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Imiglucerase is a medication used in the treatment of Gaucher's disease. [2] [3]It is a recombinant DNA-produced analogue of the human enzyme β-glucocerebrosidase. Cerezyme is a freeze-dried medicine containing imiglucerase, manufactured by Genzyme Corporation.
Type III: muscle twitches known as myoclonus, convulsions, dementia, and ocular muscle apraxia; Parkinson's disease is recognized as being more common in Gaucher's disease patients and their heterozygous carrier relatives. [5] Osteoporosis: 75% of patients develop visible bony abnormalities due to the accumulated glucosylceramide.
β-Glucocerebrosidase (also called acid β-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, or GCase) is an enzyme with glucosylceramidase activity (EC 3.2.1.45) that cleaves by hydrolysis the β-glycosidic linkage of the chemical glucocerebroside, an intermediate in glycolipid metabolism that is abundant in cell membranes (particularly skin cells). [5]
The term "steroid dementia" was coined by Varney et al. (1984) in reference to the effects of long-term glucocorticoid use in 1,500 patients. [3] While the condition generally falls under the classification of Cushing's syndrome , the term "steroid dementia syndrome" is particularly useful because it recognizes both the cause of the syndrome ...
In Gaucher's disease, the enzyme glucocerebrosidase is nonfunctional and cannot break down glucocerebroside into glucose and ceramide in the lysosome. [1] Affected macrophages, called Gaucher cells, have a distinct appearance similar to "wrinkled tissue paper" under light microscopy, because the substrates build-up within the lysosome.
The systems of the body most affected by chemotherapy drugs include visual and semantic memory, attention and motor coordination and executive functioning. [9] [10] These effects can impair a chemotherapy patient's ability to understand and make decisions regarding treatment, perform in school or employment and can reduce quality of life. [10]
These side effects arise in approximately 10–20% of users, are mild to moderate in severity, and can be managed by slowly adjusting medication doses. [192] Less common secondary effects include muscle cramps , decreased heart rate ( bradycardia ), decreased appetite and weight, and increased gastric acid production.
ARIA-E refers to cerebral edema, involving the breakdown of the tight endothelial junctions of the blood-brain barrier and subsequent accumulation of fluid. [3] In a double-blind trial of the humanised monoclonal antibody solanezumab (n = 2042), sixteen patients (11 taking the drug, 5 taking a placebo), or 0.78% developed ARIA-E.