Title: Late postpoliomyelitis muscular atrophy: clinical, virologic, and immunologic studies
Author: Dalakas MC, Sever JL, Madden DL, Papadopoulos NM, Shekarchi IC, Albrecht P, Krezlewicz A
Affiliation: Not stated
Journal: Reviews of Infectious Diseases
Citation: Rev Infect Dis. 1984 May-Jun; 6 Suppl 2:S562-7
Publication Year and Month: 1984 05

Abstract: Seventeen relatively young patients, ages 31-65 years (average, 45) with prior poliomyelitis, who after a number of years of stability had experienced new neuromuscular symptoms, were studied. Seven patients had deterioration of functional capacity and then stabilization without new muscular weakness. The other 10 had late postpoliomyelitis muscular atrophy (late PPMA) characterized by focal progressive muscle weakness, wasting, fasciculations, and muscle pains affecting previously spared muscles or muscles previously affected but recovered. Four patients with late PPMA had lymphorrhages or lymphocytic infiltrates in their biopsied muscle; three of three patients had oligoclonal IgG bands in their spinal fluid, and five had variable peripheral T lymphocyte-subset ratios. In one patient with late PPMA, antibodies to poliovirus were specifically elevated in the cerebrospinal fluid. Our findings indicate that new motor-neuron disease can occur in patients with prior poliomyelitis and that immunopathologic mechanisms may play a role.

Conclusions:

Outcome of Research:

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Title: A case of post-poliomyelitis muscular atrophy with cranial nerve signs and widespread muscular atrophy of extremities
Author: Hoshino S, Hayashi A, Ohkoshi N, Mizusawa H, Shoji S
Affiliation: Department of Neurology, University of Tsukuba
Journal: Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 1997 May; 37(5):407-9
Publication Year and Month: 1997 05

Abstract: Here we report a case of a 56-year-old male with post-poliomyelitis muscular atrophy (PPMA), who presented with cranial nerve signs and widespread atrophy of the extremities. He had suffered from poliomyelitis at the age of 2 years. After recovery from the acute stage, the paralysis remained in his left arm. He noticed muscle weakness of the right upper and lower extremities at the age of 45 years and the muscle atrophy progressed to his arms, hip and thigh at the age of 55 years. Neurological examination revealed muscle atrophy of the neck and disturbance of left V, VIII, IX, X and bilateral XI cranial nerves. We diagnosed this case as PPMA from his history and electromyographic and muscle biopsy findings which suggested chronic denervation. Among the 21 PPMA cases in the past in which the acute poliomyelitis had resulted in paralysis of the only one limb, ours was the only case that had muscle atrophy of all the limbs. Cranial nerve involvement is known to occur in acute poliomyelitis; therefore, there is a possibility that the involvement of the cranial nerves in our case might be a delayed progressive symptoms.

Conclusions:

Outcome of Research:

Availability of Paper:

Comments (if any): The full text of this paper is in Japanese.

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Title: An autopsy case of progressive generalized muscle atrophy over 14 years due to post-polio syndrome
Author: Oki R (1), Uchino A, Izumi Y, Ogawa H, Murayama S, Kaji R
Affiliation: (1) Department of Clinical Neuroscience, Institute of Health Bioscience, University of Tokushima Graduate School
Journal: Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 2015 Nov 30
Publication Year and Month: 2015 11

Abstract: We report the case of a 72-year-old man who had contracted acute paralytic poliomyelitis in his childhood. Thereafter, he had suffered from paresis involving the left lower limb, with no relapse or progression of the disease. He began noticing slowly progressive muscle weakness and atrophy in the upper and lower extremities in his 60s. At the age of 72, muscle weakness developed rapidly, and he demonstrated dyspnea on exertion and dysphagia. He died after about 14 years from the onset of muscle weakness symptoms. Autopsy findings demonstrated motoneuron loss and glial scars not only in the plaque-like lesions in the anterior horns, which were sequelae of old poliomyelitis, but also throughout the spine. No Bunina bodies, TDP-43, and ubiquitin inclusions were found. Post-polio syndrome is rarely fatal due to rapid progressive dyspnea and dysphagia. Thus, the pathological findings in the patient are considered to be related to the development of muscle weakness.

