MUSCLE HIGH-ENERGY PHOSPHATES IN CENTRAL NERVOUS SYSTEM DISORDERS. THE PHOSPHORUS MRS EXPERIENCE
Argov Z; De Stefano N; Arnold DL
Magnetic Resonance Spectroscopy Unit, Montreal Neurological Institute, Canada.
Ital J Neurol Sci, Dec 1997, 18(6), p353-7
Phosphorus magnetic resonance spectroscopy (MRS) was use to study muscle phosphates metabolism in several brain disorders. Those with primary mitochondrial encephalomyopathies showed the typical pattern of impaired oxidative metabolism at rest and during recovery after exercise. In migraine, Parkinson=s Disease and alternating hemiplegia muscle MRS observations lend support to possible mitochondrial dysfunction. Similar observations in multiple sclerosis are probably the result of secondary deconditioning. In post Polio syndrome and in some of the hereditary ataxias, elevated intracellular inorganic phosphates may be the result of another, yet unknown, metabolic impairment. Thus, muscle phosphate metabolism may be altered in various central nervous system (CNS) disorders by different metabolic impairments. All these possibilities should be taken into account when evaluating MRS results in brain diseases.
Central Nervous System Diseases – Metabolism; Muscles – Metabolism; Nuclear Magnetic Resonance – Methods; Phosphates – Metabolism
Exercise – Physiology; Migraine – Metabolism; Mitochondrial Encephalomyopathies – Metabolism; Phosphorus
(THE NEW PARAMETERS OF MOTOR UNIT POTENTIAL IN THE DIAGNOSIS OF NEUROGENIC LESIONS IN SPIKE-TRIGGERED AVERAGING ELECTROMYOGRAPHY)
Liu X; Wang G; Qiu H
China-Japan Friendship Hospital, Beijing, China.
Chung Hua I Hsueh Tsa Chih, Nov 1997, 77(11), p838-41
ARTICLE IN CHINESE
OBJECTIVE: To evaluate the clinical significance of the new parameters of motor unit potential (MUP) to the diagnosis of neurogenic lesions.
METHODS: Three groups of muscles, anterior tibialis, abductor pollicis brevis and vastus medialis were studied with spike-triggered averaging electromyography (EMG). A total of 3,646 MUPs, 1,903 from normal group and 1,743 from neurogenic group, were recorded under slight muscle contraction.
RESULTS: The area, as a single parameter, produced the most significant result in discriminant analysis, about 80% MUPs from neurogenic muscles being judged to be abnormal. Compared with the area, the amplitude and duration were less sensitive, being able to assign about 77% and 70% of MUPs in the neurogenic group as abnormal. A combination of the area/amplitude ratio (thickness) and the amplitude, and a combination of the duration (width) and the amplitude could further improve the discriminating ability, around 80% and 81% MUPs in the neurogenic muscles being classified as abnormal by the combinations. The simplified discriminant functions, 2 x Log10 (amplitude) +Log10 (area/amplitude) -4 for size index, and 5 x Log10 (duration) + 3 x Log10 (amplitude) -10 for width-height index had same discriminating ability as the real discriminant function.
CONCLUSIONS: The new parameters, SI, WHI, and area in spike-triggered EMG seemed to be promising, since they produced a better yield in the diagnosis of neurogenic lesions than amplitude or duration alone.
Motor Neuron Disease – Physiopathology; Postpoliomyelitis Syndrome – Physiopathology
Adolescence; Adult; Aged; Child; Discriminant Analysis; Electromyography; Middle Age; Motor Neuron Disease – Diagnosis; Postpoliomyelitis Syndrome – Diagnosis
EARLY AND LATE LOSSES OF MOTOR UNITS AFTER POLIOMYELITIS
Mc Comas AJ; Quartly C; Griggs RC
Department of Medicine, McMaster University, Hamilton, Canada
Brain, August 1997, 120(8) p1415-21
Motor unit number estimation was employed to assess muscle innervation in 76 patients with prior poliomyelitis. Of the 68 patients who were < 70 years of age, new musculoskeletal symptoms had appeared in all but four; the mean latent interval was 38.0 +/- 10.1 years. As expected, there was a high incidence of muscles exhibiting denervation in previously affected limbs (87%). However, the incidence in supposedly unaffected limbs was also high (65%). Significant differences in the degree of denervation were found between muscles of the same hands and feet. Judged on the basis of their potential amplitudes, the surviving motor units in partially denervated muscles tended to be enlarged. The enlargement was proportional to the extent of the denervation and was comparable to that found in amyotrophic lateral sclerosis. In some muscles, possibly those innervated by failing motor neurons, motor-unit enlargement was not present. Needle examination confirmed the high incidences of denervation in affected and allegedly unaffected limbs. Of the 188 muscles with EMG features of chronic denervation, only nine exhibited fibrillations or positive sharp waves (4.8%). Ninety-five muscles of 18 patients were studied a second time after an interval of 2 years. Overall, there was a 13.4% reduction in motor-unit number and a 18.4 diminution in M-wave amplitude (P< 0.001). The rate of motor-unit loss was twice that occurring in healthy subjects aged > 60 years. Analysis of individual patients indicated that some were deteriorating more rapidly than others. These studies confirm that denervation progresses in patients with prior poliomyelitis in both clinically affected and unaffected muscles, and indicate that this progression is more rapid than that in normal ageing.
