Abstract
This chapter discusses some degenerative cerebral diseases. The cerebral form of Gaucher's disease can present in the early years of life, or later, with hepatosplenomegaly, bone marrow depression, and mental and physical deterioration, associated with epilepsy. Cells in various parts of the body, including the brain, are distended by glucocerebroside, there being a deficiency of glucocerebrosidase that splits it into its component parts. The course of the disease is more often acute than chronic. The diagnosis may be suggested by an increased level of acid phosphatase in the serum and by the appearance of distended cells obtained by bone marrow puncture. The cerebral form of Niemann–Pick's disease can present a similar clinical picture. A number of different types of this disease have been described, starting at any age up to adult life. In the most common type A, the onset is early in life, with enlargement of the liver and spleen, physical and mental deterioration, and epileptic seizures. In type B, or the chronic form, the nervous system is not involved.
Published Version
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