1/44. amyotrophic lateral sclerosis in an adult following acute paralytic poliomyelitis in early childhood.About 30% of polio survivors develop a post-polio syndrome. Some of these patients develop slowly progressive muscle weakness known as post-poliomyelitis muscular atrophy (PPMA). We describe an unusual form of amyotrophic lateral sclerosis (ALS) in a patient with acute poliomyelitis in childhood. An 80-year-old woman had acute poliomyelitis at 2 years of age and developed weakness limited to the lower extremities. Residual weakness was stable until the age of 75 when she developed rapidly progressive weakness that first affected her left arm and subsequently the right arm. Neurological examination revealed both upper and lower motor neuron signs. These clinical features were more consistent with ALS than PPMA. At autopsy, there was marked atrophy of the precentral gyrus. Microscopic examination revealed a severe loss of all nerve cells and pronounced fibrillary astrocytosis of the lumbar ventral horns in the spinal cord, presumably a result of poliomyelitis. Superimposed on these spinal cord alterations were the pathological features of ALS, consisting of loss of Betz cells, corticospinal tract degeneration and loss of motor neurons of other levels of the spinal cord. The findings included some atypical features for ALS, namely, sparing of the hypoglossal nucleus, absence of Bunina bodies and absence of ubiquitin-immunoreactive inclusions. Although poliomyelitis and ALS may be coincidental, the unusual pathological expression of ALS raise the possibility that it is related to the antecedent poliomyelitis.- - - - - - - - - - ranking = 1keywords = muscular atrophy, atrophy, muscle (Clic here for more details about this article) |
2/44. Surgical treatment of poliomyelitic scoliosis.Between 1968 and 1973 forty nine patients suffering from poliomyelitic scoliosis were treated surgically at the Rizzoli Institute. They were due to asymmetrical paralysis and contracture in the muscles of the trunk and limbs. Associated pathological conditions were found, such as pelvic obliquity, and vascular and trophic changes due to ganglionic lesions. The differing incidence and combination of these factors gave rise to various clinical types of spinal deformity. The average severity of curve was 39 degrees, the localisation was predominantly central, the average extent was ten vertebrae, and there was a marked predominance of right convexity (twenty nine out of thirty six). The rate of progression was maximum during puberty and almost negligible after bony maturity. It was greater in males and was unfavourably affected by the severity and asymmetrical distribution of the paralysis, by the early appearance of the disease, by high localisation of the deformity, and by the erect posture in patients who were ambulant. The most frequent visceral complications were in the respiratory system (ten patients with a deficit over 50%), followed by cardiac changes. Surgical treatment was adopted in patients with progressive curves over 60 degrees, because of the inevitable deterioration in their general condition and the tendency of the deformity to become fixed. Pre-operative correction by Halo-traction results (52% correction) than Risser plasters (38%). Posterior arthrodesis by Harrington's method was carried out in all the more recent cases (forty four). Post-operative plaster was maintained for eight months and then replaced by an orthopaedic corset. At bony maturity there was an averaged improvement of 35% in the angle of curvature, and an average improvement of 6% in vital capacity. The best corrections were obtained in patients under fourteen (42%), in dorso-lumbar scoliosis (40%) and in patients with curves above 100 degrees (38%). There was an average increase in height of 9.1 cms and a reduction in the gibbus of 3.4 cms. The complications included one traumatic pneumothorax, eight pseudarthroses, and breakage of the distraction rod in two cases resulting in complete relapse of the deformity. In six cases the upper hooks became loos and there were two cases of postoperative staphylococcal infection. In the distally sited curves our present policy is towards combining posterior arthrodesis with Dwyer's anterior interbody fusion.- - - - - - - - - - ranking = 0.00065976970025151keywords = muscle (Clic here for more details about this article) |
