfunctions, which are frequently misinterpreted or overlooked in the course of clinical diagnostics and management. [14, 33] Because the patient elected not to undergo muscle biopsy investigation, it was not possible to histologically confirm the diagnosis of polymyositis in the tibialis anterior muscles. Furthermore, because she also declined duodenal biopsy, a definitive diagnosis of Celiac disease versus gluten sensitivity could not be made. Her clinical response however, strongly suggests an immune-based inflammatory etiology of her pain. To date, there have been no case reports, which document the presentation of distal, symmetrical myopathy as a consequence of gluten sensitivity in a child. There have been however, reports of gluten sensitivity and even celiac disease presenting with proximal myopathy, and distal myopathy in a 64 year old male. [3]

Proposed Mechanism of Action Based on the physical and neurological findings displayed by the current patient, and her responses to treatment, the author proposes the following mechanism of action regarding the development and resolution of leg pain. An autoimmune, inflammatory assault on the muscles in the anterior compartments of the legs is proposed to be the primary culprit in the onset and development of the patients’ pain. [26,34-36] This process produced prolonged activation of C-fiber afferent projections from those compartments. With chronic nociceptive afferent input, the associated excitatory interneurons of the dorsolateral tract of Lissauer and their corresponding second order neurons of the anterolateral system adapted to become highly efficient in transmitting nociceptive afferent signals. In other words, the nociceptive afferent pathways underwent neuroplastic changes, or ‘wind-up’, which up-regulated their frequency of signal transmission. [37,38] Once the offending antigens were discovered and removed from the diet, the immunological assault was allowed to subside. In time, inflammatory activity in the legs reached a minimum and was no longer the main nociceptive signal generator, if at all. What remained, as the source of nociceptive transmission, was the neuro-plastic changes that had occurred in the dorsal horns of the patient’s spinal cord. More specifically, the patient continued to feel pain because inhibitory inter-neuronal pools had become reduced in their ability to hyperpolarize nociceptive projection neurons of the spino-thalamic tract. This apparently ineffective “gating” mechanism could have also been produced via upregulation of glutamate receptors and/or increases in synaptic glutamate concentrations within the inter-neuronal pools of the dorsal horn and corresponding projection fibers.

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Because clinical examination revealed the profound analgesic effects of large diameter Aβ mechanoreceptive afferentation, that same type of Aβ fiber stimulation was used as a means to facilitate the upregulation of anti-nociceptive inter-neuronal pool integration. Repeated stimulation of this pathway over a twelve week course of care led to an ‘unwinding’ of pro-nociceptive signal bias while enhancing the anti-nociceptive integration of the associated inhibitory inter-neuronal pools. The beneficial effects of Aβ mechanoreceptive afferentation make sense in the context of her history, where pain levels would rise sharply during periods of rest, when the frequency of afferent input was diminished or absent. Alternatively, another mechanism of action could be possible. Because tissue biopsy of the legs had not been performed, the author was unable to definitively confirm inflammatory assault in the lower extremities. If in fact, there had been no significant muscle compartment inflammatory involvement, it would suggest decreased output from inhibitory, anti-nociceptive inter-neuronal pools as the primary site of dysfunction. In this scenario, an autoimmune attack against those cell populations with their resulting dysfunction, could still explain the patients’ positive response to a gluten-free diet, as well as the residual pain relief, which was achieved through Aβ mechanoreceptive afferentation. This mechanism is thought to be less likely due to the fact that application of ice to the painful area brought some palliative, albeit temporary effect. Because nociceptive afferents and sensation to cold both ascend in the anterolateral system, it is plausible that increasing activity in that system via cold stimulation would heighten it’s output and exacerbate the sensation of pain. Because this effect was not observed (in fact cold stimulation provided relief), it suggests a primary nociceptive generator from the periphery. Upon decreasing the frequency of activation of free nerve endings and C-fibers through the application of ice, inter-neuronal pools were able to exert some degree of anti-nociceptive functionality. Upon cessation of cold stimulation however, the amount of C-fiber activation from an immunologically based inflammatory assault in the legs was enough to overwhelm any inhibitory effects of dorsal horn interneurons and as a result, the pain returned immediately.

