Cerebral Blood Flow Deficits in Hereditary Essential Myoclonus

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 resu...

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Bibliographic Details
Published inArchives of neurology (Chicago) Vol. 49; no. 2; pp. 179 - 182
Main Authors Delecluse, Florence, Waldemar, Gunhild, Vestermark, Svein, Paulson, Olaf B
Format Journal Article
LanguageEnglish
Published Chicago, IL American Medical Association 01.02.1992
Subjects
Adult
Biological and medical sciences
Brain - diagnostic imaging
Cerebrovascular Circulation
Child
Humans
Male
Medical sciences
Myoclonus - genetics
Myoclonus - physiopathology
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Tomography, Emission-Computed, Single-Photon
Radionuclide study
Human
Regional blood flow
Nervous system diseases
Myoclonus
Family study
Xenon
Hereditary
Photon
Involuntary movement
Case study
Hemodynamics
Positron emission tomography
Brain (vertebrata)
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:0003-9942
1538-3687
DOI:10.1001/archneur.1992.00530260081025