3/44. Acute care pediatric electromyography.The recognition of uncommon pediatric motor unit disorders or unusual clinical presentations of common illnesses, such as guillain-barre syndrome (GBS), have increased the need for electromyography (EMG) in childhood critical care units. There are two different clinical sets, one appropriate to newborns and infants and the other to older children. Some illnesses that present as an acute floppy infant are not found in the differential diagnosis of motor unit disorders in the older child or adult. These include spinal muscular atrophy, postvaccine poliomyelitis, intrauterine GBS, infantile botulism, and severe myopathies, such as myotonia dystrophy, and some glycogen storage diseases. An appreciation of the neurophysiological maturational norms is essential to an effective pediatric EMG consultation for children ages 0-3 years. Additionally, the neuromuscular complications of extended intubation and sepsis in children are gaining broader recognition. An increased dialogue between clinical neurophysiologists and pediatric neurologists and intensivists in both neonatal and pediatric intensive care units is essential.- - - - - - - - - - ranking = 0.99344381513192keywords = muscular atrophy, atrophy (Clic here for more details about this article) |
4/44. Nonpoliovirus poliomyelitis simulating guillain-barre syndrome.BACKGROUND: Paralytic poliomyelitis due to the wild-type poliovirus has been eradicated in the united states because of effective immunization programs. In the postvaccination era, most cases are caused by other rna viruses, such as coxsackievirus or echovirus. The condition usually begins with a fever and upper respiratory tract or gastrointestinal tract symptoms that progress to a "paralytic" phase characterized by limb weakness, areflexia, and, occasionally, respiratory failure that superficially resemble guillain-barre syndrome. OBJECTIVE: To describe 2 patients with nonpoliovirus poliomyelitis and highlight the findings on magnetic resonance imaging of the spinal cord to distinguish these cases from variants of guillain-barre syndrome. DESIGN AND SETTING: Case series from an academic medical center. patients: Following a viral illness, the patients, aged 35 and 50 years, had painless, progressive, asymmetrical weakness in the arms followed by respiratory failure in one patient, and generalized limb weakness in the other patient, reaching a nadir in 1 week. Both patients had fevers but no signs of meningitis at onset. Tendon reflexes were absent or reduced in affected regions. The cerebrospinal fluid findings were as follows: mononuclear leukocyte counts of 100 000 cells/mm(3) and 700 000 cells/mm(3), respectively, and the protein level was above 10 g/dL in both patients. Compound muscle action potential amplitudes were reduced in some nerves with active denervation in clinically affected muscles, and F-responses were absent but there were no other demyelinating features. magnetic resonance imaging showed discrete T2-weighted signal changes of the ventral horns of the spinal cord, and one had elevated coxsackievirus titers in the serum. There was little recovery and significant atrophy in weak muscles after 3 years. CONCLUSIONS: The poliomyelitis syndrome still occurs in adults in developed countries. It has superficial similarities to a motor axonal variant of guillain-barre syndrome but can be distinguished by clinical, cerebrospinal fluid, and, perhaps specifically, magnetic resonance imaging characteristics.- - - - - - - - - - ranking = 0.0078757242685849keywords = atrophy, muscle (Clic here for more details about this article) |
5/44. A part of the ulnar nerve as an alternative donor nerve for functioning free muscle transfer: a case report.A patient with no active elbow flexion because of poliomyelitis-like syndrome underwent functioning free muscle transfer for elbow flexion reconstruction in which a part of the ulnar nerve was used as a donor motor nerve. Fourteen months after surgery the patient had achieved 120 degrees of active elbow flexion against gravity without functional deficit of the donor ulnar nerve. A part of the ulnar nerve can be used as an alternative donor motor nerve for reconstruction of chronic C5-C6 (or C5-7) brachial plexus injury.- - - - - - - - - - ranking = 0.0032988485012575keywords = muscle (Clic here for more details about this article) |
6/44. Long-term follow-up of patients with nonparalytic poliomyelitis.OBJECTIVE: To examine patients with previous nonparalytic poliomyelitis in search of muscle atrophy, weakness, and other late symptoms of poliomyelitis. DESIGN: A mailed questionnaire followed up with neurologic and neurophysiologic examinations of respondents who reported symptoms possibly related to the late sequelae of polio. SETTING: neurology department at a university hospital. PARTICIPANTS: Thirty-nine of 47 patients diagnosed with nonparalytic poliomyelitis and hospitalized at a Norwegian hospital between 1950 and 1954, during the Norwegian polio epidemic. