A relationship between preceding acute paralytic poliomyelitis and the later development of motor neuron disease has only occasionally been suggested since it was first postulated by Charcot in 1875. The authors recently experienced a 20-year-old male who was considered to have postpoliomyelitis muscular atrophy. We report this case in view of its rarity and necessity of differential diagnosis from other neuromuscular disorders. Clinical presentation included slowly progressive muscle wasting of left thigh for 4 years, mild weakness of left arm and both thigh, intermittent fasciculation, and previous history of acute paralytic poliomyelitis. Electromyographic findings showed fibrillation potentials, positive sharp waves, fasciculations, giant motor unit potentials and reduced interference patterns. Muscle biopsy revealed scattered small angulated fibers, individual myofiber degeneration and mild inflammatory cell infiltration.
Arm ; Biopsy ; Diagnosis, Differential ; Fasciculation ; Humans ; Male ; Motor Neuron Disease ; Poliomyelitis ; Postpoliomyelitis Syndrome* ; Thigh ; Young Adult

OBJECTIVE: To evaluate the psychometric properties of the Fatigue Severity Scale (FSS), the Fatigue Impact Scale (FIS), and the Multidimensional Fatigue Inventory (MFI-20) in persons with late effects of polio (LEoP). More specifically, we explored the data completeness, scaling assumptions, targeting, reliability, and convergent validity. METHODS: A postal survey including FSS, FIS, and MFI-20 was administered to 77 persons with LEoP. Responders received a second survey after 3 weeks to enable test-retest reliability analyses. RESULTS: Sixty-one persons (mean age, 68 years; 54% women) responded to the survey (response rate 79%). Data quality of the rating scales was high (with 0%–0.5% missing item responses), the corrected item-total correlations exceeded 0.4 and the scales showed very little floor or ceiling effects (0%–6.6%). All scales had an acceptable reliability (Cronbach’s α≥0.95) and test-retest reliability (intraclass correlation coefficient, ≥0.80). The standard error of measurement and the smallest detectable difference were 7%–10% and 20%–28% of the possible scoring range. All three scales were highly correlated (Spearman’s correlation coefficient r(s)=0.79–0.80; p < 0.001). CONCLUSION: The FSS, FIS, and MFI-20 exhibit sound psychometric properties in terms of data completeness, scaling assumptions, targeting, reliability, and convergent validity, suggesting that these three rating scales can be used to assess fatigue in persons with LEoP. As FSS has fewer items and therefore is less time consuming it may be the preferred scale. However, the choice of scale depends on the research question and the study design.
Data Accuracy ; Fatigue* ; Humans ; Poliomyelitis* ; Postpoliomyelitis Syndrome ; Psychometrics* ; Rehabilitation ; Reproducibility of Results ; Weights and Measures*

OBJECTIVE: To investigate the clinical feature and quantitative electromyographic (QEMG) findings in the patients with post-polio syndrome (PPS). METHOD: Eleven patients who had clinical evidences of antecedent poliomyelitis were evaluated with standardized clinical history, physical examination and QEMG study. If a patient had fulfilled provisional criteria for PPS, he was regarded as PPS and six patients had fulfilled the criteria. Other patients were treated as control group with stable poliomyelitis. Manual muscle testing and needle EMG study including quantitative motor unit analysis was performed at the tibialis anterior and vastus medialis muscles. The existence of abnormal spontaneous activity and parameters of quantitative motor unit analysis, mean duration and amplitude of motor unit action potentials (MUAPs), were compared between PPS and control groups. The correlation between the muscle strength and parameters of QEMG was investigated. RESULTS: Abnormal spontaneous activities were noted in 4 out of 11 patients (36.4%) and three of these 4 patients were PPS. Mean duration and amplitude of MUAPs of tibialis anterior and vastus medialis muscles were not different significantly between the PPS and stable poliomyelitis group (p>0.05). The parameters of MUAPs were poorly correlated with muscle strength. CONCLUSION: Distribution of abnormal spontaneous activities and parameters of QEMG study were not different in PPS and stable poliomyelitis patients. QEMG study may not have additional benefit in differentiating PPS from stable poliomyelitis.
Action Potentials ; Humans ; Muscle Strength ; Muscles ; Needles ; Physical Examination ; Poliomyelitis ; Postpoliomyelitis Syndrome* ; Quadriceps Muscle

Dermatomyositis (DM) is an idiopathic inflammatory myopathy, characterized by inflammation of the proximal skeletal muscles and typical skin manifestations, which results in symmetric muscle weakness. A 43-year-old man was presented with skin rash and left leg weakness, and he had a history of poliomyelitis. Initially, he was diagnosed as having post-polio syndrome (PPS) due to unilateral muscle weakness and a result of an the electromyography (EMG), which had shown patterns of PPS. After 4 months with conservative therapy for PPS, weakness of bilateral upper arms had developed and skin rashes on his entire body had aggravated and progressed. He was diagnosed as having dermatomyositis, based on elevated muscle enzyme levels, typical skin rashes, and typical EMG findings, which indicated muscle disease. When a patient with previous poliomyelitis has a newly developed muscle weakness or pain, we should consider various possible causes other than PPS.
Adult ; Arm ; Dermatomyositis* ; Diagnosis, Differential* ; Electromyography ; Exanthema ; Humans ; Inflammation ; Leg ; Muscle Weakness* ; Muscle, Skeletal ; Myositis ; Poliomyelitis* ; Postpoliomyelitis Syndrome* ; Skin Manifestations

