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Titolo:
Brain involvement in Salla disease
Autore:
Sonninen, P; Autti, T; Varho, T; Hamalainen, M; Raininko, R;
Indirizzi:
Turku Univ Hosp, Dept Diagnost Radiol, FIN-20520 Turku, Finland Turku UnivHosp Turku Finland FIN-20520 Radiol, FIN-20520 Turku, Finland Univ Helsinki, Cent Hosp, Dept Radiol, FIN-00014 Helsinki, Finland Univ Helsinki Helsinki Finland FIN-00014 ol, FIN-00014 Helsinki, Finland Univ Turku, Dept Med Genet, SF-20500 Turku, Finland Univ Turku Turku Finland SF-20500 ept Med Genet, SF-20500 Turku, Finland Univ Helsinki Hosp, Dept Child Neurol, Helsinki, Finland Univ Helsinki Hosp Helsinki Finland ept Child Neurol, Helsinki, Finland Univ Uppsala Hosp, Dept Diagnost Radiol, S-75185 Uppsala, Sweden Univ Uppsala Hosp Uppsala Sweden S-75185 Radiol, S-75185 Uppsala, Sweden
Titolo Testata:
AMERICAN JOURNAL OF NEURORADIOLOGY
fascicolo: 3, volume: 20, anno: 1999,
pagine: 433 - 443
SICI:
0195-6108(199903)20:3<433:BIISD>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
NEURONAL CEROID-LIPOFUSCINOSIS; LYSOSOMAL STORAGE DISORDER; KRABBE DISEASE; HISTOPATHOLOGIC CORRELATION; MRI EVALUATION; RETT-SYNDROME; CT FINDINGS; INFANTILE; LEUKODYSTROPHY; CHILDHOOD;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
29
Recensione:
Indirizzi per estratti:
Indirizzo: Sonninen, P Turku Univ Hosp, Dept Diagnost Radiol, FIN-20520 Turku, Finland Turku Univ Hosp Turku Finland FIN-20520 20520 Turku, Finland
Citazione:
P. Sonninen et al., "Brain involvement in Salla disease", AM J NEUROR, 20(3), 1999, pp. 433-443

Abstract

BACKGROUND AND PURPOSE: Our purpose was to document the nature and progression of brain abnormalities in Salla disease, a lysosomal storage disorder,with MR imaging. METHODS: Fifteen patients aged 1 month to 43 years underwent 26 brain MR examinations. In 10 examinations, signal intensity was measured and comparedwith that of healthy volunteers of comparable ages. RESULTS: MR images of a 1-month-old asymptomatic child showed no pathology. In all other patients, abnormal signal intensity was found: on T2-weighted images, the cerebral white matter had a higher signal intensity than the gray matter, except in the internal capsules. In six patients, the white matter was homogeneous on all images. In four patients, the periventricular white matter showed a somewhat lower signal intensity; in five patients, a higher signal intensity. In the peripheral cerebral white matter, the measured signal intensity remained at a high level throughout life. No abnormalities were seen in the cerebellar white matter. Atrophic changes, if present,were relatively mild but were found even in the cerebellum and brain stem. The corpus callosum was always thin. CONCLUSION: In Salla disease, the cerebral myelination process is defective. In some patients, a centrifugally progressive destructive process is also seen in the cerebral white matter. Better myelination in seen in patientswith milder clinical symptoms.

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Documento generato il 23/01/20 alle ore 12:24:52