11/40. Successful treatment of a postpolio tinnitus with type a botulinum toxin. OBJECTIVES/HYPOTHESIS: Objective tinnitus is a symptom often observed in patients with chronic hyperactivity of masticatory muscles. methods: We report here the unusual case of a 63-year-old woman who developed a distressing tinnitus related to contractions of reinnervated masticatory muscles. This reinnervation process was caused by a postpolio syndrome that gave rise to an acoustic resonance phenomenon transmitted to the middle ear as an audible sound. RESULTS: The tinnitus was successfully treated with electromyography-guided intramuscular injections of type A botulinum toxin. CONCLUSION: The intramuscular injection of botulinum toxin was found to be effective in relieving severe and disabling postpolio tinnitus. ( info) |
12/40. gait evaluation of an automatic stance-control knee orthosis in a patient with postpoliomyelitis. OBJECTIVE: To determine gait differences in a subject ambulating with a knee-ankle-foot orthosis (KAFO) with a locked knee joint versus an automatic stance-control knee joint. DESIGN: Single-subject crossover design. SETTING: Tertiary rehabilitation facility with a motion analysis laboratory. PARTICIPANT: A 61-year-old ambulatory male volunteer with postpoliomyelitis walking with a stance-control KAFO. INTERVENTIONS: Instrumented gait analysis and Physiological Cost Index in the locked knee and stance-control modes. MAIN OUTCOME MEASURE: Differences in gait parameters. RESULTS: On the braced limb, stance-control mode showed a near-normal knee flexion wave in swing, reduced pelvic retraction and rotational excursion, and improved hip power generation. On the nonbraced limb, the stance-control mode allowed elimination of vaulting, reduction in abnormal ankle and hip power generation, increased knee power absorption, and more typical quadriceps activation. There was a trend toward improved energy efficiency in the stance-control mode. CONCLUSIONS: Use of a stance-control knee joint in a KAFO appears to improve gait biomechanics and improve energy efficiency compared with a locked knee. ( info) |
13/40. Post-poliomyelitis syndrome: case report and review of the literature. INTRODUCTION: postpoliomyelitis syndrome (PPS) refers to the new neuromuscular symptoms that occur in patients years after their acute poliomyelitis has stabilised. PPS cases seen now are probably related to the poliomyelitis epidemics of the 1940s and 1950s. CLINICAL PICTURE AND INVESTIGATION: A 57-year-old Chinese man with a history of poliomyelitis affecting both lower limbs presented with left upper limb weakness. physical examination revealed atrophy of his left upper limb muscles. There were fasciculations in the biceps and brachioradialis muscles. electromyography revealed ongoing denervation neurogenic units in the C5 to TI myotomes comparable with PPS. CONCLUSION: The aim of this paper is to review the present situation and to give a short summary of PPS, which can be difficult to diagnose because the symptoms of presentation are usually non-specific. We describe a case of PPS and review the current literature. ( info) |
14/40. Respiratory failure in a patient with antecedent poliomyelitis: amyotrophic lateral sclerosis or post-polio syndrome? We report a 69-year-old man who developed paralytic poliomyelitis in childhood and then decades later suffered from fatal respiratory failure. Six months before this event, he had progressive weight loss and shortness of breath. He had severe muscular atrophy of the entire right leg as a sequela of the paralytic poliomyelitis. He showed mild weakness of the facial muscle and tongue, dysarthria, and severe muscle atrophy from the neck to proximal upper extremities and trunk, but no obvious pyramidal signs. Electromyogram revealed neurogenic changes in the right leg, and in the paraspinal, sternocleidomastoid, and lingual muscles. There was a slight increase in central motor conduction time from the motor cortex to the lumbar anterior horn. Pulmonary function showed restrictive ventilation dysfunction, which was the eventual cause of death. Some neuropathological features were suggestive of amyotrophic lateral sclerosis (ALS), namely Bunina bodies. In patients with a history of paralytic poliomyelitis who present after a long stable period with advanced fatal respiratory failure, one may consider not only respiratory impairment from post-polio syndrome but also the onset of ALS. ( info) |
15/40. Intrathecal immune response in patients with the post-polio syndrome. BACKGROUND. The syndrome of progressive muscular atrophy decades after acute paralytic poliomyelitis (post-polio syndrome) is not well understood. The theory that physiologic changes and aging cause the new weakness does not explain the immunologic abnormalities reported in some patients. An alternative explanation is persistent or recurrent poliovirus infection. methods. We assessed the intrathecal antibody response to poliovirus and intrathecal production of interleukin-2 and soluble interleukin-2 receptors in 36 patients with the post-polio syndrome and 67 controls (including 13 who had had poliomyelitis but had no new symptoms and 18 with amyotrophic lateral sclerosis). Intrathecal antibody responses to measles, mumps, herpes simplex, and varicella zoster viruses were also determined. RESULTS. Oligoclonal IgM bands specific to poliovirus were detected in the cerebrospinal fluid of 21 of the 36 patients with the post-polio syndrome (58 percent) but in none of the control group (P less than 0.0001). In quantitative studies there was evidence of increased intrathecal synthesis of IgM antibodies to poliovirus only among the patients