Neuropsychology of Post-Polio Syndrome

Neuropsychology of Post-Polio Syndrome

Anales de la Academia de Nueva York de Ciencias.


Volumen 753,

 El Síndrome Post-Polio: Avances en la patogénesis y tratamiento del as páginas 103 a 110, mayo de 1995

1. JORDAN GRAFMAN^1,†, 
 
2. KIM CLARK¹,
 
3. DAVID RICHARDSON¹, 
 
4. STEVE DINSMORE², 
 
5. DANIEL STEIN², 
 
6. MARINOS C. DALAKAS²Article first published online: 17 DEC 2006 DOI: 10.1111/j.1749-6632.1995.tb27536.x

The Post-Polio Syndrome As an 

Evolved Clinical Entity

Definition and Clinical Description

1. MARINOS C. DALAKAS

Article first published online: 17 DEC 2006

DOI: 10.1111/j.1749-6632.1995.tb27532.x

Post-polio syndrome (PPS) refers to the new neuromuscular symptoms that occur at least 15 years after stability in patients with prior acute paralytic poliomyelitis. They include: (1) new muscle weakness and atrophy in the limbs, the bulbar or the respiratory muscles [post-poliomyelitis muscular atrophy (PPMA)] and (2) excessive muscle fatigue and diminished physical endurance. PPS is a clinical diagnosis that requires exclusion of all other medical, neurological, orthopedic or psychiatric diseases that could explain the cause of the new symptoms. Routine electromyography is useful to confirm chronic and ongoing denervation and exclude neuropathies. Muscle biopsy, single fiber electromyography (EMG), macro-EMG, serum antibody titers to polio virus, and spinal fluid studies are very useful research tools but they are rarely needed to establish the clinical diagnosis. PPS is a slowly progressive phenomenon with periods of stability that vary from 3 to 10 years. Current evidence indicates that PPS is the evolution of a subclinically ongoing motor neuron dysfunction that begins after the time of the acute polio. It is clinically manifested as PPS when the well-compensated reinnervating process crosses a critical threshold beyond which the remaining motor neurons cannot maintain the innervation to all the muscle fibers within their motor unit territory.

.  of the New York Academy of Sciences

Volume 753, The Post-Polio Syndrome: Advances in the Pathogenesis and Treatmentpages 68–80, May 1995

THE NEUROPSYCHOLOGY OF POST-POLIO FATIGUE

Richard L. Bruno, Ph.D., Thomas Galski, Ph.D., John DeLuca, Ph.D.

Bruno RL, Galski T, DeLuca J. The neuropsychology of post-polio fatigue.

 Archives of Physical Medicine and Rehabilitation, 1993; 74: 1061-1065.

Lincolnshire Post-Polio Library copy by arrangement 

with the Harvest Center Library

http://www.ott.zynet.co.uk/polio/lincolnshire/library/harvest/neuropsy.html

J Rehabil Med 2008; 40: 709–714

ORIGINAL REPORT

VITALITY AMoNG SWEdISH PATIENTS WITH PoST-PoLIo: A PHYSIoLoGICAL PHENoMENoN*http://jrm.medicaljournals.se/article/pdf/10.2340/16501977-0253

Gunilla Östlund, MSci1, Åke Wahlin, PhD2, Katharina S. Sunnerhagen, MD, PhD3,4 and Kristian Borg, MD, PhD1

From the 1Divison of Rehabilitation Medicine, Karolinska Institutet, Department of Clinical Sciences at Danderyd Hospital, 2Department of Psychology, Stockholm University, Stockholm, 3Institute for Neuroscience and Physiology, Section for Clinical Neuroscience and Rehabilitation, Göteborg University, Göteborg, Sweden and 4Sunnaas Rehabilitation Hospital, Faculty of Medicine, University of Oslo, Oslo, Norway

Objective: To evaluate vitality and fatigue in post-polio pa- tients, and the relative contributions of physiological and psychological parameters to the level of vitality. Design: Multi-centre study.

Subjects: One hundred and forty-three patients with post- polio syndrome.

Methods: Inventories of background, quality of life, fatigue and sleep quality were used. Pain was evaluated using a visual analogue scale. Descriptive statistics and correlations were used for all selected parameters. Hierarchical regres- sion models were constructed to examine predictors of varia- tions in vitality, pain, reduced activity and physical fatigue.

Results: General fatigue accounted for 68% of the variation in vitality. Of this, 91% was accounted for by physiologi- cal indicators. After controlling for age, physiological para- meters accounted for 56.6% and 25%, if entered before and after the psychological parameters, respectively. The impact of the psychological parameters decreased after accounting for the physiological parameters. Physical fatigue, age and sleep quality were associated with variation in pain. Body mass index, pain and sleep quality accounted for differences in reduced activity and physical fatigue.

Conclusion: Vitality in post-polio patients depends on physio- logical parameters. Mental fatigue is not a prominent pre- dictor. Subgroups with or without fatigue, independent of age, need further study.

