Background Antiphospholipid syndrome (APS) is a complex acquired autoimmune disease with a wide clinical spectrum. Chorea is a rare neurological manifestation of APS. Cases We report two elderly patients with APS-related chorea in whom functional imaging (18F-FDG positron emission tomography, FDG-PET) supported the diagnosis and compare our findings with existing literature. Literature Review Among 142 clinical cases of antiphospholipid-related chorea found in literature, only 10 had undergone brain metabolic imaging. Striatal hypermetabolism was evident in all cases (6) that underwent FDG-PET cerebral imaging. Cerebral perfusion single photon emission computed tomography (SPECT) was normal in two cases, while the other two presented with basal ganglia hypoperfusion. Conclusions Brain FDG-PET usually shows striatal hypometabolism in neurodegenerative types of chorea as opposed to striatal hypermetabolism observed in most cases of chorea from reversible etiologies, such as APS-related chorea. When a patient's clinical presentation is not clearly suggestive of either a neurodegenerative or autoimmune chorea, and first-line investigations are normal, FDG-PET may help in the differential diagnosis, especially in the presence of striatal hypermetabolism. SPECT data are less numerous and show either normal scans or basal ganglia hypoperfusion.

Antiphospholipid-Related Chorea: Two Case Reports and Role of Metabolic Imaging

Lerjefors, Lisa;Andretta, Silvia;Bonato, Giulia;Mainardi, Michele;Carecchio, Miryam;Antonini, Angelo
2022

Abstract

Background Antiphospholipid syndrome (APS) is a complex acquired autoimmune disease with a wide clinical spectrum. Chorea is a rare neurological manifestation of APS. Cases We report two elderly patients with APS-related chorea in whom functional imaging (18F-FDG positron emission tomography, FDG-PET) supported the diagnosis and compare our findings with existing literature. Literature Review Among 142 clinical cases of antiphospholipid-related chorea found in literature, only 10 had undergone brain metabolic imaging. Striatal hypermetabolism was evident in all cases (6) that underwent FDG-PET cerebral imaging. Cerebral perfusion single photon emission computed tomography (SPECT) was normal in two cases, while the other two presented with basal ganglia hypoperfusion. Conclusions Brain FDG-PET usually shows striatal hypometabolism in neurodegenerative types of chorea as opposed to striatal hypermetabolism observed in most cases of chorea from reversible etiologies, such as APS-related chorea. When a patient's clinical presentation is not clearly suggestive of either a neurodegenerative or autoimmune chorea, and first-line investigations are normal, FDG-PET may help in the differential diagnosis, especially in the presence of striatal hypermetabolism. SPECT data are less numerous and show either normal scans or basal ganglia hypoperfusion.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11577/3454990
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