Conclusions:

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): The full paper is in Japanese.

Link to Paper (if available): Click here to download

Title: Muscle atrophy is not always sarcopenia
Author: Russell T. Hepple
Affiliation: Department of Kinesiology, Department of Medicine, McGill University, Montreal, Canada
Journal: Journal of Applied Physiology
Citation: J Appl Physiol 113: 677–679, 2012;
doi:10.1152/japplphysiol.00304.2012.
Publication Year and Month: 2012 04

Abstract: The following Viewpoint article uses the histopathology of aging muscle to make the case that sarcopenia of aging is likely distinct from several other clinical causes of muscle atrophy, including some that are now using the term sarcopenia. As will be shown, many of the morphological features of sarcopenia resemble features seen in muscle that has been impacted by sporadic denervation, such as that seen in neurological disorders like amyotrophic lateral sclerosis (7). Due to space constraints, we will only consider how the histopathology of aging
muscle compares with cancer cachexia. However, many of the points made here to distinguish sarcopenia from cancer cachexia should also be considered in other clinical conditions
associated with muscle atrophy.

Conclusions: In summary, the available evidence strongly implicates sporadic and repeating cycles of denervation-reinnervation in the histopathology of aging muscle, including fiber size heterogeneity, fiber type grouping, and MHC coexpression. This point is consistent with the neuromuscular junction deterioration in both human (21) and rodent models (6). These alterations distinguish sarcopenia from cancer cachexia and may also differ from other clinical conditions where aging is not the cause of muscle atrophy but which are currently “borrowing” the term sarcopenia. It should also be pointed out that in contrast to recent arguments that rodents are not suitable models of human muscle aging (23, 32), rodent models faithfully reproduce these hallmark morphological traits of human muscle aging, supporting their value in understanding the mechanisms underlying these phenomena. Finally, although this Viewpoint has focused on histological traits resulting from denervation in aging muscle, there are likely many other morphological, physiological, and molecular traits that also distinguish sarcopenia from other forms of atrophy. Among additional traits that are likely to be relevant are things such as the slow time course of atrophy in sarcopenia, the electrophysiological response to activation (33), the excitation-contraction coupling response (15), alterations in the proteomic profile (13), and likely many others. Thus it is hoped that this Viewpoint will also stimulate broader appreciation for those features so they, too, can be used to further our understanding of the causes of sarcopenia and its treatment.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any):

Link to Paper (if available): Click here to view full text or to download

There are currently 4 papers in this category.

Title: Muscle atrophy is not always sarcopenia
Author: Russell T. Hepple
Affiliation: Department of Kinesiology, Department of Medicine, McGill University, Montreal, Canada
Journal: Journal of Applied Physiology
Citation: J Appl Physiol 113: 677–679, 2012;
doi:10.1152/japplphysiol.00304.2012.
Publication Year and Month: 2012 04

Abstract: The following Viewpoint article uses the histopathology of aging muscle to make the case that sarcopenia of aging is likely distinct from several other clinical causes of muscle atrophy, including some that are now using the term sarcopenia. As will be shown, many of the morphological features of sarcopenia resemble features seen in muscle that has been impacted by sporadic denervation, such as that seen in neurological disorders like amyotrophic lateral sclerosis (7). Due to space constraints, we will only consider how the histopathology of aging
muscle compares with cancer cachexia. However, many of the points made here to distinguish sarcopenia from cancer cachexia should also be considered in other clinical conditions
associated with muscle atrophy.