Muscle, Skeletal – Innervation; Muscle, Skeletal – Pathology; Poliomyelitis – Pathology
Adult; Aged; Aged, 80 and over; Electromyography; Follow-Up Studies; Middle Age; Motor Neurons – Pathology; Motor Neurons – Physiology; Motor Neurons – Virology; Muscle Denervation; Muscle Fibers – Pathology; Muscle Fibers – Physiology; Muscle, Skeletal – Physiology
STRENGTH, ENDURANCE, AND WORK CAPACITY AFTER MUSCLE STRENGTHENING EXERCISE IN POSTPOLIO SUBJECTS
Agre JC; Rodriquez AA; Franke TM
Department of Rehabilitation Medicine, University of Wisconsin Medical School, Madison, USA
Arch Phys Med Rehabil, Jul 1997, 78(7), p681-6
OBJECTIVE: To determine whether a 12-week home quadriceps muscle strengthening exercise program would increase muscle strength, isometric endurance, and tension time index (TTI) in postPolio syndrome subjects without adversely affecting the surviving motor units or the muscle.
DESIGN: A longitudinal study to investigate the effect of a 12-week exercise program on neuromuscular function and electromyographic variables.
SUBJECTS: Seven subjects were recruited from a cohort of 12 subjects who had participated in a previous exercise study. All subjects had greater than antigravity strength of the quadriceps. Upon completion of a postPolio questionnaire, all acknowledge common postPolio syndrome symptoms such as new fatigue, pain, and weakness; 6 of the 7 acknowledged new strength decline.
INTERVENTION: On Mondays and Thursdays subjects performed three sets of four maximal isometric contractions of the quadriceps held for 5 seconds each. On Tuesdays and Fridays subjects performed three sets of 12 dynamic knee extension exercises with ankle weights.
MAIN OUTCOME MEASURES: Neuromuscular variables of the quadriceps muscles were measured at the beginning and completion of the exercise program and included: isokinetic peak torque (ISOKPT, at 60 degrees/sec angular velocity) and total work performed of four contractions (ISOKTW), isometric peak torque (MVC), endurance (EDUR, time subject could hold isometric contraction at 40% of the initial MVC), isometric tension time index (TTI, product of endurance time and torque at 40% of MVC), and initial and final ankle weight (WGT, kg) lifted. Electromyographic variables included: fiber density (FD), jitter (MCD), and blocking (BLK) from single fiber assessment and median macro amplitude (MACRO). Serum creatine kinase (CK) was also measured initially and at 4-week intervals throughout the study.
RESULTS: The following variables significantly (p < 0.05) increased: WGT by 47%, ISOKPT, 15%, ISOKTW, 15%; MVC, 36%; EDUR, 21%; TTI, 18%. The following variables did not significantly (p > 0.05) change: FD, MCD, BLK, MACRO, and CK.
CONCLUSIONS: This home exercise program significantly increased strength, endurance, and TTI without apparently adversely affecting the motor units or the muscle, as the EMG and CK variables did not change.
Exercise Therapy – Methods; Isometric Contraction; Physical Endurance; Postpoliomyelitis Syndrome – Rehabilitation; Weight Lifting; Work Capacity Evaluation
MANAGEMENT OF POSTPOLIO SYNDROME
Department of Physical Medicine and Rehabilitation, Mayo Clinic Jacksonville, FL 32224, USA
Mayo Clin Proc, Jul 1997, 72(7), p627-38
Recent research has shed light on the pathogenesis of the postpolio syndrome and has helped explain its symptoms and the rationale for management. The aim of this article is to familiarize physicians with this syndrome. The history, acute infection, definition, and diagnosis are discussed, as well as the various symptoms and their management. People with postpolio syndrome can educate health professionals about this condition and can help others inflicted with this syndrome. Thus far, no cure is available. A correct diagnosis is important, and the physician must realize that severe comorbidities tend to afflict people with this syndrome. Numerous management options are available to help these people enjoy a high quality of life.
