Reduced cerebral fluoro-L-dopamine uptake in adult patients suffering from phenylketonuria.
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Reduced cerebral fluoro-L-dopamine uptake in adult patients suffering from phenylketonuria. / Landvogt, Christian; Mengel, Eugen; Bartenstein, Peter; Buchholz, Hans Georg; Schreckenberger, Mathias; Siessmeier, Thomas; Scheurich, Armin; Feldmann, Reinhold; Weglage, Josef; Cumming, Paul; Zepp, Fred; Ullrich, Kurt.
in: J CEREBR BLOOD F MET, Jahrgang 28, Nr. 4, 4, 2008, S. 824-831.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Reduced cerebral fluoro-L-dopamine uptake in adult patients suffering from phenylketonuria.
AU - Landvogt, Christian
AU - Mengel, Eugen
AU - Bartenstein, Peter
AU - Buchholz, Hans Georg
AU - Schreckenberger, Mathias
AU - Siessmeier, Thomas
AU - Scheurich, Armin
AU - Feldmann, Reinhold
AU - Weglage, Josef
AU - Cumming, Paul
AU - Zepp, Fred
AU - Ullrich, Kurt
PY - 2008
Y1 - 2008
N2 - Deficiency of phenylalanine hydroxylase activity in phenylketonuria (PKU) causes an excess of phenylalanine (Phe) throughout the body, predicting impaired synthesis of catecholamines in the brain. To test this hypothesis, we used positron emission tomography (PET) to measure the utilization of 6-[18F]fluoro-L-DOPA [corrected] (FDOPA) in the brain of adult patients suffering from PKU and in healthy controls. Dynamic 2-h long FDOPA emission recordings were obtained in seven adult PKU patients (five females, two males; age: 21 to 27 years) with elevated serum Phe levels, but lacking neurologic deficits. Seven age-matched, healthy volunteers were imaged under identical conditions. The utilization of FDOPA in striatum was calculated by linear graphical analysis (k3S, min(-1)), with cerebellum serving as a nonbinding reference region. The time to peak activity in all brain time-radioactivity curves was substantially delayed in the PKU patients relative to the control group. The mean magnitude of k3S in the striatum of the PKU patients (0.0052+/-0.0004 min(-1)) was significantly lower than in the control group (0.0088+/-0.0009 min(-1)) (P
AB - Deficiency of phenylalanine hydroxylase activity in phenylketonuria (PKU) causes an excess of phenylalanine (Phe) throughout the body, predicting impaired synthesis of catecholamines in the brain. To test this hypothesis, we used positron emission tomography (PET) to measure the utilization of 6-[18F]fluoro-L-DOPA [corrected] (FDOPA) in the brain of adult patients suffering from PKU and in healthy controls. Dynamic 2-h long FDOPA emission recordings were obtained in seven adult PKU patients (five females, two males; age: 21 to 27 years) with elevated serum Phe levels, but lacking neurologic deficits. Seven age-matched, healthy volunteers were imaged under identical conditions. The utilization of FDOPA in striatum was calculated by linear graphical analysis (k3S, min(-1)), with cerebellum serving as a nonbinding reference region. The time to peak activity in all brain time-radioactivity curves was substantially delayed in the PKU patients relative to the control group. The mean magnitude of k3S in the striatum of the PKU patients (0.0052+/-0.0004 min(-1)) was significantly lower than in the control group (0.0088+/-0.0009 min(-1)) (P
M3 - SCORING: Zeitschriftenaufsatz
VL - 28
SP - 824
EP - 831
JO - J CEREBR BLOOD F MET
JF - J CEREBR BLOOD F MET
SN - 0271-678X
IS - 4
M1 - 4
ER -
