A Hummingbirds Guide to M.E.

Information on the neurological disease Myalgic Encephalomyelitis

General Muscle Research

*O* Alterations in muscles of CFS patients at morphological, biochemical and molecular level. Pizzigallo E, Di Girolamo A, Montanari G, Dragani L, Vecchiet J, Calella G. Journal of Chronic Fatigue Syndrome 1996; 2(2/3) 76-77.

Abstract: OBJECTIVES. The peripheral origin of symptoms related to CFS has been hypothesized from various AA and is still under investigation to determine if symptoms can be related to muscular damage. Our studies aimed to look for specific alterations in muscles of CFS patients, followed in our Clinic and enrolled according to the 1988 CDC criteria (Holmes et al.) revised in 1994 by Fukuda et al. (CDC). METHODS. Fourteen CFS patients, 3 male and 11 females, 17 to 60 years old (mean 34.6), mean illness duration 49.9 months, post viral onset in 10 cases, underwent muscular biopsy of the vastus lateralis according to Edwards, et al., using a UCH needle. We analyzed the specimens by electron (EM) and light (LM) microscopy. Moreover, we performed histochemical and quantitative analysis of enzymatic activities and studies of mitochondrial DNA (mtDNA) deletions. RESULTS. All specimens showed: hypotrophy, especially of the type 11 (a and b) fibres; fibres fragmentation, red ragged fibres and fusion events with nuclei centralisation; and fatty and fibrous degeneration. EM observations confirmed these alterations, showed degenerative changes in the I band, and allowed us to detect the poli/pleiomorphism and cristae thickening of the mitochondia. The alterations of the fibres always began from an I band of a sarcomere. The histochemical and quantitative determination of the enzymatic activities showed important reduction, in particular of the cytochrome-oxydase and citrate-synthetase. Finally, the "common deletion" of 4977 bp of the mtdna was increased as high as 3,000 times the normal values in 3 patients. CONCLUSIONS. Our results agree with those of other AA (Behan et al., 1991; Gow et al., 1994). The alterations are compatible with a myopathy of probable mitochondrial origin. This could explain the drop in the functional capability of the muscle as a reduction in potency but, above all, as a reduction in resistance. In conclusion, even if CFS seems to be attributable to mitochondrial and/or muscular alterations, a damage in the central nervous system cannot be excluded. This could explain the neurophychological, behavioral, and neuroendocrinological alterations often found in these patients. 


Impaired oxygen delivery to muscle in chronic fatigue syndrome. McCully KK, Natelson BH Department of Medicine, Medical College of Pennsylvania and Hahnemann University, Philadelphia, PA 19129, USA. kmccully@coe.uga.edu
Clin Sci (Colch)
1999 Nov;97(5):603-8

The purpose of this study was to determine if chronic fatigue syndrome (CFS) is associated with reduced oxygen delivery to muscles. Patients with CFS according to CDC (Center for Disease Control) criteria (n=20) were compared with normal sedentary subjects (n=12). Muscle oxygen delivery was measured as the rate of post-exercise and post-ischaemia oxygen-haem resaturation. Oxygen-haem resaturation was measured in the medial gastrocnemius muscle using continuous-wavelength near-IR spectroscopy. Phosphocreatine resynthesis was measured simultaneously using (31)P magnetic resonance spectroscopy. The time constant of oxygen delivery was significantly reduced in CFS patients after exercise (46.5+/-16 s; mean+/-S.D.) compared with that in controls (29.4+/-6.9 s). The time constant of oxygen delivery was also reduced (20.0+/-12 s) compared with controls (12.0+/-2.8 s) after cuff ischaemia. Oxidative metabolism was also reduced by 20% in CFS patients, and a significant correlation was found between oxidative metabolism and recovery of oxygen delivery. In conclusion, oxygen delivery was reduced in CFS patients compared with that in sedentary controls. This result is consistent with previous studies showing abnormal autonomic control of blood flow. Reduced oxidative delivery in CFS patients could be specifically related to CFS, or could be a non-specific effect of reduced activity levels in these patients. While these results suggest that reduced oxygen delivery could result in reduced oxidative metabolism and muscle fatigue, further studies will be needed to address this issue.


*O* Post-viral fatigue syndrome: evidence for underlying organic disturbance in the muscle fibre. Jamal GA, Hansen S. European Neurology 1989; 29: 273-276.

