Average age at disease onset is about 64, with the youngest confirmed onset being at age 43. There may be a slight female predominance. History
Corticobasal syndrome was first recognized in 1967 when Rebeiz, Kolodny, and Richardson Jr described three people with a progressive asymmetric akinetic-rigid syndrome combined with apraxia, which they named corticodentatonigral degeneration with neuronal achromasia. The condition was "mostly forgotten" until 1989, when Marsden et al. used the name corticobasal degeneration. In 1989 Gibb and colleagues provided detailed clinical and pathological descriptions in a further three cases adopting the name corticobasal degeneration, after which various other names included "corticonigral degeneration with nuclear achromasia" and "cortical basal ganglionic degeneration". Although the underlying cause of CBD is unknown, the disease occurs as a result of damage to the basal ganglia, specifically marked by neuronal degeneration or depigmentation (loss of melanin in a neuron) in the substantia nigra. Additional distinguishing neurological features of those diagnosed with CBD consist of asymmetric atrophy of the frontal and parietal cortical regions of the brain. Postmortem studies of patients diagnosed with CBD indicate that histological attributes often involve ballooning of neurons, gliosis, and tauopathy. Much of the pioneering advancements and research performed on CBD has been completed within the past decade or so, due to the relatively recent formal recognition of the disease.The Office of Rare Diseases in the United States created the first criteria in 2002, and the Armstrong clinical diagnostic criteria were created in 2013.
Chemistry
It is a hydrophilic white powder in pure form and dissolves in cold (but not in hot) water, forming a clear viscous solution or gel. Methyl cellulose is used as a buffer additive in capillary electrophoresis to control electroosmotic flow for improved separations. Special effects
The slimy, gooey appearance of an appropriate preparation of methyl cellulose with water, in addition to its nontoxic, nonallergenic, and edible properties, makes it popular for use in special effects for motion pictures and television wherever vile slimes must be simulated. In the film Ghostbusters, the gooey substance the supernatural entities used to "slime" the Ghostbusters was mostly a thick water solution of methyl cellulose. The Aliens ooze and drip a great deal of methyl cellulose—especially the queen.Methyl cellulose has been used to safely simulate molten materials, as well. In several of the Terminator films, it was back-lit with colored gels and films to reproduce the heated glow of iron in the large pouring ladles used to transport the metal from the smelting ovens to the various molds and forms. Methyl cellulose was also a stand-in for the lava flows in Los Angeles in Volcano and on the volcanic surface of Mustafar, in Star Wars: Episode III – Revenge of the Sith. Chemistry
Methyl cellulose does not occur naturally and is synthetically produced by heating cellulose with caustic solution (e.g. a solution of sodium hydroxide) and treating it with methyl chloride. In the substitution reaction that follows, the hydroxyl residues (-OH functional groups) are replaced by methoxide (-OCH3 groups).