Postpoliomyelitis Syndrome – Physiopathology; Postpoliomyelitis Syndrome – rehabilitation
Activities of Daily Living; Autonomic Nervous System Diseases – Physiopathology; Body Weight; Deglutition; Fatigue – Physiopathology; Gait – Physiology; Insomnia – Physiopathology; Muscle Weakness – Physiopathology; Pain – Physiopathology; Poliomyelitis – Diagnosis; Postpoliomyelitis Syndrome – Psychology; Respiratory Insufficiency – Physiopathology; Scoliosis – Physiopathology; Spinal Cord – Physiopathology; Stress, Psychological – Psychology
POST POLIO SYNDROME: AN UPDATE FOR THE PRIMARY HEALTH CARE PROVIDER
Nurse Pract, Jun 1997, 22(6), p133-6, 139, 142-6 passim
Post Polio Syndrome, or PPS, is defined as a clinical syndrome of new weakness, fatigue, and pain in people who have previously recovered from acute paralytic poliomyelitis. Other common symptoms include cold intolerance, dysphagia, dyspnea, and overuse syndromes. PPS afflicts an estimated 50% of Polio survivors, a population estimated at 1.6 million people, and begins roughly 30 years after the acute disease. The main impact of PPS is on mobility related activities affecting one=s daily routine. With an insidious onset, and several differential diagnoses for each symptom, PPS can be difficult to diagnose and to validate. However, once identified, there are treatment plans and many avenues of support for this disabling syndrome. The purpose of this article is to provide an overview of the pathophysiology of both acute paralytic poliomyelitis as well as PPS. This article also reviews the current literature concerning the etiology and pathophysiology of both poliomyelitis and PPS, symptom evaluation and differential diagnoses, and treatment recommendations. The psychosocial impact and care of the client are also identified, and several resources for support and education of both the client and provider are provided.
Adaptation, Psychological; Diagnosis, Differential; Medical History Taking; Patient Care Planning; Patient Education; Postpoliomyelitis Syndrome – Complications; Postpoliomyelitis Syndrome – Diagnosis; Postpoliomyelitis Syndrome – Physiopathology; Postpoliomyelitis Syndrome – Therapy
CHRONIC FATIGUE SYNDROME-AETIOLOGICAL ASPECTS (see comments)
Wolfson Institute of Preventive Medicine, St Bartholomew=s & Royal London School of Medicine & Dentistry, London, UK.
Eur J Clin Invest, Apr 1997, 27(4), p257-67
The chronic fatigue syndrome (CFS) has been intensively studied over the last 40 years, but no conclusions have yet been agreed as to its cause. Most cases nowadays are sporadic. In the established chronic condition there are no consistently abnormal physical signs or abnormalities on laboratory investigation. Many physicians remain convinced that the symptoms are psychological rather than physical in origin. This view is reinforced by the emotional way in which may patients present themselves. The overlap of symptoms between CFS and depression remains a source of confusion and difficulty. But even if all CFS patients were rediagnosed as depressives, this would not negate the possibility of an underlying organic cause for the condition, in view of the growing evidence that depression itself has a physical cause and responds best to physical treatments. There is some evidence both for active viral infection and for an immunological disorder in the CFS. Many observations suggest that the syndrome could derive from residual damage to the reticular activating system (RAS) of the upper brain stem and/or to its cortical projections. Such damage could be produced by a previous viral infection, leaving functional defects unaccompanied by any gross histological changes. In animal experiments activation of the RAS can change sleep state and activate or stimulate cortical functions. RAS lesions can produce somnolence and apathy. Studies by modern imaging techniques have not been entirely consistent, but many magnetic resonance imaging (MRI) studies already suggest that small discrete patchy brain stem and subcortical lesions can often be seen in CFS. Regional blood flow studies by single photon-emission computerized tomography (SPECT) have been more consistent. They have revealed blood flow reductions in many regions, especially in the hind brain. Similar lesions have been reported after poliomyelitis and in multiple sclerosis – in both of which conditions chronic fatigue is characteristically present. In the well-known post-polio fatigue syndrome, lesions predominate in the RAS of the brain stem. If similar underlying lesions in the RAS can eventually be identified in CFS, the therapeutic target for CFS would be better defined than it is at present. A number of logical approaches to treatment can already be envisaged.
Fatigue Syndrome, Chronic – Etiology
LATE FUNCTIONAL DETERIORATION FOLLOWING PARALYTIC POLIOMYELITIS
Kidd D; Howard RS; Williams AJ; Heatley FW; Panayiotopoulos CP; Spencer GT
Department of Neurology, St Thomas= Hospital, London, UK.