Abstract: Ten patients with post-viral fatigue syndrome and abnormal serological, virological, immunological and histological studies were examined by the single-fibre electromyographic (EMG) technique after excluding concurrent problems in the neuromuscular system. No abnormality of fibre density was noted but all patients had abnormal jitter values. Very high jitter values were not associated with impulse or concomitant blocking. The findings confirm the organic nature of the disease. A muscle membrane disorder probably arising from defective myogenic enzymes is the likely mechanism for the fatigue and the single-fibre EMG abnormalities. This muscle membrane defect may be due to the effects of a persistent viral infection.


*O* Biochemical and muscle studies in patients with acute onset post-viral fatigue syndrome. Preedy VR, Smith DG, Salisbury JR, Peters TJ. Journal of Clinical Pathology 1993; 46(8): 722-6.

Abstract: AIMS-To investigate in detail various biochemical and pathophysiological indices of muscle pathology in acute onset post-viral fatigue syndrome (PVFS). METHODS-Twenty three patients with PVFS (of mean duration 4.6 years) were subjected to needle biopsy for histomorphometry and total RNA contents. Plasma analysis included serology and creatine kinase activities. Indices of whole body mass were also measured-namely, whole body potassium content and plasma carnosinase activities. RESULTS-About 80% of the patients had serology indicative of persistent enteroviral infection as determined by VP1 antigen assay. Only about 10% of that same group of patients had serological indications of current enterovirus infection by IgM assay; a separate subset of 10% showed antibody changes suggestive of reactivation of Epstein-Barr virus. Quantitative morphometric analysis of skeletal muscle fibres indicated that the quadriceps muscle was normal or displayed only minor abnormalities in 22 patients. The Quetelet's Index (body mass index) and whole-body potassium values (index of lean body mass) were not affected in PVFS. The mean plasma carnosinase and creatinine kinase activities were also generally normal in these patients. The mean muscle RNA composition-mg RNA/mg DNA-was significantly reduced in acute onset PVFS by about 15%. The protein:DNA ratio was not significantly affected. CONCLUSIONS-Patients with acute onset PVFS, therefore, lose muscle protein synthetic potential, but not muscle bulk. Histopathology is consistent with these observations. These perturbations may contribute to the apparent feature of perceived muscle weakness associated with the persistent viral infection in the muscle themselves.


 
*O* Enteroviruses and postviral fatigue syndrome. Behan PO, Behan WM, Gow JW, Cavanagh H, Gillespie S.Department of Neurology, University of Glasgow, UK.

Postviral fatigue syndrome (PFS) occurs both in epidemics and sporadically. Many of the original epidemics were related to poliomyelitis outbreaks which either preceded or followed them. The core clinical symptoms are always the same: severe fatigue made worse by exercise, myalgia, night sweats, atypical depression and excessive sleep. The other common symptoms include dysequilibrium disorders and irritable bowel syndrome. We have detected enteroviral genome sequences in muscle biopsies from cases of PFS, using specific enteroviral oligonucleotide primers in the polymerase chain reaction (PCR). In addition, whole virus particles can be demonstrated in PCR-positive muscle, using solid-phase immuno-electron microscopy. An increase in the number and size of muscle mitochondria was found in 70% of PFS cases, suggesting an abnormality in metabolic function. Evidence of hypothalamic dysfunction was present, particularly involving 5-hydroxytryptamine metabolism. A putative model of PFS, based on persistent enteroviral infection in laboratory mice, revealed resolving inflammatory lesions in muscle with, however, a marked increase in the production of certain cytokines in the brain. This model may help to explain the pathogenesis of PFS.
 

 
*O* Mitochondrial abnormalities in the postviral fatigue syndrome. Behan WM, More IA, Behan PO. Acta Neuropathol (Berl). 1991;83(1):61-5. Department of Pathology, University of Glasgow, Scotland.

We have examined the muscle biopsies of 50 patients who had postviral fatigue syndrome (PFS) for from 1 to 17 years. We found mild to severe atrophy of type II fibres in 39 biopsies, with a mild to moderate excess of lipid. On ultrastructural examination, 35 of these specimens showed branching and fusion of mitochondrial cristae. Mitochondrial degeneration was obvious in 40 of the biopsies with swelling, vacuolation, myelin figures and secondary lysosomes. These abnormalities were in obvious contrast to control biopsies, where even mild changes were rarely detected. The findings described here provide the first evidence that PFS may be due to a mitochondrial disorder precipitated by a virus infection.
 

