Abstract
We report three children with hemidystonia in whom anti-cardiolipin (aCL) antibodies were demonstrated. Systemic lupus erythematosus was excluded on the basis of both clinical and serological criteria, and the diagnosis of primary antiphospholipid syndrome (PAPS) was made. In two cases, aCL antibodies could be causally related to a presumed immune-mediated thrombotic event involving the basal ganglia as shown by magnetic resonance imaging (MRI). In the remaining patient the finding of white matter alteration on NMR might be due to cross-reactivity of anti-phospholipid (aPL) antibodies with cerebral phospholipids, resulting in demyelination. We suggest that PAPS must always be considered when isolated or recurrent focal cerebral ischaemia, and particularly hemidystonia, occur in childhood.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Antibodies, Anticardiolipin / blood
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Antibodies, Anticardiolipin / immunology*
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Antiphospholipid Syndrome / diagnosis
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Antiphospholipid Syndrome / immunology
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Antiphospholipid Syndrome / physiopathology*
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Basal Ganglia / physiopathology
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Basal Ganglia Diseases / diagnosis
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Basal Ganglia Diseases / physiopathology
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Brain / diagnostic imaging
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Brain / physiopathology
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Brain Diseases / diagnosis
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Brain Diseases / physiopathology
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Child
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Demyelinating Diseases
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Dystonia / diagnosis
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Dystonia / immunology
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Dystonia / physiopathology*
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Female
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Humans
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Immunoglobulin G / blood
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Immunoglobulin M / blood
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Magnetic Resonance Imaging
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Male
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Movement Disorders / diagnosis
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Movement Disorders / immunology
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Movement Disorders / physiopathology*
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Radiography
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Videotape Recording
Substances
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Antibodies, Anticardiolipin
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Immunoglobulin G
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Immunoglobulin M