Conclusions: In summary, the available evidence strongly implicates sporadic and repeating cycles of denervation-reinnervation in the histopathology of aging muscle, including fiber size heterogeneity, fiber type grouping, and MHC coexpression. This point is consistent with the neuromuscular junction deterioration in both human (21) and rodent models (6). These alterations distinguish sarcopenia from cancer cachexia and may also differ from other clinical conditions where aging is not the cause of muscle atrophy but which are currently “borrowing” the term sarcopenia. It should also be pointed out that in contrast to recent arguments that rodents are not suitable models of human muscle aging (23, 32), rodent models faithfully reproduce these hallmark morphological traits of human muscle aging, supporting their value in understanding the mechanisms underlying these phenomena. Finally, although this Viewpoint has focused on histological traits resulting from denervation in aging muscle, there are likely many other morphological, physiological, and molecular traits that also distinguish sarcopenia from other forms of atrophy. Among additional traits that are likely to be relevant are things such as the slow time course of atrophy in sarcopenia, the electrophysiological response to activation (33), the excitation-contraction coupling response (15), alterations in the proteomic profile (13), and likely many others. Thus it is hoped that this Viewpoint will also stimulate broader appreciation for those features so they, too, can be used to further our understanding of the causes of sarcopenia and its treatment.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any):

Link to Paper (if available): Click here to view full text or to download

Title: Late postpoliomyelitis muscular atrophy: clinical, virologic, and immunologic studies
Author: Dalakas MC, Sever JL, Madden DL, Papadopoulos NM, Shekarchi IC, Albrecht P, Krezlewicz A
Affiliation: Not stated
Journal: Reviews of Infectious Diseases
Citation: Rev Infect Dis. 1984 May-Jun; 6 Suppl 2:S562-7
Publication Year and Month: 1984 05

Abstract: Seventeen relatively young patients, ages 31-65 years (average, 45) with prior poliomyelitis, who after a number of years of stability had experienced new neuromuscular symptoms, were studied. Seven patients had deterioration of functional capacity and then stabilization without new muscular weakness. The other 10 had late postpoliomyelitis muscular atrophy (late PPMA) characterized by focal progressive muscle weakness, wasting, fasciculations, and muscle pains affecting previously spared muscles or muscles previously affected but recovered. Four patients with late PPMA had lymphorrhages or lymphocytic infiltrates in their biopsied muscle; three of three patients had oligoclonal IgG bands in their spinal fluid, and five had variable peripheral T lymphocyte-subset ratios. In one patient with late PPMA, antibodies to poliovirus were specifically elevated in the cerebrospinal fluid. Our findings indicate that new motor-neuron disease can occur in patients with prior poliomyelitis and that immunopathologic mechanisms may play a role.

Conclusions:

Outcome of Research:

Availability of Paper:

Comments (if any):

Link to Paper (if available):

Title: A case of post-poliomyelitis muscular atrophy with cranial nerve signs and widespread muscular atrophy of extremities
Author: Hoshino S, Hayashi A, Ohkoshi N, Mizusawa H, Shoji S
Affiliation: Department of Neurology, University of Tsukuba
Journal: Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 1997 May; 37(5):407-9
Publication Year and Month: 1997 05

Abstract: Here we report a case of a 56-year-old male with post-poliomyelitis muscular atrophy (PPMA), who presented with cranial nerve signs and widespread atrophy of the extremities. He had suffered from poliomyelitis at the age of 2 years. After recovery from the acute stage, the paralysis remained in his left arm. He noticed muscle weakness of the right upper and lower extremities at the age of 45 years and the muscle atrophy progressed to his arms, hip and thigh at the age of 55 years. Neurological examination revealed muscle atrophy of the neck and disturbance of left V, VIII, IX, X and bilateral XI cranial nerves. We diagnosed this case as PPMA from his history and electromyographic and muscle biopsy findings which suggested chronic denervation. Among the 21 PPMA cases in the past in which the acute poliomyelitis had resulted in paralysis of the only one limb, ours was the only case that had muscle atrophy of all the limbs. Cranial nerve involvement is known to occur in acute poliomyelitis; therefore, there is a possibility that the involvement of the cranial nerves in our case might be a delayed progressive symptoms.

Conclusions:

Outcome of Research:

Availability of Paper:

Comments (if any): The full text of this paper is in Japanese.

