1/7. 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 = sclerosis (Clic here for more details about this article) |
2/7. amyotrophic lateral sclerosis with antecedent poliomyelitis.Motor neurone disease may occur in patients with antecedent, sometimes remote, paralytic poliomyelitis. A Jamaican patient with this sequence is described. research exploring the relationship between the two diseases is giving a new insight into the aetiology of motor neurone disease.- - - - - - - - - - ranking = 0.8keywords = sclerosis (Clic here for more details about this article) |
3/7. 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.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/7. amyotrophic lateral sclerosis with antecedent poliomyelitis.Histopathological and virological studies were performed on autopsy tissue from a 47-year-old man who had a history of acute poliomyelitis at age 15 years and died after a three-year course of amyotrophic lateral sclerosis (ALS). The poliovirus serologic tests suggested prior infection with poliovirus type 3 but no ongoing poliovirus infection. The CNS showed typical features of ALS with no inclusion bodies or inflammatory cells. Attempts to isolate poliovirus in the CNS were unsuccessful and results of immunofluorescence studies for poliovirus antigen were negative. Molecular hybridization experiments using a dna copy of the complete poliovirus genome failed to demonstrate poliovirus-related rna or dna sequences in the CNS. These studies, using sensitive techniques, indicate that there was no evidence of the continuing presence of poliovirus in this patient with ALS and antecedent poliomyelitis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/7. amyotrophic lateral sclerosis and parkinsonian syndromes in high incidence among the Auyu and Jakai people of West new guinea.amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) occur in the highest recorded incidence among primitive Auyu and Jakai people on the southern coastal plain of West new guinea, in association with a heretofore unrecognized subacute, often recurrent, paralytic "poliomyeloradiculitis" (PMR). Ninety-seven cases of ALS, 19 cases of PD and 18 cases of PMR were recorded, with mean ages of onset of 33, 43, and 26, respectively, in a small affected population of only about 7000. The ecology, culture, and diet of the remote, primitive ALS- and PD-affected people are indistinguishable from that of their unaffected neighbors, except for a remarkable deficiency of calcium and magnesium in their soil and drinking water. The distribution of affected and nonaffected villages indicates that communicable infectious or genetic etiology is unlikely. As a result of the isolation and primitive technology, domestic animals (except dogs and pigs) were not found among the Auyu and Jakai, and no manufactured products (including metals, ceramics, textiles, petrochemicals, medicines, food additives, condiments, paints, dyes, or solvents) were available to them.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/7. multiple sclerosis presenting as late functional deterioration after poliomyelitis.We describe five patients with previous poliomyelitis who developed multiple sclerosis (two laboratory supported definite and three clinically definite). The initial symptoms of functional deterioration developed a mean of 30 years following poliomyelitis. Initial functional deterioration was due to progressive limb weakness or impaired mobility and in three cases this led to an initial diagnosis of post-polio muscular atrophy. The clinical diagnosis became apparent with the subsequent development of characteristic clinical features, including optic nerve, brainstem, cerebellum, and spinal cord involvement. The occurrence of multiple sclerosis in these patients emphasises that late functional deterioration may be apparently unrelated to previous poliomyelitis. Furthermore, characteristic clinical features may be masked by the severe pre-existing neuromuscular and orthopaedic impairment leading to diagnostic delay and confusion with the ill-defined clinical syndrome of progressive post-polio muscular atrophy.- - - - - - - - - - ranking = 1.2keywords = sclerosis (Clic here for more details about this article) |
7/7. Differential patterns of atherosclerotic disease in patients with unilateral hemiparesis resulting from poliomyelitis: case reports demonstrating the possible effect of hemodynamics.The relative importance of hereditary and mechanical factors in the pathogenesis of aneurysms remains as controversial today as it was two decades ago. The cases of two patients with unilateral paresis resulting from poliomyelitis who presented with abdominal aortic aneurysms are reported. In addition, each patient had iliofemoral aneurysms contralateral to, and iliofemoral occlusive disease ipsilateral to, their affected extremity. The two cases detailed within this report suggest that hemodynamic forces may alter the pattern of disease in arteries affected by arteriosclerosis.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |