Krabbe's Disease

Abstract

The demyelinating diseases are characterized by a primary breakdown of myelin with more or less persistence of the axis cylinders and with secondary proliferation of astrocytes and microglial cells leading to sclerosis. The demyelination may be disseminated, as in multiple sclerosis, or diffuse. A disorder first described by Krabbe¹in 1916 and known since then as Krabbe's disease is an example of the diffuse type of cerebral sclerosis. In the disseminated type of sclerosis numerous discrete, demyelinating foci of variable size are scattered throughout the brain and spinal cord. In the diffuse type, the lesions begin in one focus in the cerebral hemispheres and spread to involve adjacent white matter in a continuous fashion. Ultimately the demyelination extends from the paraventricular areas to the cortex, and usually it becomes bilateral and symmetrical. The classifications of the diffuse demyelinating diseases have undergone considerable revision since the development of histochemical