 
[Postviral fatigue syndrome] [Article in Norwegian] Haukenes G, Aarli JA.

Avdeling for mikrobiologi og immunologi Gades Institutt, Universitetet i Bergen, Armauer Hansens hus.

The post-viral fatigue syndrome occurs sporadically and in local outbreaks (Los Angeles, Akureyri, Royal Free Hospital). The clinical picture is marked by long-lasting muscular fatigue directly following an acute infection. Other conditions associated with pronounced fatigue must be excluded. The diagnostic criteria set up by Centers for Disease Control (CDC) are the ones most commonly used. Aetiology and pathogenesis are unknown. Coxsackie B-virus seems to be associated with some cases at least. Immunological and endocrinological aberration, morphological changes in mitochondria and reduced cerebral blood perfusion have been demonstrated in some patients. There is no specific therapy. It is important for the patient that the symptoms be accepted by the doctor and society in general.
 

 
Sensory characterization of somatic parietal tissues in humans with chronic fatigue syndrome. Vecchiet L, Montanari G, Pizzigallo E, Iezzi S, de Bigontina P, Dragani L, Vecchiet J, Giamberardino MA. Institute of Medical Pathophysiology, 'G. D'Annunzio' University of Chieti, Italy.

Patients with chronic fatigue syndrome (CFS) mainly complain of symptoms in the musculoskeletal domain (myalgias, fatigue). In 21 CFS patients the deep (muscle) versus superficial (skin, subcutis) sensitivity to pain was explored by measuring pain thresholds to electrical stimulation unilaterally in the deltoid, trapezius and quadriceps and overlying skin and subcutis in comparison with normal subjects. Thresholds in patients were normal in skin and subcutis but significantly lower than normal (hyperalgesia) in muscles (P < 0.001) in all sites. The selective muscle hypersensitivity corresponded also to fiber abnormalities at muscle biopsy (quadriceps) performed in nine patients which were absent in normal subjects (four cases): morphostructural alterations of the sarchomere, fatty degeneration and fibrous regeneration, inversion of the cytochrome oxidase/succinate dehydrogenase ratio, pleio/polymorphism and monstruosity of mitochondria, reduction of some mitochondrial enzymatic activities and increments of common deletion of 4977 bp of mitochondrial DNA 150-3000 times the normal values. By showing both sensory (diffuse hyperalgesia) and anatomical (degenerative picture) changes at muscle level, the results suggest a role played by peripberal mechanisms in the genesis of CFS symptoms. They would exclude the heightened perception of physiological signals from all districts hypothesized by some authors, especially as the hyperalgesia is absent in skin/subcutis.


Muscle fibre characteristics and lactate responses to exercise in chronic fatigue syndrome Russell J M Lane,a Michael C Barrett,b David Woodrow,b Jill Moss,b Robert Fletcher,b Leonard C Archardc a Division of Neuroscience and Psychological Medicine, b Division of Diagnostic and Investigative Sciences, c Division of Biochemical Sciences, Imperial College School of Medicine, Charing Cross Hospital, London, UKJ Neurol Neurosurg Psychiatry 1998;64:362-367

OBJECTIVES To examine the proportions of type 1 and type 2 muscle fibres and the degree of muscle fibre atrophy and hypertrophy in patients with chronic fatigue syndrome in relation to lactate responses to exercise, and to determine to what extent any abnormalities found might be due to inactivity.
METHODS Quadriceps needle muscle biopsies were obtained from 105 patients with chronic fatigue syndrome and the proportions of type 1 and 2 fibres and fibre atrophy and hypertrophy factors were determined from histochemical preparations, using a semiautomated image analysis system. Forty one randomly selected biopsies were also examined by electron microscopy. Lactate responses to exercise were measured in the subanaerobic threshold exercise test (SATET).
RESULTS Inactivity would be expected to result in a shift to type 2 fibre predominance and fibre atrophy, but type 1 predominance (23%) was more common than type 2 predominance (3%), and fibre atrophy was found in only 10.4% of cases. Patients with increased lactate responses to exercise did have significantly fewer type 1 muscle fibres (p<0.043 males, p<0.0003 females), but there was no evidence that this group was less active than the patients with normal lactate responses. No significant ultrastructural abnormalities were found. CONCLUSION Muscle histometry in patients with chronic fatigue syndrome generally did not show the changes expected as a result of inactivity. However, patients with abnormal lactate responses to exercise had a significantly lower proportion of mitochondria rich type 1 muscle fibres.