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: electromyography to determine function of the anterior tibialis, vastus lateralis, and biceps brachii muscles; nerve conduction studies of the sural, peroneal, and tibial nerves; motor and sensory nerve conduction velocity, and compound muscle and sensory nerve action potentials, and distal latencies. RESULTS: Twenty-five of 47 patients (53.2%) reported symptoms possibly related to the late sequelae of poliomyelitis. Eight of 20 examined symptomatic patients had normal neurologic and neurophysiologic findings, whereas 9 others had other medical conditions that could explain the symptoms. Three patients (6.7%) had neurologic and neurophysiologic findings and development of symptoms consistent with motoneuron damage. CONCLUSION: Some nonparalytic patients may have subclinical acute motoneuron damage with subsequent development and manifestation of motor weakness and neuromuscular symptoms many years later. These symptoms should be considered a differential diagnosis in patients who have a history of nonparalytic poliomyelitis.- - - - - - - - - - ranking = 0.0078757242685849keywords = atrophy, muscle (Clic here for more details about this article) |
7/44. A case of frog breathing.Frog breathing (glossopharyngeal breathing) is a useful technique employed to increase ventilation when respiratory muscles are paralysed. It is a technique used by many patients with chronic poliomyelitis, yet many chest physicians and physiotherapists are unfamiliar with this breathing maneuver. Glossopharyngeal breathing coordinates movements of the tongue, cheeks and pharynx to force air from the mouth into the lungs. We report a case of glossopharyngeal breathing, demonstrating a 3 fold increase in vital capacity in a subject with chronic poliomyelitis.- - - - - - - - - - ranking = 0.00065976970025151keywords = muscle (Clic here for more details about this article) |
8/44. Franklin D. Roosevelt: diagnosis, clinical course, and rehabilitation from poliomyelitis.In this case report, we examine Franklin Delano Roosevelt's prognosis, clinical course, and rehabilitation from poliomyelitis, and we examine the criticisms of errors in his diagnosis and management on the basis of current knowledge of the pathophysiology of poliomyelitis. Medical and historical records reveal the onset of severe paralysis, which progressed over several days, with minimal improvement in hips and lower limbs, but recovery of facial, upper limb, and upper trunk muscles. There is no scientific basis for assertions of mismanagement that led to more severe paralysis; the paralysis was most likely caused by strenuous activities in the preparalytic phase of his illness.- - - - - - - - - - ranking = 0.00065976970025151keywords = muscle (Clic here for more details about this article) |
9/44. Endplate dysfunction causing respiratory failure in a patient with prior paralytic poliomyelitis.A 56-year-old man with late amyotrophic sequelae from poliomyelitis experienced progressive dyspnoea requiring intubation and artificial ventilation in the intensive care unit. Repetitive stimulation studies showed a marked decrement of the trapezius muscle response reversible with edrophonium. Ventilatory function considerably and lastingly improved under anticholinesterase treatment. In the absence of biological evidence for autoimmune myasthenia gravis, it is suggested that a mechanism implying endplate dysfunction related to postpolio syndrome. Repetitive stimulation procedure should be considered in postpolio syndrome patients as some of them may benefit from anticholinesterase treatment.- - - - - - - - - - ranking = 0.00065976970025151keywords = muscle (Clic here for more details about this article) |
10/44. Provocation poliomyelitis: vaccine associated paralytic poliomyelitis related to a rectal abscess in an infant.OBJECTIVE:. To describe a case of vaccine associated paralytic poliomyelitis (VAPP) and relate this to current UK immunization policy. METHOD: A case report in which the clinical course and factors leading to the diagnosis are described and then related to reports of paralytic poliomyelitis in the literature. RESULTS: The child in this case was left severely disabled by paralytic poliomyelitis. The pathological process was related to a pararectal abscess needing urgent drainage shortly after immunisation. CONCLUSION: The skeletal muscle damage due to the presence of the pararectal abscess may have acted as the 'provocation' in the development of poliomyelitis. adoption of a policy of initial vaccination by the parenteral route as in the USA and European countries has been shown to greatly reduce this risk. The UK could adopt this policy which would minimise the risk of VAPP, as all recorded paralytic poliomyelitis in the UK in the last decade has been vaccine related.- - - - - - - - - - ranking = 0.00065976970025151keywords = muscle (Clic here for more details about this article) |
| | Next -> |