Link to Paper (if available):

Title: An autopsy case of progressive generalized muscle atrophy over 14 years due to post-polio syndrome
Author: Oki R (1), Uchino A, Izumi Y, Ogawa H, Murayama S, Kaji R
Affiliation: (1) Department of Clinical Neuroscience, Institute of Health Bioscience, University of Tokushima Graduate School
Journal: Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 2015 Nov 30
Publication Year and Month: 2015 11

Abstract: We report the case of a 72-year-old man who had contracted acute paralytic poliomyelitis in his childhood. Thereafter, he had suffered from paresis involving the left lower limb, with no relapse or progression of the disease. He began noticing slowly progressive muscle weakness and atrophy in the upper and lower extremities in his 60s. At the age of 72, muscle weakness developed rapidly, and he demonstrated dyspnea on exertion and dysphagia. He died after about 14 years from the onset of muscle weakness symptoms. Autopsy findings demonstrated motoneuron loss and glial scars not only in the plaque-like lesions in the anterior horns, which were sequelae of old poliomyelitis, but also throughout the spine. No Bunina bodies, TDP-43, and ubiquitin inclusions were found. Post-polio syndrome is rarely fatal due to rapid progressive dyspnea and dysphagia. Thus, the pathological findings in the patient are considered to be related to the development of muscle weakness.

Conclusions:

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): The full paper is in Japanese.

Link to Paper (if available): Click here to download

There are currently 4 papers in this category.

Title: An autopsy case of progressive generalized muscle atrophy over 14 years due to post-polio syndrome
Author: Oki R (1), Uchino A, Izumi Y, Ogawa H, Murayama S, Kaji R
Affiliation: (1) Department of Clinical Neuroscience, Institute of Health Bioscience, University of Tokushima Graduate School
Journal: Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 2015 Nov 30
Publication Year and Month: 2015 11

Abstract: We report the case of a 72-year-old man who had contracted acute paralytic poliomyelitis in his childhood. Thereafter, he had suffered from paresis involving the left lower limb, with no relapse or progression of the disease. He began noticing slowly progressive muscle weakness and atrophy in the upper and lower extremities in his 60s. At the age of 72, muscle weakness developed rapidly, and he demonstrated dyspnea on exertion and dysphagia. He died after about 14 years from the onset of muscle weakness symptoms. Autopsy findings demonstrated motoneuron loss and glial scars not only in the plaque-like lesions in the anterior horns, which were sequelae of old poliomyelitis, but also throughout the spine. No Bunina bodies, TDP-43, and ubiquitin inclusions were found. Post-polio syndrome is rarely fatal due to rapid progressive dyspnea and dysphagia. Thus, the pathological findings in the patient are considered to be related to the development of muscle weakness.

Conclusions:

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): The full paper is in Japanese.

Link to Paper (if available): Click here to download

Title: Muscle atrophy is not always sarcopenia
Author: Russell T. Hepple
Affiliation: Department of Kinesiology, Department of Medicine, McGill University, Montreal, Canada
Journal: Journal of Applied Physiology
Citation: J Appl Physiol 113: 677–679, 2012;
doi:10.1152/japplphysiol.00304.2012.
Publication Year and Month: 2012 04

Abstract: The following Viewpoint article uses the histopathology of aging muscle to make the case that sarcopenia of aging is likely distinct from several other clinical causes of muscle atrophy, including some that are now using the term sarcopenia. As will be shown, many of the morphological features of sarcopenia resemble features seen in muscle that has been impacted by sporadic denervation, such as that seen in neurological disorders like amyotrophic lateral sclerosis (7). Due to space constraints, we will only consider how the histopathology of aging
muscle compares with cancer cachexia. However, many of the points made here to distinguish sarcopenia from cancer cachexia should also be considered in other clinical conditions
associated with muscle atrophy.

