g(m1)-ganglioside and Dementia

Recent years have witnessed considerable change in the conceptualization of the pathophysiology of the cognitive impairments in dementing disorders, as a result of synaptic neurochemical analyses. Profound reductions in the forebrain cholinergic projections occur in Alzheimer's disease. In GM1 gangliosidosis, variable alterations in neurotransmitter related processes that are located in synaptic membranes have been described. Exploitation of animal models of human disorders resulting in dementia may further clarify the dynamic alterations in the biochemical processes required for effective neurotransmission in cortex.

Topics: Alzheimer Disease; Animals; Brain; Cats; Cerebral Cortex; Cholinergic Fibers; Dementia; Disease Models, Animal; Dopamine; G(M1) Ganglioside; gamma-Aminobutyric Acid; Gangliosidoses; Glutamates; Glutamic Acid; Humans; Neurotransmitter Agents; Norepinephrine; Rats; Serotonin; Synaptic Transmission

Increasing evidence suggests that gangliosides act as important mediators in both de- and remyelination. The scope of the present research was to investigate the presence of immunoglobulin (Ig) M antibodies against GM1, GD1b, and GQ1b gangliosides in the sera of patients with dementia and the possible connection with clinical parameters of the disease.. This research topic demonstrates the investigation of 103 patients with dementia and 60 healthy individuals using enzyme-linked immunosorbent assay for the presence of 3 antiganglioside antibodies in their sera.. The authors report a positive connection between IgM anti-GM1 and the age (P = .005) and the severity of dementia (P = .005). Most of the patients who revealed increased IgM anti-GD1b levels had Alzheimer's disease (AD; P = .002).. This study indicates that elevated IgM anti-GM1 may be connected with the neurodegeneration in older patients with severe dementia and that AD may also be associated with increased IgM anti-GD1b levels.

Topics: Aged; Aged, 80 and over; Alzheimer Disease; Autoantibodies; Case-Control Studies; Dementia; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin M; Male; Middle Aged

Three sisters (ages 27, 24, and 17 years) presented with slowly progressing dystonic dementia and spastic tetraparesis with infantile onset. CSF, bone marrow, and conjunctival cells showed storage vacuoles. Biochemical analysis revealed increased urinary oligosaccharide excretion and decreased activity of acid beta-D-galactosidase and beta-D-fucosidase in serum, leukocytes, and cultured fibroblasts. The parents' enzyme values were in the heterozygous range. This is the only case in the literature of severe dementia associated with the clinical symptoms of type 3 GM1 gangliosidosis. The clinical heterogeneity of GM1 gangliosidosis and the significance of the combination of beta-D-galactosidase and beta-D-fucosidase defects in this syndrome are discussed.

Topics: Adolescent; Adult; alpha-L-Fucosidase; beta-Galactosidase; Bone Marrow; Cells, Cultured; Conjunctiva; Dementia; Female; Fibroblasts; G(M1) Ganglioside; Gangliosidoses; Humans; Leukocytes; Lysosomes; Microscopy, Electron; Oligosaccharides; Vacuoles