Key words: post-polio, fatigue, vitality, quality of life. J Rehabil Med 2008; 40: 709–714

Correspondence address: Gunilla Östlund, Department of Rehabilitation Medicine, Danderyd University Hospital, Building 39, 3rd Floor, SE-182 88 Stockholm, Sweden. E-mail: Gunilla.Ostlund@ki.se

Submitted September 21, 2007; accepted May 29, 2008

INTRoduCTIoN

Poliomyelitis leads to muscle weakness due to destruction of the anterior horn cells. After an initial recovery there is a phase

*This article has been fully handled by one of the Associate Editors, who has made the decision for acceptance, as it originates from the institute where the Editor-in-Chief is active.

© 2008 The Authors. doi: 10.2340/16501977-0253 Journal Compilation © 2008 Foundation of Rehabilitation Information.

of functional stability that usually lasts from 10 to 40 years. during this phase the life circumstances of polio survivors do not differ much from the general population with respect to work and family situation (1). However, after the stable phase deterioration may occur; a condition termed post-polio syndrome (PPS) (2). The most commonly reported symptoms of PPS are increased muscle weakness, fatigue and pain in the muscles and joints. The last epidemic of polio in Sweden was in 1953 when more than 5000 people contracted poliomyelitis. Today, the prevalence of polio-affected individuals in Sweden is estimated to be 186/100,000 (3). Reported estimates of polio survivors eventually developing PPS vary from 20% to 68% (2, 4). Thus, the majority of polio survivors in Sweden are now middle-aged or older, and consequently at risk of developing PPS. Risk factors for developing PPS include time since the acute polio infection (5), age at presentation of symptoms, muscle pain at exercise, recent weight gain, joint pain (6) and female gender.

During the last decade, increasing research interest has fo- cused on fatigue in patients with PPS (7). Jubelt & Agre (8) re- ported generalized fatigue as one of the most common symptoms in PPS. Mental, as well as physical, fatigue has been reported by both Bruno et al. (9) and Schanke & Stanghelle (10).

Interestingly, and related to mental fatigue, there are con- tradicting reports regarding cognitive dysfunction in patients with PPS. Difficulties with attention, word finding, maintaining wakefulness and ability to think clearly have been reported by Bruno et al. (11). However, in most other studies cognitive function is reported to be unaffected by mental fatigue (12, 13). Furthermore, fatigued polio survivors are reported to have more mental health problems than controls or polio survivors without severe fatigue (5). In a study by Conrady et al. (14) patients, both at a post polio-clinic and in a post-polio support group, experienced significantly elevated levels of psychologi- cal distress, such as somatization and depression. Gonzalez et al. (15) reported an increase in cytokines in the cerebrospinal fluid of patients with PPS, indicating an inflammatory proc- ess.

The inflammatory processes were down-modulated by treatment with intravenous immunoglobulin followed by a clinical effect, especially on vitality, as evaluated by means of Short Form 36 (SF-36). This indicates that vitality has a central role in PPS that may be improved by means of phar- macological treatment. The subjective experience of vitality

ISSN 1650-1977J Rehabil Med 40

Neuropsychiatry, Neuropsychology, & Behavioral Neurology:

ORIGINAL ARTICLES: PDF Only

A Neuropsychological Study of the Postpolio Syndrome: Support for Depression Without Neuropsychological Impairment

Hazendonk, Kim M. B.BSc.; Crowe, Simon F. Ph.D.

Abstract

Objective: This study aimed to examine cognitive functioning in postpolio syndrome (PPS) after controlling for the effects of depression and illness behavior.

Background: Few studies have investigated the possible cognitive sequelae of PPS, despite widespread documented subjective complaints of "mental fatigue."

Method: A total of 23 PPS sufferers, 20 polio survivors without PPS, and 22 matched controls were compared using the Beck Depression Inventory-II; the Illness Behaviour Questionnaire; a chronic fatigue syndrome symptom checklist; and several measures of memory, attention, and concentration, including the Brown-Petersen Task, Stroop Test, Austin Maze, California Verbal Learning Test, Trail Making Test, Controlled Oral Word Association Test, and Symbol-Digit Modalities Test.

Results: In those participants with a medically confirmed diagnosis of PPS, there was a significantly higher level of depressive and hypochondriacal symptomatology as compared with the other two groups. Nevertheless, no significant differences existed between the three groups on neuropsychological measures.

Conclusions: These results indicate that the attention and memory difficulties reported by PPS sufferers may be linked to the physical or psychological manifestations of the illness rather than to objective decrements in cognitive performance. (NNBN 2000;13:112-118)

http://translate.google.com/translate?hl=es&sl=en&u=http://journals.lww.com/cogbehavneurol/abstract/2000/13020/a_neuropsychological_study_of_the_postpolio.5.aspx&ei=gvalTbr2HYHQsAOvyMn5DA&sa=X&oi=translate&ct=result&resnum=1&ved=0CB8Q7gEwADgK&prev=/search%3Fq%3DNeuropsychology%2Bof%2BPost-Polio%2BSyndrome%26start%3D10%26num%3D10%26hl%3Des%26newwindow%3D1%26client%3Dsafari%26sa%3DN%26rls%3Den%26prmd%3Divnsb

Neuropsiquiatría, Neuropsicología, Neurología y del Comportamiento:

ARTÍCULOS ORIGINALES: Sólo PDF

Un estudio neuropsicológico del síndrome postpolio: Apoyo a la depresión sin deterioro neuropsicológico

Hazendonk, MBBSc Kim;. Crowe, Simon F. Ph D..