CONCLUSION The deleterious immunological effects of gluten sensitivities have been shown to be a real, and valid pathological mechanism behind not just gastroenterological conditions, but neurological and musculoskeletal disease as well. [39] Gluten-induced immunological assault should be considered part of the differential diagnosis for any type of condition which is unresponsive to standard treatments, even if no enteropathic symptoms are present. Additionally, the phenomena of neuroplasticity is a vitally important attribute of the human nervous system, especially as it relates to pain, and must be considered in the evaluation and management of chronic pain states. By understanding the mechanisms and ramifications of neuroplasticity, the knowledgeable clinician will be able to apply these concepts in a practical way, with the ultimate goal of directing positive and adaptive changes within the neuraxis. Within the context of the present case, mechanoreceptive afferentation and it’s theorized effects on pre- and postsynaptic nociceptive inhibition, along with aspects of nociceptive transmission ‘wind up’, allowed for a complete resolution of chronic pain and demonstrates the dynamic ability of the central nervous system to alter it’s function in response to environmental stimuli.


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[10] [11] [12] [13] [14] [15] [16] [17] [18]

[19] [20] [21] [22] [23]

Harzy T, Benbouazza K, Amine B, Rahmouni R, Hajjaj-Hassouni N. An unusual case of osteomalacia as the presenting feature of coeliac disease. Rheumatol Int. 2005;26:90-91. Celilglu C, Karabiber H, Selimoglu MA. Atypical presentations of celiac disease. Turkish J Pediatr. 2011;53:241-249. Hadjivassiliou M, Chattopadhyay AK, Grunewald RA, et al. Myopathy associated with gluten sensitivity. Muscle Nerve. 2007;35:443-450. Uygur-Bayramicli O, Ozel AM. Celiac disease is associated with neurological syndromes. Dig Dis Sci. 2011;56:1587-1588. Djuric Z, Kamenov B, Katic V. Celiac disease manifested by polyneuropathy and swollen ankles. World J Gastroenterol. 2007;13(18):2636-2638. Jackson JR, Eaton WW, Cascella NG, et al. Neurological and psychiatric manifestations of celiac disease and gluten sensitivity. Psychiatr Q. 2011 Aug 30 [Epub ahead of print]. Stipic J, Perkovic Z, Crnek-Kunstelj V, et al. Neurological manifestations in adult celiac disease: case report. Acta Clin Croat. 2002;41:41-44. Wills A, Hovell CJ. Neurological complications of enteric disease. Gut. 1996;39:501-504. Hadjivassiliou, M., Aeschlimann, D., Grünewald, R.A., et al. GAD antibody associated neurological illness and its relationship to gluten sensitivity. Acta Neurol Scand, 2010; 123(3):175-180. Hadjivassiliou M, Grunewald RA, Davies-Jones GAB. Gluten sensitivity as a neurological illness. J Neurol Neurosurg Psychiatry. 2002;72:560-563. Hadjivassiliou M, Grunewald RA, Kandler RH, et al. Neuropathy associated with gluten sensitivity. J Neurol Neurosurg Psychiatry. 2006;77:1262-1266. Wong M, Scally J, Watson K, Best J. Proximal myopathy and bone pain as the presenting features of celiac disease. Ann Rheum Dis. 2002;61:85-96. Kozanoglu E, Basaran S, Goncu MK. Proximal myopathy as an unusual presenting feature of celiac disease. Clin Rheumatol. 2005;24:76-78. Hadjivassiliou M, Sanders DS, Grunewald RA, et al. Gluten sensitivity: from gut to brain. Lancet Neurol. 2010;9:318-330. Hadjivassiliou M, Chattopadhyay AK, Grunewald RA, et al. Neuromuscluar disorder as a presenting feature of celiac disease. J Neurol Neurosurg Psychiatry. 1997;63:770-775. Selva-O’Callaghan A, Casellas F, de Torres I, et al. Celiac disease and antibodies associated with celiac disease in patients with inflammatory myopathy. Muscle Nerve. 2007;35:49-54. Davidson A, Diamond B. Autoimmune Diseases. N Eng J Med. 2001;245(5):340-350. Orbach H, Amiati N, Barzilai O, et al. Autoantibody screen in inflammatory myopathies high prevalence of antibodies to gliadin. Contemporary Challenges in Autoimmunity: Ann N.Y. Acad. Sci. 2009;1173:174-179. Tozzoli R. The diagnostic role of autoantibodies in the prediction of organ-specific autoimmune diseases. Clin Chem Lab Med. 2008;46(5):577-587. Hadjivassiliou M, Williamson CA, Woodroofe N. The immunology of gluten sensitivity: beyond the gut. Trends Immunol. 2004;25(11):578-582. Lebwohl B, Green P. Screening for Celiac disease. N Engl J MEd. 2003;349:1673-1674. Lindqvist U, Rudsander A, Boström A, Nilsson B, Michaëlson G. IgA antibodies to gliadin and Coeliac disease in psoriatic arthritis. Rheumatology. 2002;41(1):31-37. Dalakas MC. Review: An update on inflammatory and autoimmune myopathies. Neuropathology and Applied Neurobiology. 2001;37:226-242.

