1/16. Nonparalytic polio and postpolio syndrome.We describe four cases of postpolio syndrome with typical histories, physical examination results, and electrodiagnostic evidence of extensive anterior horn cell disease, as well as the putative pathophysiology of postpolio syndrome in persons with histories of nonparalytic polio and the diagnostic implications for individuals older than 40 yr of age who are experiencing unexplained new weakness, fatigue, and muscle or joint pain. Although the diagnosis of postpolio syndrome traditionally has required a remote history of paralytic polio, many persons such as the ones described here with typical symptoms of postpolio syndrome have no clear history of paralytic disease and are being misdiagnosed. With this in mind, we believe that the diagnostic criteria for postpolio syndrome should be modified to include the following: a history of remote paralytic polio or findings on history, physical examination results, and laboratory studies compatible with poliovirus damage of the central nervous system earlier in life.- - - - - - - - - - ranking = 1keywords = anterior horn, anterior horn cell, horn cell, horn (Clic here for more details about this article) |
2/16. Progressive amyotrophy as a late complication of myelopathy.A delayed syndrome of progressive weakness has been described in survivors of paralytic poliomyelitis - "Post-Polio muscular atrophy (PPMA)". One proposed etiology is a drop-out of motor neurons due to increased metabolic demands of an enlarged motor unit territory. We report a patient with slowly progressive lower extremity weakness 20 years after recovery from an episode of myelopathy involving the lower lumbar and sacral segments of the spinal cord. Delayed progressive amyotrophy may complicate any significant injury to anterior horn cells.- - - - - - - - - - ranking = 1.0153113200303keywords = anterior horn, anterior horn cell, horn cell, neuron, motor, horn (Clic here for more details about this article) |
3/16. Post-polio syndrome: a review and case report.A 21-year-old Thai man presented with progressive weakness and atrophy of both hands for 2 years. His left leg was atrophic and weak secondary to poliomyelitis since he was 8 months old. physical examination showed that there was atrophy of both hands and forearms as well as left leg. sensation was normal. Deep tendon reflexes were normal except for hyporeflexia of left leg. EMG showed chronic denervation pattern in the recent atrophic muscles and in normal power muscles. Motor and sensory nerve conductions were normal. biopsy of forearm muscle revealed degeneration and fibrosis of muscle fibers. These findings were compatible with post-polio syndrome (PPS) which presents in patients with late paralysis following poliomyelitis. It is not a form of amyotrophic lateral sclerosis although some clinical similarities exist. Weakness from PPS may lead to musculoskeletal pain and increased functional disability. Proper rehabilitation program is to maintain his functional status and allow him to continue to live as independently as possible by regular exercise without stressing joints and muscles.- - - - - - - - - - ranking = 0.36445800298966keywords = lateral sclerosis, sclerosis, lateral (Clic here for more details about this article) |
4/16. 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.- - - - - - - - - - ranking = 7.7517413195538E-5keywords = upper (Clic here for more details about this article) |
5/16. 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.- - - - - - - - - - ranking = 2.3003375261093keywords = anterior horn, lateral sclerosis, motor, horn, sclerosis, lateral, upper (Clic here for more details about this article) |
6/16. 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.- - - - - - - - - - ranking = 0.36445800298966keywords = lateral sclerosis, sclerosis, lateral (Clic here for more details about this article) |
7/16. 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.- - - - - - - - - - ranking = 7.7517413195538E-5keywords = upper (Clic here for more details about this article) |
8/16. 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.- - - - - - - - - - ranking = 0.00027849823167846keywords = lateral (Clic here for more details about this article) |
9/16. 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.- - - - - - - - - - ranking = 0.00027849823167846keywords = lateral (Clic here for more details about this article) |
10/16. Post-polio syndrome spinal cord pathology. Case report with immunopathology.Post-polio syndrome (PPS) describes the clinical onset of progressive muscular weakness many years after survival of acute paralytic poliomyelitis, often in muscle groups clinically unaffected by the original disease process. Prior pathologic descriptions of this often disabling, but not usually fatal, syndrome have been scanty. These have emphasized the presence of persistent or new inflammation in the meninges, spinal cord, and muscles of affected patients. The inflammation suggests several pathogenetic hypotheses, including persistent active poliovirus infection, autoimmune attack on central and peripheral nervous system elements, or increased vulnerability of poliovirus-damaged tissue to new infections. We have recently examined the central nervous system from a PPS patient. The cord showed focal perivascular intraparenchymal chronic inflammatory infiltrates. Immunoperoxidase staining demonstrated that the infiltrates were virtually pure populations of B lymphocytes (immunopositive with antibody L26, and immunonegative with the T cell marker UCHL1 as well as the macrophage marker HAM56). There were rare macrophages (HAM56 immunopositive) and no T cells. The character of the infiltrates suggests that PPS could be an autoimmune disorder mediated by antibodies produced in situ, and not by a cell-mediated process. Additional important pathological features were the presence in the anterior horns of axonal spheroids and of moderate wallerian degeneration in the lateral columns. The brain was entirely unremarkable, with no detectable cell loss of gliosis in the internal capsules or precentral gyri.- - - - - - - - - - ranking = 0.46839084397998keywords = anterior horn, horn, lateral (Clic here for more details about this article) |
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