Resumen

Objetivo: El presente estudio tuvo como objetivo examinar el funcionamiento cognitivo en el síndrome postpolio (SPP) después de controlar por los efectos de la depresión y el comportamiento de la enfermedad.

Antecedentes: Pocos estudios han investigado las posibles secuelas cognitivas del SPP, a pesar de amplia documentado quejas subjetivas de "fatiga mental".

Método: Un total de 23 enfermos de PPS, 20 supervivientes de la polio sin PPS, y 22 controles emparejados se compararon mediante el Inventario de Depresión de Beck-II, la enfermedad Cuestionario de Comportamiento, un síndrome de fatiga crónica lista de síntomas, y varias medidas de la memoria, la atención y concentración, incluyendo la tarea de Brown-Petersen, prueba de Stroop, Laberinto de Austin, California Verbal Learning Test, Trail Making Test, controlada oral Palabra Test de Asociación, y el símbolo dígitos Modalidades de prueba.

Resultados: En los participantes con un diagnóstico médico confirmado de PPS, hubo un nivel significativamente mayor de depresión y sintomatología hipocondríaca, en comparación con los otros dos grupos. Sin embargo, no existen diferencias significativas entre los tres grupos en las medidas neuropsicológicas.

Conclusiones: Estos resultados indican que las dificultades de atención y la memoria reportado por las víctimas del PPS puede estar relacionado con las manifestaciones físicas o psicológicas de la enfermedad en lugar de decrementos objetivo en el rendimiento cognitivo. (NNBN 2000, 13:112-118)

Editor (s): 

Barry Gordon, MD, PhD 

Post Polio Litaff, Association A.C _APPLAC Mexico

History of polio

Image copyrightBBC

Image captionPoliomyelitis has existed as long as human society, but became a major public health issue in late Victorian times with major epidemics in Europe and the United States. The disease, which causes spinal and respiratory paralysis, can kill and remains incurable but vaccines have assisted in its almost total eradication today.

Image copyrightGETTY IMAGES

Image captionThis Egyptian stele (an upright stone carving) dating from 1403-1365 BC shows a priest with a walking stick and foot, deformities characteristic of polio. The disease was given its first clinical description in 1789 by the British physician Michael Underwood, and recognised as a condition by Jakob Heine in 1840. The first modern epidemics were fuelled by the growth of cities after the industrial revolution.

Image copyrightOTHER

Image captionIn 1916, New York experienced the first large epidemic, with more than 9,000 cases and 2,343 deaths. The 1916 toll nationwide was 27,000 cases and 6,000 deaths. Children were particularly affected; the image shows child patients suffering from eye paralysis. Major outbreaks became more frequent during the century: in 1952, the US saw a record 57,628 cases.

Image copyrightGETTY IMAGES

Image captionIn 1928, Philip Drinker and Louie Shaw developed the "iron lung" to save the lives of those left paralysed by polio and unable to breathe. Most patients would spend around two weeks in the device, but those left permanently paralysed faced a lifetime of confinement. By 1939, around 1,000 were in use in the US. Today, the iron lung is all but gone, made redundant by vaccinations and modern mechanical ventilators.

Image copyrightGETTY IMAGES

Image captionA major breakthrough came in 1952 when Dr Jonas Salk (L) began to develop the first effective vaccine against polio. Mass public vaccination programmes followed and had an immediate effect; in the US alone cases fell from 35,000 in 1953 to 5,300 in 1957. In 1961, Albert Sabin (R) pioneered the more easily administered oral polio vaccine (OPV).

Image copyrightGETTY IMAGES

Image captionDespite the availability of vaccines polio remained a threat, with 707 acute cases and 79 deaths in the UK as late as 1961. In 1962, Britain switched to Sabin's OPV vaccine, in line with most countries in the developed world. There have been no domestically acquired cases of the disease in the UK since 1982.

Image captionBy 1988, polio had disappeared from the US, UK, Australia and much of Europe but remained prevalent in more than 125 countries. The same year, the World Health Assembly adopted a resolution to eradicate the disease completely by the year 2000.

Image copyrightBBC

Image captionThe WHO Americas region was certified polio free in 1994, with the last wild case recorded in the Western Pacific region (which includes China) in 1997. A further landmark came in 2002, when the WHO certified the European region polio-free.

Image captionIn 2012, Polio remained officially endemic in four countries - Afghanistan, Nigeria, Pakistan and India, which was on the verge of being removed from the list having not had a case since January 2011. Despite so much progress, polio remained a risk with virus from Pakistan re-infecting China in 2011, which had been polio free for more than a decade.

Image captionChina returned to its polio-free status, with no recorded incidents after 2011

Image captionIn 2015, polio remains endemic in only two countries - Pakistan and Afghanistan. No new cases have been reported in Africa for the past year.

Post Polio Litaff, Association A.C _APPLAC Mexico