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[24] Olsen NJ, Prather H, Li QZ, Burns DK. Autoantibody profiles in two patients with nonautoimmune muscle disease implicate a role for gliadin autoreactivity. Nueromusc Disorders. 2010;188-191. [25] Gendek EG, Kedziora J, Gendek-Kubiak H. Can tissue transglutaminase be a marker of idiopathic inflammatory myopathies? Immunology Letters. 2005;97:245-249. [26] Ghirardello A, Zampieri S, Tarricone E, et al. Clinical implications of autoantibody screening in patients with autoimmune myositis. Autoimmunity. 2006;39(3):217-221. [27] Choi YC, Kim TS, Kim SY. Increase in Transglutaminase 2 in idiopathic inflammatory myopathies. Eur Neurol. 2004;51:10-14. [28] Mastiglia FL, Garlepp MJ, Phillips BA, Zilko PJ. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve. 2003;27:407-425. [29] Kim SY, Jeitner TM, Steinert PM. Transglutaminases in disease. Neurochem Int. 2002;40(1):85-103. [30] Dalakas MC. Inflammatory disorders of muscle: progress in polymyositis, dermatomyositis and inclusion body myositis. Curr Opin Neurol. 2004;17:561-567. [31] Briani C, Doria A, Ruggero S, et al. Antibodies to muscle and ganglionic acetylcholine receptors (AchR) in celiac disease. Autoimmunity. 2008;41(1):100-104. [32] Tengah DSNAP, Willis AJ. Questions and answers about the neurology of gluten sensitivity. Pract Neurol. 2003;3:354-357. [33] Dalakas MC. Autoimmune muscular pathologies. Neurol Sci. 2004;25:S7-S8. [34] Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat. Rev. Neurol. 2011;7:343-354. [35] Mastaglia FL. Treatment of autoimmune inflammatory myopathies. Curr Opin Neurol. 2000;13:507-509. [36] Kleopas A, Kleopa MD, Kyriacou K, et al. Reversible inflammatory and vacuolar myopathy with vitamin E deficiency in celiac disease. Muscle Nerve. 2005;31:260-265. [37] Herrero JF, Laird J.M.A., Lopez-Garcia J. A. Wind-up of spinal cord neurones and pain sensation: much ado about something? Prog Neurobiol, 2000;61:169-203. [38] Arendt-Nielsen L, Peterson-Felix S. Wind-up and neuroplasticity: is there a correlation to clinical pain? Eur J Anaesth Supp, 1995;10:1-7. [39] Hadjivassiliou M, Grunewald RA. The neurology of gluten sensitivity: science vs. conviction. Pract Neurol. 2004;4:124-126.

Received: December 16 2013 Modified: January 8 2014 Accepted January 23 2014.



EAR INSUFFLATION PRODUCES RAPID AND SIGNIFICANT RELIEF OF TRIGEMINAL NEURALGIA David B. Sullivan Private Practice, Mechanicsburg, PA USA

ABSTRACT Trigeminal neuralgia is a condition of severe pain in the face which can cause tremendous suffering to an individual. Although there are various medical, surgical and conservative options available to treat trigeminal neuralgia, each approach offers a unique set of strengths and limitations. In an attempt to provide additional avenues of therapeutic efficacy to reduce the burden of this disease, we have investigated an application which could potentially alleviate the pain and suffering associated with trigeminal neuralgia. Herein, we describe how pneumatic insufflation to the external auditory meatus results in rapid and significant pain relief in trigeminal neuralgia. This is the first instance within the neurological literature that ear insufflation has been documented as a modulator of trigeminal neuralgia pain.