QJM, Mar 1997, 90(3), p189-96
Many patients with previous Poliomyelitis develop >post-Polio syndrome= (PPS) in which late functional deterioration follows a period of relative stability. The frequency with which PPS can be attributed to clearly defined causes remains uncertain. We reviewed 283 newly-referred patients with previous Poliomyelitis seen consecutively over a 4-year period; 239 patients developed symptoms of functional deterioration at a mean of 35 (5-65) years after the paralytic illness. Functional deterioration was associated with orthopaedic disorders in 170 cases, neurological disorders in 35, respiratory disorders in 19 and other disorders in 15. Progressive post-Polio muscular atrophy was not observed. Functional deterioration following paralytic poliomyelitis is common, and associated with orthopaedic, neurological, respiratory and general medical factors which are potentially treatable.
Joint Diseases – Etiology; Nervous System Diseases – Etiology; Poliomyelitis – Complications; Respiratory Tract Diseases – Etiology
Adult; Age of Onset; Middle Age; Time Factors
DEPRESSION AND LIFE SATISFACTION IN AGING POLIO SURVIVORS VERSUS AGE-MATCHED CONTROLS: RELATION TO POSTPOLIO SYNDROME, FAMILY FUNCTIONING, AND ATTITUDE TOWARD DISABILITY
Kemp BJ; Adams BM; Campbell ML
Rehabilitation Research and Training Center on Aging With Disability, Rancho Los Amigos Medical Center/University of Southern California, Downey, USA.
Arch Phys Med Rehabil, Feb 1997, 78(2), p187-92
OBJECTIVE: To compare depressive symptoms and life satisfaction in aging Polio survivors with age-matched controls and to relate these outcomes to scores to psychosocial and disability-related variables.
DESIGN: A planned medical, functional, and psychological study with multivariate analyses.
SETTING: A large urban rehabilitation center.
PARTICIPANTS: A volunteer sample of 121 Polio survivors and an age-matched control group of 60 people with similar sociodemographic backgrounds.
MAIN OUTCOMES: Depression as measured by the Geriatric Depression Scale and an 11-item life satisfaction scale.
RESULTS: The prevalence of depressive disorders was not significantly different in the two groups, although the postpolio group tended to have more symptomology and an overall depressive disorder prevalence of 28%. Some life satisfaction scale scores were significantly lower in the postpolio group, especially those concerned with health. People with postpolio syndrome scored significantly higher on depression scales and lower on some life satisfaction scales than people with a history of Polio but without postpolio syndrome. Several psychosocial variables, most notably family functioning and attitude toward disability, helped to mediate this effect. Among people with significant depression, there was little, evidence of adequate treatment in the community. CONCLUSIONS: Postpolio by itself does not relate to higher depression scores of lower life satisfaction. Postpolio syndrome has some relation to depression, but family functioning and attitude toward disability are more important. There is a need for better community-based psychological services.
Attitude to Health; Depressive Disorder – Etiology; Personal Satisfaction; Postpoliomyelitis Syndrome – Complications; Postpoliomyelitis Syndrome – Psychology
Activities of Daily Life; Aged; Aged, 80 and over; Family; Middle Age; Psychosocial Deprivation
EFFECTS OF ORAL SUPPLEMENTATION OF COENZYME Q10 ON 31P-NMR DETECTED SKELETAL MUSCLE ENERGY METABOLISM IN MIDDLE-AGED POST-POLIO SUBJECTS AND NORMAL VOLUNTEERS
Mizuno M; Quistorff B; Theorell H; Theorell M; Chance B
Department of Medical Biochemistry and Genetics, Panum Institute, University of Copenhagen, Denmark.
Mol Aspects Med, 1997, 18 Suppl, pS291-8
The effects of oral supplementation of 100 mg coenzyme Q10 (CoQ10) for 6 months on muscle energy metabolism during exercise and recovery were evaluated in middle-aged post-polio (n = 3) and healthy subjects (n = 4) by the use of phosphorus-31 nuclear magnetic resonance spectroscopy. The metabolic response to isometric plantar flexion at 60% of maximal voluntary contraction force (MVC) for 1.5 min was determined in gastrocnemius muscles before, after 3- (3MO) and 6-month (6MO) of CoQ10 supplementation. The MVC of plantar flexion was unchanged following CoQ10 supplementation. The resting Pi/PCr ratio in gastrocnemius muscles of all subjects decreased after 3MO- and 6MO-CoQ10 (P < 0.05). The post-Polio individuals showed a progressive decrease in this ratio, while less pronounced changes were observed in the control subjects. Similarly, the post-Polio individuals showed a lower Pi/PCr ratio at the end of 60% MVC in both 3MO- and 6MO-CoQ10, whereas no change in the ratio was observed in the control subjects. A less pronounced decrease in muscle pH was observed at the end of 60% MVC in both 3MO- and 6MO-CoQ10 in the post-Polio individuals, but not in the control subjects. No systematic difference in end-exercise ATP was observed between the three phases in both groups. The half-time recovery for PCr decreased in all subjects after 6MO-CoQ10 supplementation (P< 0.05). The results suggest that CoQ10 supplementation affects muscle energy metabolism in post-Polio individuals to a greater extent than in control subjects. The mechanism for this effect is not clear, but may involve an effect of CoQ10 on peripheral circulation in the calf muscles, its action in mitochondrial oxidative phosphorylation and/or its antioxidant potential.
