2 oct. 2019

Morphologic changes in the muscles of patients with post-poliomyelitis neuromuscular symptoms Marinos C. Dalakas,


Morphologic changes in the muscles of patients with post poliomyelitis neuromuscular symptoms
  1. Marinos C. Dalakas, MD
+Author Affiliations
  1. National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD. Presented in part at the thirty-eighth annual meeting of the American Academy of Neurology, New Orleans, LA, April 1986.

ABSTRACT

Thirty-five muscle biopsies were performed on 27 patients with postpoliomyelitis progressive muscular atrophy (PPMA) (8 patients had two biopsies) and 5 asymptomatic postpolio patients in an attempt to define diagnostic criteria for the newly weakening muscles and to provide insights into the mechanism of the disease. PPMA muscles that had been left weak since the original illness showed a mixture of myopathy with new and old denervation including group atrophy and nuclear clumps. Fully recovered or originally spared PPMA muscles showed signs of reinnervation and recent denervation. Perivascular or interstitial inflammatory cells (predominantly lymphocytes unrelated to phagocytosis) were noted in 40% of all the PPMA biopsies. It is concluded that (1) postpolio muscle biopsies show a spectrum of morphologic changes that depend on whether the biopsied muscle was originally affected and hadfully orpartiallyrecovered, (2) the newly weakened muscles show signs of recent denervation, and (3) the presence of reinnervation in the asymptomatic muscles and the patterns of recent and old denervation in PPMA muscles provide information regarding the degree of compensation of the surviving motor neurons and their apparent failure for further reinnervation via axonal sprouting.

FOOTNOTES

  • Address correspondence and reprint requests to Dr. Dalakas, NINCDS, NIH, Building 10,



Post Polio Litaff, Association A.C _APPLAC Mexico

The Polio Crusade



In the summer of 1950 fear gripped the residents of Wytheville, Virginia. Movie theaters shut down, baseball games were cancelled and panicky parents kept their children indoors — anything to keep them safe from an invisible invader. Outsiders sped through town with their windows rolled up and bandanas covering their faces. The ones who couldn’t escape the perpetrator were left paralyzed, and some died in the wake of the devastating and contagious virus. Polio had struck in Wytheville. The town was in the midst of a full-blown epidemic. That year alone, more than 33,000 Americans fell victim — half of them under the age of ten.
AMERICAN EXPERIENCE presents The Polio Crusade, a one-hour documentary from filmmaker 
Sarah Colt (Geronimo, RFK) that interweaves the personal accounts of polio survivors with the story of an 
ardent crusader who tirelessly fought on their behalf while scientists raced to eradicate this dreaded disease. 
Based in part on the Pulitzer Prize-winning book Polio: An American Story by David Oshinsky, 
The Polio Crusade features interviews with historians, scientists, polio survivors, and the only surviving
 scientist from the core research team that developed the Salk vaccine, Julius Youngner.
“Daddy and Mama took everything Sonny owned, all of his clothes, his bed, his chest of drawers, and he had a 
fabulous comic book collection. They took everything out to the middle of the garden and they made a pile and burned 
everything he owned. They were told to do that, so we would not get it,” recalls Anne Crockett-Stark, who was just seven 
years old when her brother fell ill during Wytheville’s polio epidemic.
The victims found an unlikely champion in New York lawyer Basil O’Connor. His innovative public relations campaign
 transformed polio — a devastating, but relatively rare disease — into a nationwide cause. 
He rallied the American public to fight a war against polio.
In 1928, O’Connor inherited the leadership of a polio rehabilitation center from his law partner — 
future president Franklin Delano Roosevelt. As the nation reeled from the economic trauma of the Great Depression,
O’Connor faced a pressing challenge: funding. He took an unlikely new approach, turning to the public for donations. 
Rather than relying on wealthy philanthropists, O’Connor asked every person to contribute what small change they could. 
His pleas struck a chord with Americans — within days, envelopes stuffed with change flooded the White House mailroom, 
and “The March of Dimes” was born.
Basil O’Connor made a pledge to provide care for every polio patient in America, and to invest in scientific research
 to create a vaccine that would end the disease forever. One young researcher caught O’Connor’s attention, a scientist 
whose sense of urgency for a vaccine matched his own: Dr. Jonas Salk, the director of the virus research program at the
 University of Pittsburgh.
During World War II, Jonas Salk was part of a government effort to develop an influenza vaccine.
 He believed he could apply the same killed virus approach to polio. But established researchers scoffed at 
Salk’s theory and dismissed his methods. A bitter feud arose between Salk and his leading rival, Albert Sabin, 
an established polio researcher at the University of Cincinnati who was working on his own live virus vaccine.
The two men were unrelenting in their pursuit of a vaccine, but it was Salk who would introduce his formula first. 
On April 26, 1954, at the Franklin Sherman Elementary School in McLean, 
Virginia, the Salk vaccine field trials began. 
It was the largest public health experiment in American history — 
no one was certain it was safe, or whether it could provide effective protection against the crippling disease.
By June 1954, nearly two million school children in forty-four states had taken part.
On April 12, 1955, almost a year since the end of the field trials, the Salk vaccine was ruled “safe, effective, and potent.
” Within just a few years of being licensed, the Salk vaccine decreased the number of polio cases in the United States by fifty percent. 
By the early 1960s, the number of Americans contracting polio fell to a few thousand annually.
“This vaccine vindicated twenty years of giving dimes, twenty years of volunteering. 
It was a victory for millions of faceless people who had done what they could to end the scourge of polio,” 
says David Oshinsky in the film.
“The story of the polio crusade pays tribute to a time when Americans banded together to conquer a terrible disease,
” says AMERICAN EXPERIENCE executive producer Mark Samels. “The result was a medical breakthrough that saved countless lives, and had a pervasive impact on American philanthropy that continues to be felt today.”

Post Polio Litaff, Association A.C _APPLAC Mexico

NEUROPSYCHOLOGICAL STUDY OF THE POSTPOLIO SYNDROME

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.

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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)
The Post-Polio Syndrome As an. 
Evolved Clinical Entity


Definition and Clinical Description
  1. MARINOS C. DALAKAS
Article first published online: 17 DEC 2006

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.


ANNALS OF THE NEW YORK ACADEMY OF SCIENCES

  THE  OF-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.



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 88Stockholm, 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 vitalityISSN 1650-1977J Rehabil Med 40


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