Subacute axial parkinsonism associated with anti-Ri antibodies

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LETTER TO THE EDITOR

Subacute axial parkinsonism associated with anti-Ri antibodies Chiara Di Schino 1 & Martina Nunzi 2 & Carlo Colosimo 1 Received: 24 March 2020 / Accepted: 12 August 2020 # Fondazione Società Italiana di Neurologia 2020

A 69-year-old housewife presented with progressive anterior flexion of the trunk associated with pain; the onset of the symptoms, which was insidious, occurred in August 2018. She had no family history of neurological disorders, and her past medical history was unremarkable with the exception of strabismus in childhood, for which early surgery led to excellent compensation. In the 2 months following the onset of anterior flexion of the trunk, she developed progressive “shaking” in the upper limbs, softening of the voice and a gait impairment characterized by short steps and imbalance. By November 2018, she was unable to walk independently. In early December 2018, she was admitted to a neurological department, where routine blood tests were found to be normal and a brain and full spine magnetic resonance imaging was unremarkable (suppl. Material, Fig. 1 A-B). Levodopa was started and titrated up to 600 mg/day, though with no improvement. A diagnosis of atypical parkinsonism (possible multiple system atrophy, MSA) was made. In February 2019, she was referred to our movement disorders outpatient clinic for a second opinion. Upon examination, she showed appendicular bradykinesia and rigidity, and a more severe axial picture with dysarthria, short-stepped gait, and camptocormia (defined as an axial postural deformity with abnormal thoracolumbar spinal flexion of at least 45°) [1]. The patient also displayed stimulus-sensitive myoclonus in both upper limbs, bilateral abducens palsy with strabismus, and mixed dysarthria. No dysphagia, dizziness, or cranial dystonia was present, and the cognitive assessment was normal. Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10072-020-04685-y) contains supplementary material, which is available to authorized users. * Carlo Colosimo [email protected]; [email protected] 1

Department of Neurology, Santa Maria University Hospital, Viale Tristano di Joannuccio 1, 05100 Terni, Italy

2

Department of Oncology, Santa Maria University Hospital, Terni, Italy

The following week, she was admitted to our department ward: the EMG/ENG revealed mixed polyneuropathy with occasional fasciculations, and the CSF examination yielded 37 cells and 68 mg/dl proteins. Antibody testing was normal for anti-Hu, anti-Yo, anti CV2, anti-amphiphysin, anti-Ma1, anti-Ma2, anti-NMDA, anti-LgI1, anti-CASPR2, anti-GABA, anti-GluR1, and anti-GluR2, while serum anti-Ri was positive. The total-body CT and mammography were abnormal, with a 21 × 18 mm nodule being detected in the right breast (suppl. Material, Fig. 2 A-B). Histology revealed an infiltrating, poorly differentiated, grade G3 carcinoma, with a high proliferation index (Ki67 > 50%), poor endocrine sensitivity (estrogen receptor 15%, progesterone receptor 10%