Energy Metabolism – Drug Effects; Muscle, Skeletal – Drug Effects; Phosphocreatine – Metabolism; Postpoliomyelitis Syndrome – Drug Therapy; Ubiquinone – Analogs and Derivatives
Administration, Oral; Adult; Antioxidants – Pharmacology; Antioxidants – Therapeutic Use; Endothelium – Drug Effects; Endothelium – Metabolism; Exertion; Hydrogen-Ion Concentration; Isometric Contraction; Middle Age; Muscle, Skeletal – Metabolism; Nuclear Magnetic Resonance; Oxidative Phosphorylation – Drug Effects; Phosphorus Isotopes; Postpoliomyelitis Syndrome – Metabolism; Rest; Ubiquinone – Administration and Dosage; Ubiquinone – Pharmacology; Ubiquinone – Therapeutic Use
SLEEP AND NEUROMUSCULAR DISORDERS
Neurology Service, Veterans Affairs Medical Centre, Syracuse, New York, USA
Neurol Clin, Nov 1996, 14(4), p791-805
Polysomnographic evaluation in the sleep laboratory is recommended for patients with neuromuscular disorders who develop symptoms and signs of sleep-wake abnormality or nocturnal respiratory failure. Nocturnal sleep-related ventilatory alterations may occur in disproportion to the severity of the neuromuscular disorder. Diaphragmatic paralysis occurring in the context of a neuromuscular disorder is often an overlooked complication. Failure to thrive, daytime tiredness, and incapacitating fatigue may be the result of a potentially correctable sleep-related abnormality and not due to relentless progression of the neuromuscular condition. Application of CPAP and BiPAP and administration of supplemental oxygen are relatively simple, noninvasive, ambulatory, therapeutic maneuvers that may correct sleep-related ventilatory alterations in patients with neuromuscular disorders.
Neuromuscular Diseases – Physiopathology; Sleep – Physiology
Amyotrophic Lateral Sclerosis – Physiopathology; Muscular Dystrophy – Physiopathology; Myasthenia Gravis – Physiopathology; Myotonia Atrophica – Physiopathology; Phrenic Nerve – Physiopathology; Postpoliomyelitis Syndrome – Physiopathology; Respiration Disorders – Physiopathology
ENDURANCE TRAINING EFFECT ON INDIVIDUALS WITH POSTPOLIOMYELITIS
Ernstoff B; Wetterqvist H; Kvist H; Grimby G
Department of Rehabilitation Medicine, Goteborg University, Sweden
Arch Phys Med Rehabil, Sep 1996, 77(9), p843-8
OBJECTIVE: To determine the effects of an endurance training program on the exercise capacity and muscle structure and function in individuals with postpolio syndrome.
DESIGN: Preexercise and postexercise testing was performed with muscle strength evaluations using isokinetic testing as well as hand-held Myometer. Muscle fatigue was determined by use of isokinetic testing, and endurance was determined by exercise testing. Enzymatic evaluation was performed with muscle biopsies taken at the same site; preexercise and postexercise muscle cross-sectional area was measured by computed tomography. Disability and psychosocial evaluation was performed by a Functional Status Questionnaire.
SUBJECTS: Seventeen postpolio subjects ranging in age from 39 to 49 years volunteered for a 6-month combined endurance and strength training program. They had a history of acute poliomyelitis at least 25 years earlier and were able to walk with or without aid.
INTERVENTION: Twelve of the subjects (mean age 42 years) completed the program, attending an average of 29 sessions, which were offered for 60 minutes twice a week.
MAIN OUTCOME MEASURES: Strength, endurance, enzymatic activity, and cross-sectional area were measured 3 months before the beginning of training, just before training, and at the completion of the exercise program.
RESULTS: Knee extension was reduced to an average of 60% of control values and did not change with training. Strength measured by a hand-held Myometer increased significantly for elbow flexion, wrist extension, and hip abduction. Exercise test on a bicycle-ergometer showed significant reduction (6 beats/min) in heart rate at 70W and increase (12 beats/min) in maximal heart rate with training. The training program could be performed without major complications and resulted in an increase in muscle strength in some muscle groups and in work performance with respect to heart rate at submaximal work load.