Keywords: trigeminal neuralgia, trigeminal nerve, auriculotemporal nerve, ear insufflation, neurorehabilitation, clinical application

INTRODUCTION It has recently been demonstrated that pneumatic insufflation of the ear can produce rapid and profound improvement in acute migraine headaches [1]. This application of ear insufflation had historically been used only as a diagnostic tool, but recent evidence suggests that it may serve as an effective therapeutic technique in the treatment of neurologically mediated pain. The goal of the current investigation was to explore how ear insufflation could aid in the modulation of trigeminal neuralgia pain. Trigeminal neuralgia (TN) is typically characterized by sudden and severe, sharp electric-type, neuropathic pain [2-4]. The pain attacks are usually paroxysmal and short lasting but they are generally recurrent and can become a severe burden to the individual [5]. In fact, TN has become known as the ‘suicide disease’ because of the increased rate of suicide attempts associated with this condition [6]. The prevalence rate of TN is approximately 4.5 per 100,000 individuals and usually occurs in patients between the ages of 50 and 70 [2,3]. There are some known factors understood to cause trigeminal neuralgia, including mechanical compression, demyelination and trauma [7], but in most cases, there is no clear etiology [8,9]. Because of the age-related aspects of the typically affected 

Correspondence: Dr. David B. Sullivan,1001 S. Market St. Suite B, Mechanicsburg, PA 17055 USA Email: [email protected]

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populations, and the often idiopathic nature of the disorder, successful management and treatment of trigeminal neuralgia can often times become difficult and challenging [10]. Various medical and surgical therapies are available to treat the pain of trigeminal neuralgia. Common pharmaceutical interventions include carbamezapine, baclofen, lamotrigine and gabapentin [11,12]. First line drugs such as carbamezapine can provide significant pain relief for some individuals and have a relatively mild side effect profile, whereas second line drugs have lower rates of efficacy and a moderate proportion of sufferers will fail to achieve adequate resolution will pharmaceutical therapy alone [13,14]. For those cases where pharmaceutical management fails, surgical interventions include microvascular decompression at a site of neurovascular irritation, as well as ablative and destructive methods targeting various points at the trigeminal nucleus or [15-18]. Of the most common surgical techniques, decompression at an identified site of trigeminal irritation has shown to produce the most successful long-term outcomes and has a low morbidity rate. On the other hand, ablative and destructive techniques, either peripheral or central, have less efficacious pain relief and are associated with great morbidity, the greatest being permanent sensory loss. There are also a variety of other pain control methods available for neuropathic pain. Of particular interest to the current investigation is that substantial pain relief can be achieved with nerve stimulation, applied either peripherally [19-21], or centrally [12,22]. These techniques do carry significant risks for the patient, however. An important development in the treatment of trigeminal neuralgia pain would be an effective pain modulation technique with a very low side-effect profile, whose application could exert beneficial pain reduction while leaving the patient’s anatomy intact and unaltered. The concept of pain modulation though specifically directed afferent activation serves as the basis for the therapeutic application of ear insufflation. Additionally, we offer the hypothesis that pneumatic ear insufflation can produce neuropathic pain modulation by stimulating tympanic membrane receptors whose afferent projections modulate activity in trigeminal nerve and nucleus structures.

CASE REPORT In June 2013, Amy, a 30 year old female, presented to the chiropractic clinic for treatment of severe trigeminal neuralgia of 12 months duration. The pertinent history of her present illness is as follows. Amy had been in good health, with no significant medical history before being injured in an automobile accident in July 2006. She sustained severe bodily injuries to her left side, including head trauma and spinal fractures. She spent approximately 8 days in a hospital before she was stabilized and able to return home. She subsequently developed severe spine and body pain and headaches. She was prescribed multiple pharmaceutical agents for pain management without success. Ultimately, her pain seem to subside on it’s own by the middle of 2008. At that time, she only experienced intermittent low back pain and occasional headaches. Amy was unfortunately involved in a second motor vehicle accident in March 2013. She was impacted again on the left side of her body. She spent two days in a hospital for cautious observation, but not due to severe or life threatening injury. This incident resulted in a significant increase in daily headaches, and she started to develop facial pain in front of her left ear, across the left maxillary area, and the maxillary teeth on left side (the V2 or 2nd trigeminal branch distribution). Because her facial pain continued to worsen at a rapid pace, her family physician’s assistant, an otorhinolaryngologist, a dentist and an oral surgeon saw her within a short period of time through March and April 2013. She was prescribed various medications for relief without success. At this time, her average daily pain levels were approaching 10/10 on a Verbal Analog Scale (VAS). After follow-up with her primary care physician, it was decided to use gabapentin as a diagnostic trial of care. Within approximately 24

