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Twenty-two of 32 sporadic cases of amyotrophic lateral sclerosis had argyrophilic spheroids, 20 micrometers or larger, in the anterior horns of the spinal cords. The fine structure of these spherical bodies was characterized by interwoven, small bundles of 10 nm neurofilaments. Scattered mitochondria, vesicles and fragments of smooth endoplasmic reticulum(More)
The hallmark of the brain pathology in multiple sclerosis is the white matter plaque, characterized by myelin destruction and oligodendrocyte loss. To examine the role that cell death plays in the development of MS lesions, we used the in situ TUNEL technique, a method that sensitively detects DNA fragmentation associated with death at the single cell(More)
Many studies have established the central involvement of the Golgi apparatus in the transport and processing of plasma membrane, lysosomal, and secreted proteins. The Golgi apparatus of neurons is also involved in the axoplasmic flow of fast-moving macromolecules and in the orthograde, retrograde, and transsynaptic transport of exogenous ligands. Markers of(More)
Deposits of IgG and complement were demonstrated by direct immunofluorescent techniques with sections of motor cortex and spinal cord from amyotrophic lateral sclerosis (ALS) patients. Six of 16 ALS patients showed deposits within the spinal cord while 5 of 13 showed similar deposits within the motor cortex. The specificity of this staining was shown by(More)
Distribution of phosphorylated neurofilament proteins within anterior horn cells in three cases of familial and six cases of sporadic amyotrophic lateral sclerosis (ALS) and ten control cases were investigated by using a monoclonal antibody. Two distinct staining patterns of perikarya were observed; (1) homogeneous pattern; either the entire or a part of(More)
The Golgi-Cox method was used to compare transverse sections of the lumbar cords of amyotrophic lateral sclerosis (ALS) patients and controls. Large anterior horn cells of the control cords could be divided into three groups based on arrangement of the dendrites arising from the soma; bipolar, tripolar and multipolar. Axons could be identified by their(More)
In a search for evidence of biochemical disorders in regions of postmortem brain other than the motor cortex in amyotrophic lateral sclerosis (ALS), ganglioside patterns were also examined in the frontal, temporal, and parahippocampal gyrus cortex. In 21 ALS brains studied (20 sporadic, 1 familial), abnormal patterns were found in the frontal cortex (81%),(More)
An infant with hydrocephalus, aqueductal stenosis and partial agenesis of the corpus callosum in association with Down's syndrome is reported. Review of the literature reveals that hydrocephalus is infrequent in Down's syndrome. Of special interest is the occurrence of agenesis of the corpus callosum in our patient, a lesion often reported with other(More)
Circulatory immune complexes are increased in amyotrophic lateral sclerosis (ALS) and an autoimmune mechanism has been inferred. Autoimmune diseases may have changes in the percentages of immunoregulatory T cells and increased activated T cells (Ia+ T) in peripheral blood. The latter are also increased with active viral and bacterial infection and(More)
A 66-year-old female presented with the acute onset of paraplegia progressing rapidly to quadriplegia and terminating fatally in less than a month. At autopsy extensive hemorrhagic infarction of the spinal cord was noted associated with widespread venous thrombosis. Other similar cases are reviewed and the pathophysiology is discussed.