Physical Endurance – Physiology; Poliomyelitis – Rehabilitation Adult; Exercise Test; Isometric Contraction – Physiology; Middle Age; Muscle Weakness – Physiopathology; Muscle, Skeletal – Radiography; Physical Education and Training; Poliomyelitis – Physiopathology; Tomography, X-Ray Computed
FATIGUE OF CHRONICALLY OVERUSED MOTOR UNITS IN PRIOR POLIO PATIENTS
Grimby L; Tollback A; Muller U; Larsson L
Department of Neurology, Karolinska Hospital, Stockholm, Sweden.
Muscle Nerve, Jun 1996, 19(6), p728-37
This study was undertaken to investigate the mechanisms underlying fatigue of chronically overused motor units (MUs). The force of the tibialis anterior muscle (TA) and the firing properties of single MUs were studied during prolonged maximum voluntary effort in 10 prior Polio patients selected such that daily living required all residual TA power. Almost all TA fibers were hypertrophic type I. Activities of intermyofibrillar succinate dehydrogenase (SDH) and calcium-stimulated myofibrillar adenosine triphosphatase (ATPase) were measured in single TA fibers from a representative patient. Neither insufficient motoneuron activation nor peripheral blocking of the electrical impulse played a major role in the loss of force during prolonged contraction or for slow recovery after contraction. The ration of SDH to calcium-stimulated ATPase, representing the relation between energy resynthesis and energy utilization, was significantly (P < 0.001) lower in prior Polio patients (0.230 +/- 0.097) type I fibers.
Motor Neurons – Physiology; Muscle Contraction; Muscle Fatigue; Muscle, Skeletal – Physiopathology; Poliomyelitis – Physiopathology
Aged; Analysis of Variance; Biopsy; Ca(2+) – Transporting ATPase – Analysis; Electromyography; Foot – Innervation; Leg – Innervation; Longitudinal Studies; Middle Age; Movement; Muscle Fibers – Enzymology; Muscle Fibers – Physiology; Muscle, Skeletal – Innervation; Muscle, Skeletal – Pathology; Poliomyelitis – Pathology; Reference Values; Succinate Dehydrogenase – Analysis; Time Factors; Walking
RECOGNIZING POST-POLIO SYNDROME
Bartfield H; Ma D
Post-Polio Syndrome Research Center, New York University Medical Center, NY, USA.
Hosp Pract (Off Ed), May 15 1996, 31(5), 101-3, 107 passim
The disorder consists of fatigue accompanied by new muscle weakness and muscle pain or, for patients whose acute Polio had included bulbar involvement, new difficulty in swallowing or change in voice. The epidemiology remains unclear, fueling anxiety among Polio survivors. Yet its course is not drastically progressive, and impairment is usually limited.
Postpoliomyelitis Syndrome – Diagnosis
Disabled; Electromyography; Middle Age; Physical Therapy; Postpoliomyelitis Syndrome – Epidemiology; Postpoliomyelitis Syndrome – Physiopathology; Postpoliomyelitis Syndrome – Therapy; United States – Epidemiology
PATTERNS OF DENERVATION IN CLINICALLY UNINVOLVED LIMBS IN PATIENTS WITH PRIOR POLIOMYELITIS
Bromberg MB; Waring WP; Sanders PL
Department of Neurology, University of Utah Health Sciences Center, Salt Lake City, Utah, USA.
Electromyogr Clin Neurophysiol, Mar 1996, 36(2), p107-11
In most cases of poliomyelitis, motor neuron death is extensive. In mild cases primarily involving one limb, the pattern of neuron death has not been fully determined. Nine mildly affected patients were studied systematically for electrophysiologic evidence of denervation by sampling one muscle in each limb. Fiber density was found to be increased in all but one muscle. Needle electromyography and turns and amplitude plots of the interference pattern were abnormal in all muscles in weak limbs and the majority of muscles in unaffected limbs. Turns: amplitude ratios were lower in muscles in weak limbs and greater in muscles in unaffected limbs in a pattern suggesting a horizontal gradation of motor neuron death across the spinal cord. This may be attributed to axonal transport of the virus. The horizontal pattern of sub clinical involvement in mild cases of poliomyelitis was compared and found to be similar to reported patterns of progression of limb weakness in amyotrophic lateral sclerosis type motor neuron disease.
Denervation; Extremities – Physiopathology; Poliomyelitis – Physiopathology
Adult; Electromyography; Middle Age; Muscles – Physiopathology
SEXUAL FUNCTIONING AMONG WOMEN WITH PHYSICAL DISABILITIES
Nosek MA; Rintala DH; Young ME; Howland CA; Foley CC; Rossi D; Chanpong G
Department of Physical Medicine and Rehabilitation, Baylor College of Medicine, Houston, TX 77030, USA.
Arch Phys Med Rehabil, Feb 1996, 77(2), p107-15
OBJECTIVE: Three a priori hypotheses were tested: (1) There are significant differences in sociosexual behaviours of women with physical disabilities compared with women without disabilities; (2) the sexual functioning of women with disabilities is significantly related to age at onset of disability; (3) psychological factors explain more of the variance in the sexual functioning of women with physical disabilities than do disability, social and environmental factors.