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hours, Amy’s pain levels were dramatically reduced. She continued to use gabapentin for a few weeks but was dismayed to find that the side effects were impacting her life more significantly than the pain. Although she had significantly less pain, she became basically unable to function in her daily life so she decided to discontinue the gabapentin. As expected, discontinuation of gabapentin brought her back to normal functioning, but her pain levels returned to the same levels they had been previously. She described her facial pain as a constant 10/10 on VAS with no periods of reprieve from the pain. She was then prescribed pregabalin 150 mg/day in order to bring relief with fewer side effects. She experienced fewer side effects on this drug as well as less frequent stabs of excruciating pain. The reduction of her constant daily facial pain however, was not substantial; Amy reported that her pain level had only dropped to a constant 6/10 on VAS after 2 months of pregabalin therapy. She presented to the chiropractic clinic in late June, 2013 for assistance, and with her consent, it was decided to attempt this experimental treatment.

TREATMENT Insufflation was performed at the left ear using a pneumatic otoscope outfitted with a insufflation bulb and specula. At each office visit, the therapy was performed to the left ear for approximately 45 seconds per treatment. The insufflation treatment was performed in three successive rounds with short rest periods in between, totaling approximately four minutes during the course of an office visit. Pain levels were monitored and recorded before treatment, between subsequent rounds of treatment, and after treatment.

OUTCOME AND FOLLOW-UP After the first two visits, she could feel that there was a definite change in her symptoms. Although the pain was not significantly different, she noted that the left side of her face felt like it was ‘starting to come back to life’. There was a dramatic event on the third treatment though, where she noted that there was a profound and almost immediate reduction in her pain level. She reported that her pain level dropped from a 9/10 to a 4/10 on VAS with less frequent, brief surges to 7/10, within approximately 5 minutes of treatment. She was treated at a frequency of three times per week initially, and was seen less frequently as her daily pain levels continued to diminish. Because her pain levels were improving, she incrementally decreased her daily pregabalin use during the course of treatment. After approximately four weeks of insufflation therapy she noted that her baseline/constant pain continued to decrease, from the initial 7/10 to a 1/10 on VAS, the frequency of the brief surges of sharp pain were reduced by approximately 90% and her daily headaches were completely resolved. With physician approval, she had tapered her pregabalin to 50 mg/day by this time, and intended to discontinue it completely.

DISCUSSION The concept that various modes of external environmental stimuli can alter neurological function is not new [23-26]. The application of insufflation however, is a novel therapeutic strategy, which could potentially serve as an important tool in the arsenal of neuropathic pain management strategies. It is likely that ear insufflation exerts its effects by stimulating pressure-sensitive mechanoreceptors on the tympanic membrane whose afferent fibers coalesce into the

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auriculotemporal nerve (ATN) [27-30]. The ATN subsequently merges with the mandibular division of the trigeminal nerve (V3). Somatosensory afferent fibers enter the pons and synapse at various levels of the main sensory nucleus and the spinal trigeminal nucleus [31,32]. It is in these locations that both pre- and post-synaptic pain modulation can occur. It has been documented that GABAergic nociceptive gating occurs in the various laminae of the dorsal horn [33], mesencephalic [34] and trigeminal nucleus caudalis [35-37] and can result in down-regulation of trigeminothalamic projection fibers from craniofacial structures. The majority of these trigeminothalamic projection fibers ascend through the contralateral ventral trigeminothalamic tract, synapse in the ventral posteromedial nucleus of the thalamus where third-order neurons ascend and terminate in the somatosensory cortex [31,38]. If this pathway could be down-regulated to a substantial degree through the use of ear insufflation, this would represent a tremendous potential benefit for trigeminal neuralgia sufferers, because it carries an extremely low side effect profile while leaving the individual’s anatomy intact and unaltered. The observed effects of insufflation therapy are obviously confounded in this case, because she was concurrently using pregabalin. Although her pain levels had not changed significantly after three months of use, and she was able to decrease her dosage while still experiencing significant improvements in her pain, we cannot fully attribute the observed changes to insufflation therapy alone. Insufflation therapy for trigeminal neuralgia will be more fully understood when applied to a non-medicated sufferer.

