Cerebral blood flow deficits in hereditary essential myoclonus
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Cerebral blood flow deficits in hereditary essential myoclonus. / Delecluse, F; Waldemar, G; Vestermark, S; Paulson, O B.
I: Archives of Neurology, Bind 49, Nr. 2, 02.1992, s. 179-82.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Cerebral blood flow deficits in hereditary essential myoclonus
AU - Delecluse, F
AU - Waldemar, G
AU - Vestermark, S
AU - Paulson, O B
PY - 1992/2
Y1 - 1992/2
N2 - Hereditary essential myoclonus is a disease in which segmental myoclonus is the sole clinical abnormality and whose cause is unknown. It is characterized by an early onset, a benign course, an autosomal dominant pattern of inheritance, the absence of any other neurologic dysfunction, and normal results of auxiliary tests. Cerebral blood flow studies of a father and son with this disease showed a cortical blood flow reduction contralateral to the myoclonus symptoms. We postulate the cause to be a focal unilateral subcortical cerebral lesion, either in the basal ganglia or in the brain stem, with subsequent cortical deafferentation.
AB - Hereditary essential myoclonus is a disease in which segmental myoclonus is the sole clinical abnormality and whose cause is unknown. It is characterized by an early onset, a benign course, an autosomal dominant pattern of inheritance, the absence of any other neurologic dysfunction, and normal results of auxiliary tests. Cerebral blood flow studies of a father and son with this disease showed a cortical blood flow reduction contralateral to the myoclonus symptoms. We postulate the cause to be a focal unilateral subcortical cerebral lesion, either in the basal ganglia or in the brain stem, with subsequent cortical deafferentation.
KW - Adult
KW - Brain/diagnostic imaging
KW - Cerebrovascular Circulation
KW - Child
KW - Humans
KW - Male
KW - Myoclonus/genetics
KW - Tomography, Emission-Computed, Single-Photon
U2 - 10.1001/archneur.1992.00530260081025
DO - 10.1001/archneur.1992.00530260081025
M3 - Journal article
C2 - 1736852
VL - 49
SP - 179
EP - 182
JO - JAMA Neurology
JF - JAMA Neurology
SN - 2168-6149
IS - 2
ER -
ID: 274927414