Antecedent Poliomyelitis Research Articles

A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: Consideration of the pathology of post-polio syndrome

Post-polio syndrome (PPS) characterized by new neuromuscular problems can appear many years after acute poliomyelitis in polio survivors. We report a 77-year-old man with antecedent poliomyelitis who newly developed neuromuscular disease with a clinical course of 27 years, the final 10 years of which were characterized by apparent progression, thus raising doubt as to the clinical diagnosis of amyotrophic lateral sclerosis (ALS) following PPS. Pathologically, plaque-like, old poliomyelitis lesions were found almost exclusively in the lumbosacral cord, showing complete neuronal loss and glial scars in the anterior horns. Although less severe, neuronal loss and gliosis were also evident outside the old lesions, including the intermediate zone. Moreover, symmetrical degeneration of the corticospinal tracts, as evidenced by CD68 immunostaining, was a feature of the white matter of the lower spinal cord. In the motor cortex, loss of Betz cells was also confirmed. Synaptophysin immunostaining of the lumbosacral cord also revealed decreased expression outside the old lesions, excluding the posterior horn. Interestingly, decreased expression of synaptophysin was also evident in the cervical anterior horns, where no old lesions were observed. No Bunina bodies, TDP-43 inclusions, or Golgi fragmentation were found. Neurogenic atrophy was evident in the iliopsoas and scalenus muscles, and inclusion body myositis-like changes were also observed in these muscles and the tongue. Was it possible to have diagnosed this patient as having ALS? We consider that the features in this case may have represented the pathology of long-standing and/or fatal PPS itself, and not ALS.

Multiple bilateral asymmetrical deficiency of trunk muscles

Trunk muscles are an important source for pedicled and free flaps in reconstructive surgery. Unilateral deficiencies of trunk muscles are well known, either isolated or as part of Poland’s syndrome. Bilateral muscular deficiencies and a “bilateral Poland anomaly” have also been sporadically reported, but this is rare. We report on an 82-year-old male cadaver with clinically obscure, asymmetric bilateral deficiencies of the majority of trunk muscles. There was a history of acute poliomyelitis in childhood. Histological examination of representative muscle samples of the trunk showed extensive muscle atrophy with fat and connective tissue replacement. This was compatible with the prior diagnosis of poliomyelitis. However, representative sections of the spinal cord failed to reveal the antecedent poliomyelitis. The possibility of subclinical bilateral deficiencies of trunk muscles has to be taken into account in patients with a history of poliomyelitis when planning reconstructions in cases of regional pedicled muscle transfers or free microvascular tissue transfers in reconstructive surgery.

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.

Sequences specific for enterovirus detected in spinal cord from patients with motor neurone disease.

To investigate the association of enteroviruses with motor neurone disease, also known as amyotrophic lateral sclerosis. Analysis by enterovirus polymerase chain reaction of wax embedded material from spinal cords taken at necropsy from subjects with motor neurone disease and from age and sex matched controls. Specimens were collected in the west of Scotland and in London between 1982 and 1992. Sequences specific for a non-poliovirus type enterovirus were detected in spinal cord tissue from subjects with motor neurone disease. Amplification of a 414 base RNA target sequence in the conserved enterovirus 5' untranslated region from wax embedded tissue sections was successful in tissue from eight of 11 cases of sporadic motor neurone disease, one of two cases of familial motor neurone disease, and the one case of poliomyelitis, but not in the six matched controls or one case of antecedent poliomyelitis. In addition, sequences were detected in spinal cords from one monkey infected with wild type poliovirus and one monkey infected with polio vaccine. Comparison of sequences from cases of motor neurone disease with sequences of corresponding regions of the 5' untranslated regions of known picornaviruses showed them to be tightly grouped within the enterovirus genus closely related to coxsackievirus type B but not to polioviruses. Sequences derived from different parts of the spinal cord of the same subjects were identical, but sequences differed between individual subjects. Conserved enteroviral sequences closely related to coxsackie B virus sequences were detectable in spinal cords from subjects with sporadic motor neurone disease and from one subject with possible familial motor neurone disease.

How frequently does classic amyotrophic lateral sclerosis develop in survivors of poliomyelitis?

There is a paucity of reports of classic amyotrophic lateral sclerosis (ALS) developing in survivors of paralytic poliomyelitis. We describe a patient with classic ALS and an antecedent paralytic disease thought to have been poliomyelitis from which she recovered completely. If the paucity of ALS preceded by true poliomyelitis is not merely a matter of underreporting, antecedent paralytic poliomyelitis may have a protective role against the development of ALS. This has implications relevant to pathogenesis and to projected secular trends of ALS incidence since the introduction of poliomyelitis vaccines. There is a need to establish the incidence of cases of classic ALS in patients with antecedent poliomyelitis.