CONCLUSION This case suggests that pneumatic insufflation at the ear can have a significant impact on the pain associated with trigeminal neuralgia. More studies will be needed to determine: 1, the exact mechanisms behind the observed effects, 2, the extent to which insufflation can produce long-term pain reduction, and 3, criteria for patient selection, and 4, the potential for symptomatic change in a non-medicated individual. Indeed, the understanding of insufflation therapy is in its infancy and will need to be fully elucidated in order to serve patients at the highest level. This case, however, is the first instance within the neurological literature that ear insufflation has been documented as a modulator of trigeminal neuralgia symptomatology.

ACKNOWLEDGMENT Special thanks to Corey W. Kirshner DC, FIACA for performing this investigative therapy under the direction of, and in collaboration with the author.

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Sullivan D. Ear insufflation as a novel therapy which produces rapid relief of migraine headache - a case series. Funct Neurol Rehabil Ergon. 2013;3(1):93-107. Ibrahim S. Trigeminal neuralgia: diagnostic criteria, clinical aspects and treatment outcomes. A retrospective study. Gerodontology. 2012 (Epub ahead of print). Toda K. Trigeminal Neuralgia - symptoms, diagnosis, classification, and related disorders. Oral Sci Int. 2007;4(1):1-9. Zakrzewska J, Linsky M. Trigeminal neuralgia. Clin Evid. 2009;3:1-22.

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[28] Nagai T, Tono T. Encapsulated nerve corpuscles in the human tympanic membrane. Arch Otorhinolaryngol. 1989;246:169-172. [29] Nagai T. Innervation of the tympanic membrane. Acta Otorhinolaryngol Belg. 1995;49(2):11720. [30] Nagai T. Innervation of the tympanic membrane. Acta Oto-Rhino-Laryngologica Belg. 1995;49:117-120. [31] Schuenke M, Schulte E, Schumacher U, Ross L. Thiene Atlas of Anatomy: Head and Neuroanatomy. 1st ed. New York, NY: Thieme;2007:66. [32] Go JL, Kim PE, Zee CS. The trigeminal nerve. Seminars in Ultrasound, CT and MRI. 2001. 22(6):502-20. [33] Broman J. Neurotransmitters in subcortical somatosensory pathways. Anat Embryol. 1994;189:181-214. [34] Hayar A, Poulter MO, Pelkey K, Feltz P, Marshall KC. Mesencephalic trigeminal neuron responses to gamma-aminobutyric acid. Brain Res. 1997;753(1):120-7. [35] DiFiglia M, Aronin N. Synaptic interactions between GABAergic neurons and trigeminothalamic cells in the rat trigeminal nucleus caudalis. Synapse. 1990;6(4):358-63. [36] Almond JR, Westrum LE, Henry MA. Post-embedding immunogold labeling of gammaaminoburyric acid in lamina II of the spinal trigeminal subnucleus pars caudalis: I. A qualitative study. Synapse. 1996;24(1):39-47. [37] Li JL, Wu SX, Tomioka R, Nakamura K, KanekoT, Mizuno N. Efferent and afferent connections of GABAergic neurons in the supratrigeminal and the intertrigeminal regions. An immunohistochemical tract-tracing study in the GAD67-GFP knock-in mouse. Neurosci Res. 2005;51(1):81-91. [38] Stewart WA, King RB. Fiber projections from the nucleus caudalis of the spinal trigeminal nucleus. J Comp Neurol. 1963;121(2):271-286.

Received: June 21, 2013 Revised December: 24 2013 Accepted January 12, 2014.

SCIENTIFIC PAPER



MEGACITY AIR POLLUTION AND THE IMPACT ON THE CENTRAL NERVOUS SYSTEM Lilian Calderón-Garcidueñas,1, Ana Calderón-Garcidueñas2, and Ricardo Torres-Jardón3 1

Center for Structural and Functional Neurosciences, University of Montana, Missoula, Montana, USA 2 Instituto de Medicina Forense, Universidad Veracruzana, Boca del Río, México 3 Centro de Ciencias de la Atmósfera, Universidad Nacional Autónoma de México, Mexico City, Mexico

ABSTRACT The chronic health effects associated with sustained exposures to significant concentrations of air pollutants are critical for millions of people around the world and 29 million people living in the Mexico City Metropolitan Area (MCMA). Urban outdoor pollution is a global public health problem particularly severe in megacities and in underdeveloped countries. Fine and ultrafine particulate matter broadly defined by aerodynamic diameter (