Reduction of striatal glucose metabolism in McLeod choreoacanthocytosis.
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Reduction of striatal glucose metabolism in McLeod choreoacanthocytosis. / Oechsner, M; Buchert, Ralph; Beyer, W; Danek, A.
in: J NEUROL NEUROSUR PS, Jahrgang 70, Nr. 4, 4, 2001, S. 517-520.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Reduction of striatal glucose metabolism in McLeod choreoacanthocytosis.
AU - Oechsner, M
AU - Buchert, Ralph
AU - Beyer, W
AU - Danek, A
PY - 2001
Y1 - 2001
N2 - McLeod syndrome is a distinct form of neuroacanthocytosis. Its defining feature is the depression of erythrocyte Kell antigens. The underlying X chromosomal mutations cause a dysfunction of an erythrocyte membrane protein Kx. A choreatic movement disorder with caudate atrophy in CT and MRI has been reported in McLeod syndrome later in the course of the disease. Positron emission tomography with 18F-deoxyglucose (FDG) was performed in two unrelated affected men. In the older patient, progressive chorea was seen from the 5th decade. In the second patient there were no signs of a movement disorder at the age of 28. Positron emission tomography disclosed a reduction of the striatal FDG uptake in both patients, with accentuation in patient 1. Frontal lobe metabolism was not affected. Basal ganglia dysfunction with early impairment of striatal glucose metabolism thus seems obligatory for McLeod syndrome, as found in other forms of chorea with or without acanthocytosis.
AB - McLeod syndrome is a distinct form of neuroacanthocytosis. Its defining feature is the depression of erythrocyte Kell antigens. The underlying X chromosomal mutations cause a dysfunction of an erythrocyte membrane protein Kx. A choreatic movement disorder with caudate atrophy in CT and MRI has been reported in McLeod syndrome later in the course of the disease. Positron emission tomography with 18F-deoxyglucose (FDG) was performed in two unrelated affected men. In the older patient, progressive chorea was seen from the 5th decade. In the second patient there were no signs of a movement disorder at the age of 28. Positron emission tomography disclosed a reduction of the striatal FDG uptake in both patients, with accentuation in patient 1. Frontal lobe metabolism was not affected. Basal ganglia dysfunction with early impairment of striatal glucose metabolism thus seems obligatory for McLeod syndrome, as found in other forms of chorea with or without acanthocytosis.
M3 - SCORING: Zeitschriftenaufsatz
VL - 70
SP - 517
EP - 520
JO - J NEUROL NEUROSUR PS
JF - J NEUROL NEUROSUR PS
SN - 0022-3050
IS - 4
M1 - 4
ER -
