1/16. 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 = upper (Clic here for more details about this article) |
2/16. 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 = 1keywords = upper (Clic here for more details about this article) |
3/16. 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 = 1keywords = upper (Clic here for more details about this article) |
4/16. 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 = 2318.9775332149keywords = breathing (Clic here for more details about this article) |
5/16. 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 = 2keywords = upper (Clic here for more details about this article) |
6/16. Clinical spectrum of muscle weakness in human west nile virus infection.poliomyelitis has recently been identified as a cause of muscle weakness in patients with west nile virus (WNV) infection. However, the clinical spectrum of WNV-associated weakness has not been described. We reviewed data on 13 patients with WNV infection. patients with muscle weakness were classified into one of three distinct groups based on clinical features. Group 1 comprised five patients who developed acute flaccid paralysis, four with meningoencephalitis and one without fever or other signs of infection. paralysis was asymmetric, and involved from one to four limbs in individual patients. Electrodiagnostic studies confirmed involvement of anterior horn cells or motor axons. Group 2 involved two patients without meningoencephalitis who developed severe but reversible muscle weakness that recovered completely within weeks. muscle weakness involved both lower limbs in one patient and one upper limb in the other. Group 3 consisted of two patients who experienced subjective weakness and disabling fatigue, but had no objective muscle weakness on examination. In addition to the three distinct groups, two other patients developed exaggerated weakness in the distribution of preexisting lower motor neuron dysfunction. We conclude that the clinical spectrum of WNV-associated muscle weakness ranges from acute flaccid paralysis, with or without fever or meningoencephalitis, to disabling fatigue. Also, preexisting dysfunction may predispose anterior horn cells to additional injury from WNV. awareness of this spectrum will help to avoid erroneous diagnoses and inappropriate treatment.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
7/16. Electrophysiological findings in poliomyelitis patients at the subacute phase.Electrodiagnostic tests-needle EMG, nerve conduction and somatosentory evoked potential (SEP) studies of the upper and the lower limbs were performed in three patients during the subacute phase of poliomyelitis. Although poliomyelitis is traditionally considered a "pure motor" disease, involvement of the sensory system was demonstrated by prolonged sensory nerve conduction and by delayed latencies and amplitude asymmetries of SEPs obtained from the lower limbs. Sensory deficit in poliomyelitis is well known to exist during the acute phase of illness. The present report describes the electrophysiological findings in patients during the subacute phase, several months after onset of illness. Sensory nerve action potentials and sensory evoked potentials were abnormal, especially those elicited by lower limb stimulation although the patients had no overt signs of sensory loss at that time. The associated EMG findings are described, and the probable pathologic changes of the motor unit are discussed.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
8/16. MRI in poliomyelitis-like syndrome.We report the MRI findings in a patient with poliomyelitis-like syndrome. MRI demonstrated a lesion extending from the dorsal region of the pons to the upper level of the thoracic spinal cord. The spinal cord was particularly affected in a region involving the bilateral anterior horns. Although its pathology is unknown, the lesion of the spinal cord seemingly reflects the pathological features of this disease.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
9/16. thoracic outlet syndrome secondary to childhood poliomyelitis.2 women with acute anterior poliomyelitis affecting the upper limb girdles in early childhood later developed thoracic outlet syndromes, 1 of them bilaterally. It is thought that the poliomyelitis was a contributory factor in each case by allowing undue descent of the shoulder girdles, although cervical ribs were also present in the second case. This association had not been recorded previously. Both patients obtained substantial relief from anterior scalenotomy, indicating that previous involvement of the shoulder girdle by poliomyelitis does not negate the efficacy of this operative procedure.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
10/16. speech production with flaccid paralysis of the rib cage, diaphragm, and abdomen.Anteroposterior diameter changes of the rib cage and abdomen were measured during breathing maneuvers and utterance activities in an adult subject with flaccid paralysis of the rib cage, diaphragm, and abdomen. Data were charted to solve for lung volume, volumes of the rib cage and abdomen, chest wall configuration, and inferred neck muscle actions. Free breathing was accomplished through an alternate waxing and waning of neck muscle drive or through step-wise glossopharyngeal pumping. Connected speech was performed in breath groups begun by neck gestures that "cocked" the breathing apparatus and stored recoil energy in it for use during ensuing expirations. Some breath groups were also extended through intermittent glossopharyngeal pumping. Connected speech was characterized by frugal control of the air supply through compensatory adjustments in the larynx and upper airway. Compensations involved in the breathing maneuvers and utterance activities are described, and consideration is given to two understandings--the human potential for functional restoration and the clinical evaluation and management of individuals with neuromuscular impairment.- - - - - - - - - - ranking = 1031.6566814289keywords = breathing, upper (Clic here for more details about this article) |
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