[18F]fluorodopa PET shows striatal dopaminergic dysfunction in juvenile neuronal ceroid lipofuscinosis.
OBJECTIVE
To investigate whether nigrostriatal dopaminergic hypofunction is related to the extrapyramidal symptoms in patients with juvenile neuronal ceroid lipofuscinosis (JNCL).


METHODS
Nine patients with JNCL and seven healthy controls were studied using [18F]fluorodopa PET.


RESULTS
In the patients with JNCL [18F]fluorodopa uptake (K[i][occ]) in the putamen was 60% of the control mean and the corresponding figure in the caudate nucleus was 79%. There was a weak correlation between putamen K(i)(occ) values and extrapyramidal symptoms of the patients evaluated by the motor part of the unified Parkinson's disease rating scale (r = -0.57, P < 0.05). The overall severity of the disease also displayed a negative correlation with the K(i)(occ) values in the putamen (r = -0.71, P < 0.05).


CONCLUSIONS
In patients with JNCL there was reduced striatal [18F]fluorodopa uptake, which had a modest correlation with extrapyramidal symptoms. Dysfunction of nigrostriatal dopaminergic neurons is therefore not the only cause of the patients' extrapyramidal symptoms, but degenerative changes in other brain areas are also contributory.
Ruottinen
H M
HM
Department of Neurology, University of Turku, Finland.
Rinne
J O
JO
Haaparanta
M
M
Solin
O
O
Bergman
J
J
Oikonen
V J
VJ
Järvelä
I
I
Santavuori
P
P
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001480:Basal Ganglia Diseases / Q000175:diagnosis; D003342:Corpus Striatum / Q000378:metabolism*; D004298:Dopamine / Q000378:metabolism*; D006801:Humans; D008875:Middle Aged; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism*; D013378:Substantia Nigra / Q000378:metabolism; D014055:Tomography, Emission-Computed
D004298:Dopamine