Abstract:
:Forty patients suffering from neuronal ceroid lipofuscinosis, 33 of the juvenile type (JNCL) and 7 of the infantile type (INCL), underwent a brain CT. All INCL patients showed severe atrophic changes which were most pronounced supratentorially and in the brainstem. The white matter was hypodense and markedly reduced and the cortical gray matter was quite thin in the oldest patients. The CT signs appeared early and can be used in differential diagnosis. In the JNCL group, CT findings were normal under the age of nine, but in the older patients (n = 28) atrophic changes were detected in all but two. Both supra- and infratentorial atrophy was detected and the severity of the alterations increased with age. No abnormal densities in the brain parenchyma were detected on CT. The severity of the cerebellar atrophy correlated positively with disturbances in motor functions, balance, co-ordination and EEG.
journal_name
Neuropediatricsjournal_title
Neuropediatricsauthors
Raininko R,Santavuori P,Heiskala H,Sainio K,Palo Jdoi
10.1055/s-2008-1071470subject
Has Abstractpub_date
1990-05-01 00:00:00pages
95-101issue
2eissn
0174-304Xissn
1439-1899journal_volume
21pub_type
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