In vivo magnetic resonance spectroscopy in chronic fatigue syndrome. Chaudhuri A, Behan PO.Division of Clinical Neurosciences, Institute of Neurological Sciences, Southern General Hospital, University of Glasgow, 1345 Govan Road, Glasgow G51 4TF, UK. ac54p@udcf.gla.ac.uk

The pathogenic mechanisms of chronic fatigue syndrome (CFS) are not clearly known. Fatigue, poor short-term memory and muscle pain are the most disabling symptoms in CFS. Research data on magnetic resonance spectroscopy (MRS) of muscles and brain in CFS patients suggest a cellular metabolic abnormality in some cases. 31P MRS of skeletal muscles in a subset of patients indicate early intracellular acidosis in the exercising muscles. 1H MRS of the regional brain areas in CFS have shown increased peaks of choline derived from the cell membrane phospholipids. Cell membrane oxidative stress may offer a common explanation for the observed MRS changes in the muscles and brain of CFS patients and this may have important therapeutic implications. As a research tool, MRS may be used as an objective outcome measure in the intervention studies. In addition, regional brain 1H MRS has the potential for wider use to substantiate a clinical diagnosis of CFS from other disorders of unexplained chronic fatigue.


Excessive intracellular acidosis of skeletal muscle on exercise in a patient with a post-viral exhaustion fatigue syndrome. Arnold DL, Radda GK, Bore PJ, Styles P, Taylor DJ. Lancet 1984; 1: 1367-9.

Abstract: A patient with prolonged post-viral exhaustion and excessive fatigue was examined by 31P nuclear magnetic resonance. During exercise, muscles of the forearm demonstrated abnormally early intracellular acidosis for the exercise performed. This was out of proportion to the associated changes in high-energy phosphates. This may represent excessive lactic acid formation resulting from a disorder of metabolic regulation. The metabolic abnormality in this patient could not have been demonstrated by traditional diagnostic techniques.


Specific oxidative alterations in vastus lateralis muscle of patients with the diagnosis of chronic fatigue syndrome Stefania Fulle (a), Patrizia Mecocci (b), Giorgio Fano (c), Iacopo Vecchiet (d), Alba Vecchini (e), Delia Racciotti (d), Antonio Cherubini (b), Eligio Pizzigallo (d), Leonardo Vecchiet (c), Umberto Senin (b) and M. Flint Beal (f). Address correspondence to: Dr. M. Flint Beal, Chairman, Neurology Department, New York Hospital-Cornell Medical Center, 525 East 68th Street, New York, NY 10021, USA; Tel: (212) 746-6575; Fax: (212) 746-8532; email: fbeal@mail.med.cornell.edu

Free Radical Biology and Medicine Dec 15, 2000, Vol. 29, No. 12, 1252-59Chronic fatigue syndrome (CFS) is a poorly understood disease characterized by mental and physical fatigue, most often observed in young white females. Muscle pain at rest, exacerbated by exercise, is a common symptom. Although a specific defect in muscle metabolism has not been clearly defined, yet several studies report altered oxidative metabolism. In this study, we detected oxidative damage to DNA and lipids in muscle specimens of CFS patients as compared to age-matched controls, as well as increased activity of the antioxidant enzymes catalase, glutathione peroxidase, and transferase, and increases in total glutathione plasma levels. From these results we hypothesize that in CFS there is oxidative stress in muscle, which results in an increase in antioxidant defenses. Furthermore, in muscle membranes, fluidity and fatty acid composition are significantly different in specimens from CFS patients as compared to controls and to patients suffering from fibromyalgia.

These data support an organic origin of CFS, in which muscle suffers oxidative damage.


