A favorable response to antiparkinsonian treatment in juvenile neuronal ceroid lipofuscinosis

被引:21
作者
Åberg, LE
Rinne, JO
Rajantie, I
Santavuori, P
机构
[1] Univ Helsinki, Hosp Children & Adolescents, Dept Pediat, Helsinki 00029, Finland
[2] Univ Helsinki, Hosp Children & Adolescents, Dept Neurol, Helsinki 00029, Finland
[3] Univ Turku, Dept Neurol, FIN-20520 Turku, Finland
[4] Univ Turku, Turku PET Ctr, FIN-20520 Turku, Finland
关键词
D O I
10.1212/WNL.56.9.1236
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
To study the effect of dopaminergic drugs on the parkinsonism in juvenile neuronal ceroid lipofuscinosis, the authors conducted an open study of 21 patients. According to the motor Unified PD Rating Scale (UPDRS) score, treatment was initiated with either levodopa (n = 10) or selegiline (n = 6). Five patients served as a control group. The UPDRS score after 1 year was compared with the score at onset. Both in the control group and in the selegiline group, the mean UPDRS score increased, whereas in the levodopa group, the mean UPDRS score decreased. The difference between the levodopa group and the control group was significant.
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收藏
页码:1236 / 1239
页数:4
相关论文
共 10 条
[1]   Decreased striatal dopamine transporter density in JNCL patients with parkinsonian symptoms [J].
Åberg, L ;
Liewendahl, K ;
Nikkinen, P ;
Autti, T ;
Rinne, JO ;
Santavuori, P .
NEUROLOGY, 2000, 54 (05) :1069-1074
[2]   MRI of neuronal ceroid lipofuscinosis .2. Postmortem MRI and histopathological study of the brain in 16 cases of neuronal ceroid lipofuscinosis of juvenile or late infantile type [J].
Autti, T ;
Raininko, R ;
Santavuori, P ;
Vanhanen, SL ;
Poutanen, VP ;
Haltia, M .
NEURORADIOLOGY, 1997, 39 (05) :371-377
[3]  
Fahn S., 1987, RECENT DEV PARKINSON, P153
[4]  
Hofman I, 1999, BIOM HLTH R, V33, P55
[5]   Clinical and magnetic resonance imaging findings in batten disease: Analysis of the major mutation (1.02-kb deletion) [J].
Jarvela, I ;
Autti, T ;
Lamminranta, S ;
Aberg, L ;
Raininko, R ;
Santavuori, P .
ANNALS OF NEUROLOGY, 1997, 42 (05) :799-802
[6]   Rapid diagnostic test for the major mutation underlying Batten disease [J].
Jarvela, I ;
Mitchison, HM ;
Munroe, PB ;
ORawe, AM ;
Mole, SE ;
Syvanen, AC .
JOURNAL OF MEDICAL GENETICS, 1996, 33 (12) :1041-1042
[7]   Batten's disease: eight genes and still counting? [J].
Mole, SE .
LANCET, 1999, 354 (9177) :443-445
[8]   [F-18]fluorodopa PET shows striatal dopaminergic dysfunction in juvenile neuronal ceroid lipofuscinosis [J].
Ruottinen, HM ;
Rinne, JO ;
Haaparanta, M ;
Solin, O ;
Bergman, J ;
Oikonen, VJ ;
Jarvela, I ;
Santavuori, P .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1997, 62 (06) :622-625
[9]  
Zeman W., 1970, HDB CLINICAL NEUROLO, P588
[10]   ANTIPARKINSON DRUGS IN THE BATTEN-SPIELMEYER-VOGT SYNDROME - A PILOT TRIAL [J].
ZWEIJEHOFMAN, IL ;
VANDERZEE, HJ ;
VANNIEUWENHUIZEN, O .
CLINICAL NEUROLOGY AND NEUROSURGERY, 1982, 84 (02) :101-105