Krabbe disease

Abstract

In three patients with Krabbe disease (galactosylceramide lipidosis), CT and MRI patterns progressed with the evolution of the disease. At first, discrete and symmetric dense areas on CT were found in deep gray matter of hemispheres and brainstem, and also in periventricular and capsular white matter. MRI showed decreased T1 values with normal or slightly decreased T2 values in those areas and large symmetric "plaque-like" lesions with high T1 and T2 values in white matter of the centrum semiovale. Later, both CT and MRI revealed diffuse reduction in gray matter and, more profoundly, in white matter mass. These findings may alert clinicians to the possibility of Krabbe disease in infants with progressive encephalopathy.

MR findings in globoid cell leucodystrophy

“…In this case, they represented involvement of the corticospinal tracts. These lesions have also been observed by Baram et al [1]. Lesions of the callosal body have once been observed by Winants et al [6].…”

“…Microscopically the diagnosis is confirmed by the presence of typical globoid cells, containing crystalloid cytoplasmic inclusions. These cells could be induced by the accumulation of cerebrosides [1].…”

“…Radiologically, CT and MRI are the examinations of choice [1,2]. Signs of corticosubcortical atrophy and a diffuse hypodense aspect of the parieto-occipital white matter may be seen.…”

Serial Magnetic Resonance Imaging Studies in a Case of Late Onset Globoid Cell Leukodystrophy

“…CT findings of infantile GLD in previous reports are summarized as folIows: In an early stage, symmetrical increased attenuation is recognized in the thalami, caudate nuclei, braim stern, cerebellum and corona radiata with or without patchy low density in the deep white matter of the cerebral and cerebellar hemispheres and minor cerebral atrophy (1-5, 10, 13, 14). Later, diffuse atrophy accompanied with ventriculomegaly and low density changes in the central white matter was recognized (1,2,4,7,10).…”

“…In the centrum semiovale, large symmetrie plaque-like lesions with high Tl and T2 values are recognized. Later, MR revealed diffuse reduction in gray matter and, more profoundly, in the white matter mass (1,13).…”