Isoelectric focusing studies of serum and cerebrospinal fluid in patients with antecedent poliomyelitis

The post-poliomyelitis syndrome (PPS) refers to symptoms of new weakness, fatigue, and pain years after recovery from acute poliomyelitis. Oligoclonal IgG bands have been reported in the cerebrospinal fluid (CSF) from PPS patients, suggesting that the syndrome is immune mediated or caused by persistent viral infection. We studied 15 paired serum and CSF samples and 6 unpaired CSF samples from a total of 21 patients with a prior history of poliomyelitis. Quantitative immune studies failed to show evidence for increased intrathecal IgG production relative to patients with noninflammatory central nervous system (CNS) disease. We found definite oligoclonal IgG bands in the CSF from only 1 patient, who also carried a diagnosis of multiple sclerosis. An isoelectric focusing poliovirus antigen overlay study showed evidence that suggested a CNS-specific antipoliovirus immune response in only 1 patient. Our results fail to support a dysimmune or persistent viral cause for post-poliomyelitis progressive muscular atrophy or PPS.

Autonomic cardiovascular responses in antecedent poliomyelitis.

Autonomically mediated cardiovascular responses were evaluated in 20 subjects with antecedent poliomyelitis and compared to data from an age- and sex-matched control group. The polio subjects had a lower heart rate response to the Valsalva manoeuvre but the same respiratory sinus arrhythmia as the controls. From this it is concluded that the polio subjects had a normal vagal function. The polio subjects had a greater initial heart rate increase but the same blood pressure response to the orthostatic position as the controls. This indicates a normal function of the sympathetic nerves. The greater heart rate increase is most likely caused by a displacement of blood to the legs because of muscle atrophy. The polio subjects had a smaller blood flow increase as an initial response to an isometric handgrip than the controls. This might be attributed to a reduced beta-adrenergic vasodilation, possibly due to a reduced central vasomotor drive. It is concluded that subjects with antecedent poliomyelitis have no significant dysfunction of the peripheral autonomic nerves. Thus, there is no deterioration of the peripheral autonomic nerve function in parallel with the progressive muscle atrophy and paralysis earlier described in post-polio subjects.

Sensory involvement in distal myopathy (Welander)

Eleven patients with Welander distal myopathy were subjected to detailed sensory testing including measurements of perception thresholds for vibration and temperature in both hands and feet. The threshold values were compared with normal, age-corrected values and also with data from an age-matched control group consisting of patients with antecedent poliomyelitis with the same degree of paresis. The screening examination indicated impaired thermal sensibility in all 11 patients and impairment for at least one other sensory modality in 9 patients. In comparison with age-corrected normal values, the measured warm-cold difference limen was abnormal in the feet of 9 patients and the vibratory threshold at least at one test point in 6 patients. When compared with the data from the paretic controls, the thermal abnormality was significant with regard to warm and cold thresholds, the warm-cold difference limen and the heat pain threshold. The vibration threshold abnormality was significant in the feet. It is concluded that sensibility impairment is present in Welander's myopathy indicating a peripheral sensory neuropathy involving both unmyelinated (C-fibers) and myelinated fibers.

Late denervation in patients with antecedent paralytic poliomyelitis.

The development of new weakness, fatigue, and pain decades after acute paralytic poliomyelitis is a recognized syndrome. We conducted a controlled study of this syndrome by analyzing clinical, electromyographic, and muscle-biopsy features in 18 patients with a history of poliomyelitis--13 reporting 1 to 20 years of new weakness and 5 without new symptoms. The patients with new weakness also reported new muscle atrophy (9 of 13) and fatigue (10 of 13), symptoms not reported by the controls. The age at the time of acute poliomyelitis, severity of poliomyelitis, residual disability, number of years since acute poliomyelitis, and age at the time of study were comparable in the weakening and control groups. Evidence of remote denervation consistent with antecedent poliomyelitis was demonstrated in all patients by electromyography or muscle biopsy or both. In addition, active denervation (as evidenced by spontaneous activity on conventional electromyography, increased jitter on single-fiber electromyography, or atrophic myofibers) was found in 12 patients in the weakening group and in all 5 controls. Immunohistochemical detection of myofibers expressing the neural-cell adhesion molecule corroborated ongoing denervation in both patient groups. When muscle data from both groups were pooled, correlations were observed between the extent of past reinnervation and the degree of ongoing motor-unit instability. We conclude that the extensive reinnervation of denervated muscle that occurs in paralytic poliomyelitis may be followed by late denervation of the previously reinnervated muscle fibers. Electromyographic and muscle-biopsy evidence of ongoing denervation does not distinguish between stable patients with prior paralytic poliomyelitis and those with new weakness.

Conduction velocity and refractory period of single motor nerve fibres in antecedent poliomyelitis.

The axonal conduction velocity and the axonal refractory period were determined using electromyographic techniques for 60 single motor units in 11 patients with antecedent poliomyelitis. The results were compared with previous observations in healthy subjects as well as subjects with motor neuron disease. In antecedent poliomyelitis there was a reduced proportion of nerve fibres with low conduction velocity as compared with the findings in healthy subjects. The mean value of the axonal conduction velocities in antecedent poliomyelitis (42.4 +/- 3.7 m/s, M +/- SD) was significantly higher (p less than 0.01) while in motor neuron disease the corresponding value (35.2 +/- 7.8 m/s) was significantly lower (p less than 0.005) than in healthy subjects (39.8 +/- 4.9 m/s). In antecedent poliomyelitis the conduction velocity and refractory period were related as in healthy subjects while abnormally long refractory periods were observed in motor neuron disease.