Relationship between musculoskeletal symptoms and blood markers of oxidative stress in patients with chronic fatigue syndrome.
Jacopo Vecchiet, Francesco Cipollone, Katia Falasca, Andrea Mezzetti, Eligio Pizzigallo, Tonino Bucciarelli, Silvana De Laurentis, Giannapia Affaitati, Domenico De Cesare, Maria Adele Giamberardino. Department of Medicine and Science of Aging, 'G. D'Annunzio' University of Chieti, Italy Source: Neuroscience Letters 2003; 335(3):151-154.

Abstract: In 21 patients with chronic fatigue syndrome (CFS) versus 20 normal subjects, we investigated the oxidant/antioxidant balance and its correlation with muscle symptoms. Patients versus controls showed significantly: lower Lag Phase and Vitamin E (Vit E) concentrations in plasma and low-density lipoproteins (LDL), higher LDL thiobarbituric acid reactive substances (TBARS), higher fatigue and lower muscle pain thresholds to electrical stimulation. A significant direct linear correlation was found between fatigue and TBARS, thresholds and Lag Phase, thresholds and Vit E in plasma and LDL. A significant inverse linear correlation was found between fatigue and Lag Phase, fatigue and Vit E, thresholds and TBARS. Increased oxidative stress and decreased antioxidant defenses are related to the extent of symptomatology in CFS, suggesting that antioxidant supplementation might relieve muscle symptoms in the syndrome.


Demonstration of delayed recovery from fatiguing exercise in chronic fatigue syndrome.

The authors attempted to confirm the consistent report by patients with the CFS of delay in recovery of peripheral muscle function after exercise. They tested the quadriceps muscle group of 10 patients and 10 controls. Recovery was prolonged in the patient group, with a significant difference between the two groups after exercise and after 24 hours. These findings support the clinical complaint of delayed recovery after exercise in patients with CFS.


Chronic fatigue syndrome: assessment of increased oxidative stress and altered muscle excitability in response to incremental exercise.

Plus a discussion of the text.

"Thus, as in inherited muscular dystrophy in which a variety of cellular abnormalities can be accounted for by free radical-mediated damages including abnormal functions of the sarcolemma and an altered activity of membrane-bound enzymes involved in excitation-contraction coupling, an increased level of free radical damage in CFS may be a contributor to the underlying functional defects and symptom presentation. This should promote further researches towards the goal of an effective treatment of CFS-suffering patients."


Some salient points arising from the AACFS 6th International Conference which the MRC Research Advisory Group on "CFS/ME" might wish to consider

Skeletal muscle function and mitochondrial function suggests a defect in oxidative metabolism with a residual acceleration of glycolysis in the working skeletal muscles in CFS. There is also reduced oxidative muscle metabolism (shown by MRI), and muscle recovery is delayed.


Skeletal muscle metabolism in the chronic fatigue syndrome - In vivo assessment by 31P nuclear magnetic resonance spectroscopy. Wong R, Lopaschuk G, Zhu G, Walker D, Catellier D, Burton D, Teo K, Collins-Nakai R, Montague T. Chest 1992; 102(6): 1716-22.