DESIGN: Case-comparison study using written survey.
SETTING: General community.
PARTICIPANTS: The questionnaire was mailed to 1,150 women with physical disabilities who were recruited as volunteers or through independent living centers. Each woman gave a second copy of the questionnaire to an able-bodied friend, which comprised the comparison group. The response rate was 45%, with 475 cases and 425 comparisons eligible to participate. The most common disability type was spinal cord injury (24%), followed by Polio (18%), muscular dystrophy (11%), cerebral palsy (11%), multiple sclerosis (10%), joint disorders (7%), and skeletal abnormalities (5%).
MAIN OUTCOME MEASURES: Sexual-functioning, consisting of four factors: (1) sexual desire, (2) sexual activity, (3) sexual response, (4) sexual satisfaction.
RESULTS: Highly significant differences were found in level of sexual activity (p = 0.000001), response (p = 0.000009), and satisfaction (p = 0.000001) between women with and without disabilities. No significant differences were found between groups on sexual desire. Severity of disability was not significantly related to level of sexual activity. CONCLUSIONS: Psychological and social factors exert a strong impact on the sexual functioning of women with physical disabilities. Further investigation is needed of the effect of social environment on development of self-esteem and sexual self-image, and how these influences affect levels of sexual functioning in women with physical disabilities.
Disabled; Psychosexual Dysfunctions – Etiology; Sex Disorders – Etiology
Adolescence; Adult; Body Image; Case-Control Studies; Knowledge, Attitudes, Practice; Middle Age; Psychosexual Dysfunctions – Psychology; Questionnaires; Regression Analysis; Risk Factors
LOW-INTENSITY, ALTERNATE-DAY EXERCISE IMPROVES MUSCLE PERFORMANCE WITHOUT APPARENT ADVERSE EFFECT IN POSTPOLIO PATIENTS
Agre JC; Rodriquez AA; Franke TM; Swiggum ER; Harmon RL; Curt JT
Department of Rehabilitation Medicine, University of Wisconsin – Madison Medical School, 53791, USA
Am J Phys Med Rehabil, Jan-Feb 1996, 75(1), p50-8
The purpose of this study was to examine the effect of a low-intensity, alternate-day, 12 wk quadriceps muscle-strengthening exercise program on muscle strength and muscle and motor unit integrity in 12 postpolio patients. Patients performed six to ten repetitions of a 5-s duration knee extension exercise with ankle weights. After completing six repetitions, patients rated the perceived exertion (RPE) on the exercised muscle. The patient continued repetitions until RPE was >/= 17 or ten repetitions were performed. The weight was increased the next exercise day whenever the RPE was < 17 after ten repetitions. Before and after the training program, median macroamplitude as well as jitter and blocking were determined electromyographically (EMG), serum creatine kinase (CK) was measured, and quadriceps muscle strength was assessed. The ankle weight lifted after 2 wk of training and at the end of the program were also recorded. Although the ankle weight lifted at the end of the program significantly (P < 0.05) increased from a mean +/- SD of 7.1 +/- 2.7 to 11.2 +/- 4.7 kg, the dynametrically determined muscle strength measures did not significantly (P > 0.05) increase. The EMG and the serum CK variables also did not significantly (P > 0.05) change as a result of the exercise program. We conclude that performance was improved, as demonstrated by an increase in the amount of weight the patients lifted in the exercise program. No evidence was found to show that this program adversely affected the motor units or the muscle as the EMG and CK did not change.
Exercise Therapy – Methods; Muscle Contraction; Postpoliomyelitis Syndrome – Rehabilitation
Adult; Creatine Kinase – Blood; Electromyography; Exertion; Middle Age; Postpoliomyelitis Syndrome – Physiopathology; Weight Lifting
AN OPEN TRIAL OF PYRIDOSTIGMINE IN POST-POLIOMYELITIS SYNDROME
Trojan DA; Cashman NR
Department of Neurology, Montreal Neurological Institute and Hospital, McGill University, Quebec, Canada.
Can J Neurol Sci, Aug 1995, 22(3), p223-7
BACKGROUND: One of the major symptoms of postpoliomyelitis syndrome (PPS) is disabling generalized fatigue. Subjects with PPS also report muscle fatiguability and display electrophysiologic evidence of anticholinesterase-responsive neuromuscular junction transmission defects, suggesting that anticholinesterase therapy may be useful in the management of disabling fatigue.
METHODS: We initiated an open trial of the oral anticholinesterase pyridostigmine, up to 180 mg per day, in 27 PPS patients with generalized fatigue and muscle fatiguability. Response to pyridostigmine was assessed with the Hare fatigue scale, the modified Barthel index for activities of daily living, and a modified Klingman mobility index.
