The enduring enigma of sporadic chorea: A single center case series

Tremor Other Hyperkinet Mov (N Y). 2023 Sep 7:13:33. doi: 10.5334/tohm.800. eCollection 2023.

Abstract

Chorea can have a wide variety of causes including neurodegenerative, pharmacological, structural, metabolic, infectious, immunologic and paraneoplastic processes. We reviewed the clinical records of patients with apparently sporadic choreic movements and no relevant family history, who presented to our neurology department (Hospital Fundación Jimenez Diaz) between 1991 and 2022. We detected 38 cases of apparent sporadic chorea (ASC); Our analysis revealed 5 cases of genetic chorea (including 3 cases with Huntington's disease) while 6 cases were autoimmune/hematological; 6 drug-related chorea, 5 metabolic-vascular, 5 due to miscellaneous conditions and 4 were of mixed etiology. No clear etiology was identified in 8 cases. The differential diagnosis of ASC is extensive and challenging.

Highlights: Chorea can have a wide variety of genetic and sporadic causesWe reviewed the clinical records of patients with apparently sporadic chorea (ASC), who presented to our neurology department over the last 30 yearsWe detected 38 cases of apparent ASC; Our analysis revealed a wide array of different sporadic conditions and 5 cases of genetic choreaThe differential diagnosis of ASC is extensive and challenging.

Keywords: Hereditary chorea; Secondary chorea; Sporadic chorea.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies
  • Chorea* / diagnosis
  • Chorea* / genetics
  • Diagnosis, Differential
  • Humans
  • Huntington Disease* / genetics

Substances

  • Autoantibodies

Grants and funding

Pedro J Garcia-Ruiz received personal compensation as a consultant/scientific advisory board from Italfarmaco, Britannia, Bial, Esteve, Stada and Zambon and speaking honoraria from Italfarmaco, Zambon, Bial, Esteve, Stada and Abbvie. Lola Diaz-Feliz received speaking honoraria from Bial and Abbvie. Javier del Val, Cici E Feliz, Inmaculada Navas, Isabel Lorda Sanchez, Almudena Avila Fernandez, Fiona Blanco Kelly and Maria Jose Trujillo Tiebas: The authors declare that there are no additional disclosures to report.