Abstract: BACKGROUND: Previous study of patients with chronic fatigue syndrome (CFS) has demonstrated a markedly reduced dynamic exercise capacity, not limited by cardiac performance and in the absence of clinical neuromuscular dysfunction, suggesting the possibility of a subclinical defect of skeletal muscle. METHODS: The in vivo metabolism of the gastrocnemius muscles of 22 CFS patients and 21 normal control subjects was compared during rest, graded dynamic exercise to exhaustion and recovery, using 31P nuclear magnetic resonance (NMR) spectroscopy to reflect minute-to-minute intracellular high-energy phosphate metabolism. RESULTS: Duration of exercise was markedly shorter in the CFS patients (8.1 ± 2.8 min) compared with the normal subjects (11.3 ± 4.3 min) (p = 0.005). There were large changes in phosphocreatine (PCr), inorganic phosphate (Pi), and pH from rest to clinical fatigue in all subjects, reflecting the high intensity of the exercise. The temporal metabolic patterns were qualitatively similar in the CFS patients and normal subjects. There were early and continuous changes in PCr and Pi that peaked at the point of fatigue and rapidly reversed after exercise. In contrast, pH was relatively static in early exercise, not declining noticeably until 50 percent of total exercise duration was achieved, and reaching a nadir at 2 min postexercise, before rapidly reversing. There were no differences in pH at rest (7.08 ± 0.04 vs 7.10 ± 0.04), exhaustion (6.85 ± 0.17 vs 6.76 ± 0.17) or early (6.64 ± 0.25 vs 6.56 ± 0.24) or late recovery (7.09 ± 0.04 vs 7.10 ± 0.05), CFS patients vs normal subjects, respectively (NS). Neither were there intergroup differences (NS) in PCr or Pi. Although, quantitatively, the changes in PCr, Pi, and pH were marked and similar in both groups from rest to exhaustion, the changes all occurred much more rapidly in the CFS patients. Moreover, adenosine triphosphate (ATP) was significantly (p = 0.007) less at exhaustion in the CFS group. CONCLUSIONS: Patients with CFS and normal control subjects have similar skeletal muscle metabolic patterns during dynamic exercise and reach similar clinical and metabolic end points. However, CFS patients reach exhaustion much more rapidly than normal subjects, at which point they also have relatively reduced intracellular concentrations of ATP. These data suggest a defect of oxidative metabolism with a resultant acceleration of glycolysis in the working skeletal muscles of CFS patients. This metabolic defect may contribute to the reduced physical endurance of CFS patients. Its etiology is unknown. Whether CFS patients' overwhelming tiredness at rest has a similar metabolic pathophysiology or etiology also remains unknown.


Muscle performance, voluntary activation, twitch properties and perceived exertion in normal subjects and patients with chronic fatigue syndrome. Lloyd AR, Gandevia SC, Hales JP. Brain 1991; 114: 85-98.

Abstract: The decrease in maximal force-generating capacity, the degree of central activation of the muscle, and the subjective perception of effort were measured during prolonged submaximal isometric exercise in 12 male patients suffering from the 'chronic fatigue syndrome' and 13 naive, healthy male subjects. Maximal voluntary isometric torque generated by the elbow flexors was measured before, and at 5 min intervals during an endurance sequence of 45 min of repetitive isometric contractions (6 s duration, 4 s rest interval) producing 30% of the initial maximal voluntary torque. Electrical stimuli were also delivered to the elbow flexors to measure the contractile force in the intervals between voluntary contractions. The degree of central motor activation during maximal voluntary contractions was assessed using a sensitive method of twitch interpolation. In addition, the perceived effort required to achieve the target submaximal contractions was recorded using a standardized self-report scale. A high degree of central activation was achieved in maximal contractions during the endurance sequence both in the patients (mean of maximal force 93.6%; SD 7.8%), and in the control subjects (mean 90.9%; SD 9.5%). The relative torque produced by either voluntary or electrically stimulated contractions was not significantly different between patients and control subjects throughout the test. There was no significant difference in the perceived exertion between the patients and control subjects. These findings support the concept that neither poor motivation, nor muscle contractile failure is important in the pathogenesis of 'fatigue' in patients with the chronic fatigue syndrome.

Relevant Books

*O* The Clinical and Scientific Basis of Myalgic Encephalomyelitis / Chronic Fatigue Syndrome Edited by Byron Hyde, M.D. , Nightingale Research Foundation, Ottawa, Canada
 

 
Mitochondrial Cytopathies: A Primer written by Dr. Bruce Cohen, MD
 


The Sinatra Solution produced by metabolic cardiologist, Dr Stephen Sinatra

 
 

 
 
Hard copies of the Canadian Guidelines are available for purchase from Haworth Press
 

 
*O* Engaging with M.E. (DVD format and printed format) Professor Malcolm Hooper
 

 
*O* What is ME? What is CFS? Information for Clinicians and Lawyers (Online format and printed format) by Professor Malcolm Hooper, Margaret Williams and Eileen Marshall
 

 
 

 
Faces of CFS by David S. Bell MD.
 
Available as a free PDF download


The Doctor's Guide to CFIDS by David S. Bell MD


A Parents Guide to CFIDS by David S. Bell MD


The Disease of a Thousand Names by David S. Bell MD


 
Note: Links given with certain book titles are for informational purposes only and are not recommendations of any particular book seller. It is recommended that you shop around for the best prices at a number of stores for any books you might wish to purchase.

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Page 1: Mitochondrial Muscle Research

Page 2: General Muscle Research and Relevant Books