OBJECTIVE: In patients with a history of acute paralytic poliomyelitis, late progressive muscle weakness, fatigue, pain may arise, a symptom complex of known as post-poliomyelitis syndrome (PPS). Dysphagia may also develop in some PPS patients. The purpose of this study was to assess the presence of is swallowing difficulty in polio survivors and to describe the nature of the swallowing difficulty. METHOD: Polio survivors answered the questionnaire pertaining to swallowing function and received a videofluroscopic evaluation of the oral and pharyngeal phases using 3 consistencies of material: liquid barium; semisolid barium paste; boiled yolk of an egg coated with barium. RESULTS: Of the 16 subjects, 8 had subjective symptoms of swallowing difficulties. All of the 6 PPS patients, regardless of whether they had symptoms of swallowing difficulties, had some abnormal oropharyngeal function through video fluoroscopic swallowing study. CONCLUSION: In patients with post-polio syndrome, there is progressive deterioration of swallowing functions similar to that in the muscles of the limbs. This swallowing dysfunction is not related with their subjective symptoms.
Barium ; Deglutition Disorders ; Deglutition* ; Extremities ; Fatigue ; Humans ; Muscle Weakness ; Muscles ; Ovum ; Poliomyelitis* ; Postpoliomyelitis Syndrome ; Surveys and Questionnaires ; Survivors*

The Postpoliomyelitis syndrome (PPS) is characterized by new neuromuscular symptoms such as muscle weakness, joint pain, fatigue, and occasional new neuromuscular impairments. Although the development of PPS shows individual differences, it usually occurs many years after the recovery from an acute paralytic poliomyelitis. The purpose of this study was to investigate the incidence and the clinical features of PPS. The subjects were 33 patients(26 males and 7 females) who had partial recoveries of motor function, ten or more years of functional stabilities, and residual muscle atrophies. The mean age was 36.8?7.7 years with the range of 23 - 59 years. The average onset age of acute poliomyelitis was 2.3 - 1.5 years. The questionnaires asking their current physical conditions and three major categories of PPS symptoms, weakness, fatigue, and pain were used. As the sequelae of poliomyelitis, 66.7% of the subjects showed scoliosis and 39.4% had claw toes. Thirty six percents of the subjects were using orthoses including wheelchair, KAFO, and crutches. According to the questionnaire study, 72.5% of subjects had at least one of the three major symptoms. And 36.4% of them reported all of three categories of PPS symptoms. The onset age of the new neuromuscular symptoms was 34.2?8.3 years which was about 32 years after the original acute poliomyelitis. Further researches to investigate the importance and the impacts of proper managements and to develop educational plans for the PPS including exercise programs, cardiopulmonary and energy-conservation trainings are recommended.
Age of Onset ; Arthralgia ; Crutches ; Fatigue ; Hammer Toe Syndrome ; Humans ; Incidence ; Individuality ; Male ; Muscle Weakness ; Muscular Atrophy ; Orthotic Devices ; Poliomyelitis* ; Postpoliomyelitis Syndrome ; Surveys and Questionnaires ; Scoliosis ; Wheelchairs

Postpoliomyelitis syndrome is a common neurological disorder that occurs in patients who have experienced paralytic poliomyelitis. Recently, as a result of vaccination against poliovirus, incidence of poliomyelitis is exceedingly low. However, many patients with postpolio syndrome may encounter anesthesia when undergoing surgery, such as for correction of foot deformity and other operations. We report on a 45-year-old woman who experienced paralysis of her contralateral limb after operation on the left foot under spinal anesthesia. Postoperative electromyography/nerve conduction study (EMG/NCS) was performed in order to determine the cause of paralysis. Motor power of the sequelae involved leg showed improvement with time and recovered fully to the preoperative level at six months after the index operation. A precise evaluation, including a physical examination and EMG/NCS, should be performed preoperatively when spinal anesthesia is planned for postpolio syndrome patients.
Anesthesia ; Anesthesia, Spinal ; Congenital Abnormalities* ; Extremities* ; Female ; Foot Deformities ; Foot* ; Humans ; Incidence ; Leg ; Middle Aged ; Nervous System Diseases ; Paralysis ; Physical Examination ; Poliomyelitis* ; Poliovirus ; Postpoliomyelitis Syndrome ; Vaccination

