The Authors present the CT and MR findings in three patients with Krabbe disease: early infantile, late infantile and juvenile. Three children, 5 and 9 months old respectively with Krabbe disease, were studied with CT and MR. the children presented with spasticity and signs of peripheral neuropathy, blindness and no interaction with the environment. In all patient CT revealed nonenhancing discrete dense lesions, simmetrically distribuited in the posterior limb of the internal capsule and posterior thalami and focal hypodensities of the midcerebellar peduncles and dentate nuclei, with mild cerebral atrophy. In one patients CT demonstrated bilateral calcifications of paraventricular white matter. Mr, in all, showed a generalized increase in T2 values of the periventricular white matter. The thalami and adjacents regions had markedly shortened T1 and slightly shortenede T2. The cerebellum and the brainstem appeared mottled with scattered areas of increased T2. MR confirmed the cerebral atrophy. In conclusion CT and MR findings in Krabbe disease (early infantile and late infantile) are usually not specific. However when they are both performed at the onset of neurological symptoms they can alert clinicians to the possibility of Krabbe disease.

Early infantile and late infantile form of Krabbe disease : CT and MRI findings / M. Bonetti, A. Fontana, B. Cotticelli, V. Sansone, R. Micheli. - In: PEDIATRIA MEDICA E CHIRURGICA. - ISSN 0391-5387. - 29:4(2007 Jul), pp. 206-211.

Early infantile and late infantile form of Krabbe disease : CT and MRI findings

V. Sansone;
2007-07

Abstract

The Authors present the CT and MR findings in three patients with Krabbe disease: early infantile, late infantile and juvenile. Three children, 5 and 9 months old respectively with Krabbe disease, were studied with CT and MR. the children presented with spasticity and signs of peripheral neuropathy, blindness and no interaction with the environment. In all patient CT revealed nonenhancing discrete dense lesions, simmetrically distribuited in the posterior limb of the internal capsule and posterior thalami and focal hypodensities of the midcerebellar peduncles and dentate nuclei, with mild cerebral atrophy. In one patients CT demonstrated bilateral calcifications of paraventricular white matter. Mr, in all, showed a generalized increase in T2 values of the periventricular white matter. The thalami and adjacents regions had markedly shortened T1 and slightly shortenede T2. The cerebellum and the brainstem appeared mottled with scattered areas of increased T2. MR confirmed the cerebral atrophy. In conclusion CT and MR findings in Krabbe disease (early infantile and late infantile) are usually not specific. However when they are both performed at the onset of neurological symptoms they can alert clinicians to the possibility of Krabbe disease.
Settore MED/26 - Neurologia
PEDIATRIA MEDICA E CHIRURGICA
Article (author)
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/210529
Citazioni
  • ???jsp.display-item.citation.pmc??? 0
  • Scopus 1
  • ???jsp.display-item.citation.isi??? ND
social impact