with the post-polio syndrome; there was no increased synthesis of IgM to measles, mumps, herpes simplex, or varicella zoster viruses. The patients with post-polio syndrome had significantly higher mean ( /- SD) (cerebrospinal fluid levels of interleukin-2 and soluble interleukin-2 receptors than the controls (8.1 /- 5.3 vs. 1.4 /- 0.8 U per milliliter and 159.6 /- 102.9 vs. 10.7 /- 6.2 U per milliliter, respectively). The intrathecal synthesis of IgM antibodies to poliovirus correlated with the cerebrospinal fluid concentrations of interleukin-2 (P less than 0.0005) and soluble interleukin-2 receptors (P less than 0.001). CONCLUSIONS. An intrathecal immune response against poliovirus is present in many patients with the post-polio syndrome. In some of these patients the recrudescence of muscle weakness may be caused by persistent or recurrent infection of neural cells with the poliovirus. ( info) |
16/40. myasthenia gravis masquerading as post-poliomyelitis syndrome. A 79-year-old man with previous bulbar poliomyelitis developed dysphagia and was diagnosed as having post-polio syndrome. Over 2 years, his swallowing deteriorated and he suffered an aspiration pneumonia. Only after the subsequent development of fatigue and facial weakness was myasthenia gravis diagnosed. Diagnostic criteria for post-polio syndrome include the exclusion of all other neurological conditions such as myasthenia gravis. Moreover, in any instance where a patient develops new symptoms, it is advisable to reconsider the original diagnosis. ( info) |
17/40. Insidious phrenic nerve involvement in postpolio syndrome. A 49-year-old woman with amyotrophic sequelae of poliomyelitis experienced progressive left upper limb weakness and breathing discomfort while walking that had developed over one year prior to presentation. She had flaccid quadriplegia which was more marked in the left upper limb, with the C4- and C5-innervated muscles being most severely affected. Chest radiographs double exposed at maximal inspiration and expiration revealed poor respiratory movements in the left hemidiaphragm. phrenic nerve conduction study demonstrated conspicuous diaphragmatic paralysis on the left side. The electrophysiological study supports radiological findings and provides useful evidence for insidious phrenic nerve involvement in postpolio syndrome. ( info) |
18/40. Effective intervention strategies for management of impaired posture and fatigue with post-polio syndrome: a case report. This case report describes effective intervention strategies that included gait training with the use of an acceptable, assistive device for the management of impaired posture and fatigue associated with post-polio syndrome. review of videotaped gait-training sessions enhanced patient compliance with the assistive device, a single forearm crutch. The 49-year-old female acquired childhood polio with resulting leg length discrepancy, lower extremity weakness, and complaints of fatigue. She walked with a right ankle foot orthosis and increased right lateral trunk flexion during right stance. Interventions consisted of patient education regarding the diagnosis and management of post-polio syndrome, health promotion, and energy conservation strategies that included gait training with a single forearm crutch. An outcome measure not previously documented in the post-polio literature was used to measure the patient's perceived fatigue. Six-month and 18-month reexaminations found that the patient was compliant with the assistive device, reported a 30% reduction in fatigue, and walked with a more upright posture for longer distances in a shorter period of time. Patient education, health promotion, and energy conservation strategies that included walking with a properly fitting assistive device reduced perceived fatigue and improved posture and function in an individual with post-polio syndrome. ( info) |
19/40. Improvement in exercise capacity after nocturnal positive pressure ventilation and tracheostomy in a postpoliomyelitis patient. Progressive neuromuscular symptoms years after recovery from acute paralytic poliomyelitis have been termed the PPS. We describe a 52-year-old man who contracted poliomyelitis at age 9 years who fully recovered and 33 years later developed progressive dyspnea. Neurologic evaluation revealed bilateral paralysis of the vocal cords, generalized weakness, and accentuated mouth occlusion pressure and ventilatory responses to hypercapnic, hyperoxic breathing. An EMG and muscle biopsy showed changes consistent with acute and chronic denervation. Cardiopulmonary exercise evaluation demonstrated a pulmonary mechanical limit with excessive ventilation relative to CO2 output. tracheostomy and nocturnal positive pressure ventilation resulted in increased respiratory muscle strength, normalization of ventilatory drive and marked improvement in exercise capacity. ( info) |
20/40. The cultural context of polio biographies. Cultural contexts influence the ways individuals interpret and experience functional losses associated with post-polio sequelae. Using in-depth multiple interview case studies from two National Institute on aging projects, the concept of "biographies" is presented to place the individuals' polio-related experiences within the context of their lives. Two major cultural contexts shape the construction of polio biographies: normative life course expectations and developmental tasks; and traditions associated with polio recovery and rehabilitation. The authors identify key dimensions of personal concern among polio survivors that can be used as entrance points for effective clinical intervention and to promote treatment compliance. ( info) |