OBJECTIVE: To evaluate the presence of swallowing difficulties in patients with post-poliomyelitis syndrome (PPS) by characterizing their swallowing patterns with videofluoroscopic swallowing study. METHOD: Eleven patients diagnosed with PPS were enrolled. All subjects answered the self assessment questionnaire for swallowing difficulty and gastric symptoms. We assessed the ability of tongue control, the oral transit time, cricopharyngeal opening time, and pharyngeal transit time. We also assessed the presence of aspiration or penetration and the amount of residue in the vallecular and pyriform sinus by four grade scales. A gastroenterologist examined esophagus, stomach and duodenum through an esophagogastroduodenoscopy. RESULTS: Six patients complained swallowing difficulty and nine patients showed symptoms of esophageal regurgitation. Although finding of penetration or aspiration was not seen, all patients showed swallowing dysfunction in the videofluoroscopic swallowing study. In the esophagogastroduodenoscopy, chronic superficial gastritis was observed in all enrolled patients (n=11) and reflux esophagitis was found in 4 patients (36%). CONCLUSION: About half patients with PPS complained of dysphagia. Routine evaluation of dysphagia with videofluoroscopic swallowing study and esophagogastroduodenoscopy is needed in all patients with PPS due to the low reliability in their subjective symptom.
Deglutition ; Deglutition Disorders ; Duodenum ; Endoscopy ; Endoscopy, Digestive System ; Esophagitis, Peptic ; Esophagus ; Gastritis ; Humans ; Postpoliomyelitis Syndrome ; Pyriform Sinus ; Surveys and Questionnaires ; Self-Assessment ; Stomach ; Tongue ; Weights and Measures

OBJECTIVE: To obtain information on the socioeconomic, medical, and functional status of polio survivors, and to use these results as the preliminary data for establishing the middle-aged cohort of polio survivors. METHODS: The subjects were recruited based on the medical records of multiple hospitals and centers. They were assessed through a structured questionnaire over the phone. Post-poliomyelitis syndrome (PPS) was identified according to the specified diagnostic criteria. Differences between polio survivors with or without PPS were evaluated, and the risk factors for PPS were analyzed by the odds ratio (OR). RESULTS: Majority of polio survivors were middle-aged and mean age was 51.2+/-8.3 years. A total of 188 out of 313 polio survivors met the adopted criteria for PPS based on the symptoms, yielding a prevalence of 61.6%. Mean interval between acute poliomyelitis and the development of PPS was 38.5+/-11.6 years. Female gender (OR 1.82; confidence interval [CI] 1.09-3.06), the age at onset of poliomyelitis (OR 1.75; CI 1.05-2.94), the use of orthoses or walking aids (OR 2.46; CI 1.44-4.20), and the history of medical treatment for paralysis, pain or gait disturbance (OR 2.62; CI 1.52-4.51) represented independent risk factors for PPS. CONCLUSION: We found that the majority of Korean polio survivors entered middle age with many medical, functional, and social problems. Female gender, early age of onset of poliomyelitis, the use of orthoses or walking aids, and the history of medical treatment for paralysis, pain or gait disturbance were identified as the significant risk factors for PPS. A comprehensive and multidisciplinary plan should be prepared to manage polio survivors considering their need for health care services and the risk factors for late effects, such as PPS.
Age of Onset ; Cohort Studies ; Surveys and Questionnaires* ; Delivery of Health Care ; Female ; Gait ; Humans ; Interviews as Topic* ; Medical Records ; Middle Aged ; Odds Ratio ; Orthotic Devices ; Paralysis ; Poliomyelitis* ; Postpoliomyelitis Syndrome* ; Prevalence ; Risk Factors* ; Social Problems ; Survivors* ; Walking ; Surveys and Questionnaires

OBJECTIVE: This study was performed to evaluate the prevalence of new neuromuscular symptoms and disabilities and the psychological characteristics-depressioin, anxiety, type A behavior, loneliness, and also to determine any relationships between physical and neuropsychological characteristics in a group of post-polio syndrome (PPS). METHOD: By 70 answered questionnaire, the polio survivors were grouped into PPS and Non- PPS. This questionnaire consisted of questions about acute polio problems; new health problems, fatigue severity scale, visual analog scale, weakness scale, Frenchay activity index, ambulation disability index; socio-economic problems; neuro-psychological inventories, Beck depression index, Spielberger state-trait anxiety inventory, revised UCLA loneliness scale, type A personality score. RESULTS: The median time from polio to the onset of new health problems was 27.6 years. Fatigue, muscle and/or joint pain, weakness in previously affected and unaffected muscles were most common newly appearing problems. The symptoms of PPS was consistent with the distribution of the anterior horn cell; spinal cord, brain stem, cerebral hemisphere, Reticular Activating System (RAS). Neuro-psychological evaluations revealed that fatigue scale was correlated with depression, type A personality. CONCLUSION: In PPS group, pain, weakness, fatigue, autonomous symptoms, decreased concentration were more serious than in Non-PPS group. The fatigue in PPS group was correlated with type A personality, depression, sleep disturbance and concentration problem.
Anterior Horn Cells ; Anxiety ; Arthralgia ; Brain Stem ; Cerebrum ; Depression ; Equipment and Supplies ; Fatigue ; Humans ; Korea* ; Loneliness ; Muscle Fatigue ; Muscles ; Poliomyelitis ; Postpoliomyelitis Syndrome* ; Prevalence ; Surveys and Questionnaires ; Spinal Cord ; Survivors ; Type A Personality ; Visual Analog Scale ; Walking