RESULTS: Two patients could not tolerate the medication. After one month of therapy, 16 patients (64%) reported a reduction in fatigue on the Hare fatigue scale; three of 16 showed improvement on the modified Barthel index for activities of daily living, and two of 16 experienced improvement on a modified Klingman mobility index. Pyridostigmine responders were significantly more fatigued than non-responders on the pre-treatment Hare score, but were not significantly different with regard to age, sex, age at acute poliomyelitis, or severity of acute poliomyelitis.
CONCLUSIONS: Pyridostigmine may be useful in the management of fatigue in selected patients with PPS. Response to pyridostigmine may be predicted by severity of pre-treatment fatigue.
Fatigue – Drug Therapy; Poliomyelitis – Drug Therapy; Pyridostigmine Bromide – Therapeutic Use
Adult; Age of Onset; Aged; Middle Age; Motor Activity – Drug Effects; Treatment Outcome
THE POST-POLIO SYNDROME AS AN EVOLVED CLINICAL ENTITY. DEFINITION AND CLINICAL DESCRIPTION.
Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
Ann N Y Acad Sci, May 25 1995, 735, p68-80
Post-Polio syndrome (PPS) refers to the new neuromuscular symptoms that occur at least 15 years after stability in patients with prior acute paralytic Polio-myelitis. 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 disfunction 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.
Fatigue – Etiology; Postpoliomyelitis Syndrome – Diagnosis; Postpoliomyelitis Syndrome – Epidemiology; Postpoliomyelitis Syndrome – Pathology; Postpoliomyelitis Syndrome – Physiopathology; Risk Factors
THE ROLE OF EXERCISE IN THE PATIENT WITH POST-POLIO SYNDROME
Department of Rehabilitation Medicine, University of Wisconsin – Madison Medical School 53792, USA
Ann N Y Acad Sci, May 25 1995, 753, p321-34
Recent studies have shown that judicious exercise can improve muscle strength, cardiorespiratory fitness, and the efficiency of ambulation in post-Polio patients. It may also add to the patient=s sense of well-being. These benefits appear to occur when the patients stay within reasonable bounds while exercising in order to avoid overuse problems. In particular, the patients should be instructed to avoid activities that cause increasing muscle or joint pain or excessive fatigue, either during or after the exercise program. The literature indicates that exercise within these constraints leads to a number of beneficial physiologic and psychologic adaptations in patients with post-Polio syndrome. Judicious exercise should be viewed as important adjuvant in the overall therapeutic program of the patient. Patients seen in post-Polio clinics frequently complain of new fatigue, weakness, muscle pain, and/or joint pain. The most frequent complaints involving activities of daily living include new difficulties with walking or stair climbing. The therapeutic benefit od exercise in these patients to minimize of reverse decline in function is an important question frequently asked by patietns with post-Polio syndrome. In the general population, physical activity is known to be an important adjunct to good health, bestowing both physiologic and psychologic benefits leading to a reduction in the risk to develop a number of serious ailments as well as leading to better psychological adjustment. On the other hand, limitation in physical activity results in a number of deletorious effects. Patients with post-Polio syndrome have unique problems, however, which need to be considered when prescribing an exercise program for an individual patient. A number of functional etiologies for declining function have been hypothesized including disuse weakness, overuse weakness, weight gain, and chronic weakness. Because of the variability in which the motor neurons to different muscle groups may have been effected in a particular patient, both asymmetric and scattered weakness may be present. The challenge in prescribing exercise for the patient with post-Polio syndrome comes in recognizing these unique factors in each patient and modifying the prescription accordingly. One must protect muscles and joints experiencing the adverse effects of over use or body areas with very significant chronic weakness (in general, in areas where the muscles have less than antigravity strength on manual muscle testing) while exercising those body areas experiencing the deleterious effects of disuse. Weight gain is to be avoided if at all possible in this population, because increased weight only leads to further difficulty in the performance of daily activities.
Activities of Daily Living; Body Weight; Cardiovascular System – Physiology; Exercise Therapy; Fatigue; Muscles – Physiology; Patient Compliance; Postpolio Syndrome – Physiopathology
POST-POLIO SYNDROME IN TWINS AND THEIR SIBLINGS. EVIDENCE THAT POST-POLIO SYNDROME CAN DEVELOP IN PATIENTS WITH NONPARALYTIC POLIO.
Nee L; Dambrosia J; Bern E; Eldridge R; Dalakas MC
National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA.
Ann N Y Acad Sci, May 25 1995, 753, p378-80
Poliomyelitis – Complications; Postpoliomyelitis Syndrome – Genetics
Follow-up Studies; Twins