Objective: We report a case of a 72 year-old woman with progressively worsening chorea of the left upper and lower extremities, who was incidentally found to have renal cell carcinoma (RCC) and an inconclusive leucine-rich, glioma inactivated 1 (LGI-1) antibody.  The patient underwent a partial nephrectomy with near-complete resolution of the choreiform movements.

Background: Chorea is a movement disorder characterized by hyperkinetic, dance-like movements of the extremities. Several antibodies including LGI-1 have been identified in association with paraneoplastic chorea, however RCC is not known to be associated with LGI-1 and only rarely presents with chorea.

Methods: A 72 year-old woman presented with a seven month history of progressively worsening chorea involving her left upper and lower extremities. Exam revealed choreiform movements with clumsy and dysrhythmic rapid alternating movements in the left arm and leg, and gait instability. Magnetic resonance imaging of the brain was negative without evidence of infarcts or abnormalities in the basal ganglia. Extensive lab testing of both serum and cerebrospinal fluid was unremarkable, except for an inconclusive anti-LGI-1. Computed tomography scan of the pelvis revealed a renal mass which was identified as RCC on biopsy. A partial left nephrectomy was performed with near-complete resolution of chorea.
 

Results: Paraneoplastic chorea is commonly associated with CRMP-5/CV2, GAD65, CASPR2, and LGI-1 antibodies. However, LGI-1 typically also presents with encephalitis, myoclonus, and ataxia, and is primarily seen in small cell lung carcinoma, thymoma, and breast and prostate adenocarcinoma, with no described association with RCC thus far. In a study by O’Toole et al in 2013 in which 36 patients with adult-onset chorea were studied, neither RCC nor anti-LGI-1 were identified in any of the patients, and the majority of patients exhibited generalized chorea (44%) as opposed to hemichorea (25%) (1). In addition, RCC in association with chorea is rare, and has only been described in the setting of encephalitis with generalized, severe bilateral chorea (2).

Conclusions: This case is unique in that RCC is rarely associated with chorea or LGI-1 antibodies and removal of the tumor led to significant symptom improvement, which highlights that RCC should be taken into consideration in patients with chorea.

References: 1.    O’Toole, Orna, et al. “Autoimmune chorea in adults.” Neurology 80.12 (2013): 1133-1144. 2.    Kujawa, Kathy A., et al. “Ballistic-choreic movements as the presenting feature of renal cancer.” Archives of neurology 58.7 (2001): 1133-1135.

« Back to 2017 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/an-unusual-case-of-hemichorea-in-a-woman-with-newly-discovered-renal-cell-carcinoma-and-inconclusive-lgi-1-antibody/