Conclusions: In summary, the available evidence strongly implicates sporadic and repeating cycles of denervation-reinnervation in the histopathology of aging muscle, including fiber size heterogeneity, fiber type grouping, and MHC coexpression. This point is consistent with the neuromuscular junction deterioration in both human (21) and rodent models (6). These alterations distinguish sarcopenia from cancer cachexia and may also differ from other clinical conditions where aging is not the cause of muscle atrophy but which are currently “borrowing” the term sarcopenia. It should also be pointed out that in contrast to recent arguments that rodents are not suitable models of human muscle aging (23, 32), rodent models faithfully reproduce these hallmark morphological traits of human muscle aging, supporting their value in understanding the mechanisms underlying these phenomena. Finally, although this Viewpoint has focused on histological traits resulting from denervation in aging muscle, there are likely many other morphological, physiological, and molecular traits that also distinguish sarcopenia from other forms of atrophy. Among additional traits that are likely to be relevant are things such as the slow time course of atrophy in sarcopenia, the electrophysiological response to activation (33), the excitation-contraction coupling response (15), alterations in the proteomic profile (13), and likely many others. Thus it is hoped that this Viewpoint will also stimulate broader appreciation for those features so they, too, can be used to further our understanding of the causes of sarcopenia and its treatment.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any):

Link to Paper (if available): Click here to view full text or to download

Title: A case of post-poliomyelitis muscular atrophy with cranial nerve signs and widespread muscular atrophy of extremities
Author: Hoshino S, Hayashi A, Ohkoshi N, Mizusawa H, Shoji S
Affiliation: Department of Neurology, University of Tsukuba
Journal: Rinsho Shinkeigaku (Clinical Neurology)
Citation: Rinsho Shinkeigaku. 1997 May; 37(5):407-9
Publication Year and Month: 1997 05

Abstract: Here we report a case of a 56-year-old male with post-poliomyelitis muscular atrophy (PPMA), who presented with cranial nerve signs and widespread atrophy of the extremities. He had suffered from poliomyelitis at the age of 2 years. After recovery from the acute stage, the paralysis remained in his left arm. He noticed muscle weakness of the right upper and lower extremities at the age of 45 years and the muscle atrophy progressed to his arms, hip and thigh at the age of 55 years. Neurological examination revealed muscle atrophy of the neck and disturbance of left V, VIII, IX, X and bilateral XI cranial nerves. We diagnosed this case as PPMA from his history and electromyographic and muscle biopsy findings which suggested chronic denervation. Among the 21 PPMA cases in the past in which the acute poliomyelitis had resulted in paralysis of the only one limb, ours was the only case that had muscle atrophy of all the limbs. Cranial nerve involvement is known to occur in acute poliomyelitis; therefore, there is a possibility that the involvement of the cranial nerves in our case might be a delayed progressive symptoms.

Conclusions:

Outcome of Research:

Availability of Paper:

Comments (if any): The full text of this paper is in Japanese.

Link to Paper (if available):

Title: Late postpoliomyelitis muscular atrophy: clinical, virologic, and immunologic studies
Author: Dalakas MC, Sever JL, Madden DL, Papadopoulos NM, Shekarchi IC, Albrecht P, Krezlewicz A
Affiliation: Not stated
Journal: Reviews of Infectious Diseases
Citation: Rev Infect Dis. 1984 May-Jun; 6 Suppl 2:S562-7
Publication Year and Month: 1984 05

Abstract: Seventeen relatively young patients, ages 31-65 years (average, 45) with prior poliomyelitis, who after a number of years of stability had experienced new neuromuscular symptoms, were studied. Seven patients had deterioration of functional capacity and then stabilization without new muscular weakness. The other 10 had late postpoliomyelitis muscular atrophy (late PPMA) characterized by focal progressive muscle weakness, wasting, fasciculations, and muscle pains affecting previously spared muscles or muscles previously affected but recovered. Four patients with late PPMA had lymphorrhages or lymphocytic infiltrates in their biopsied muscle; three of three patients had oligoclonal IgG bands in their spinal fluid, and five had variable peripheral T lymphocyte-subset ratios. In one patient with late PPMA, antibodies to poliovirus were specifically elevated in the cerebrospinal fluid. Our findings indicate that new motor-neuron disease can occur in patients with prior poliomyelitis and that immunopathologic mechanisms may play a role.

Conclusions:

Outcome of Research:

Availability of Paper:

Comments (if any):

Link to Paper (if available):

There are currently 4 papers in this category.