Autoimmune Movement Disorders Complicating Treatment with Immune Checkpoint Inhibitors.

BACKGROUND: Immune checkpoint inhibitors (ICI) may trigger autoimmune neurological conditions, including movement disorders (MD). OBJECTIVES: The aim of this study was to characterize MDs occurring as immune-related adverse events (irAEs) of ICIs. METHODS: A systematic literature review of case reports/series of MDs as irAEs of ICIs was performed. RESULTS: Of 5682 eligible papers, 26 articles with 28 patients were included. MDs occur as a rare complication of cancer immunotherapy with heterogeneous clinical presentations and in most cases in association with other irAEs. Inflammatory basal ganglia T2/fluid attenuated inversion recovery abnormalities are rarely observed, but brain imaging is frequently unrevealing. Cerebrospinal fluid findings are frequently suggestive of inflammation. Half of cases are associated with a wide range of autoantibodies. Steroids and ICI withdrawal usually lead to improvement, even though some patients experienced relapses or a severe clinical course. CONCLUSION: MDs are a rare complication of ICIs that should be promptly recognized to offer patients a correct diagnosis and treatment.

Stiff Person Spectrum Disorders-An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives.

Stiff person spectrum disorders (SPSD) are paradigm autoimmune movement disorders characterized by stiffness, spasms and hyperekplexia. Though rare, SPSD represent a not-to-miss diagnosis because of the associated disease burden and treatment implications. After decades as an enigmatic orphan disease, major advances in our understanding of the evolving spectrum of diseases have been made along with the identification of multiple associated autoantibodies. However, the most important recent developments relate to the recognition of a wider affection, beyond the classic core motor symptoms, and to further insights into immunomodulatory and symptomatic therapies. In this review, we summarize the recent literature on the clinical and paraclinical spectrum, current pathophysiological understanding, as well as current and possibly future therapeutic strategies.

Pharmacotherapy for Sydenham's chorea: where are we and where do we need to be?

INTRODUCTION: Sydenham's chorea (SC) is the most common cause of acquired chorea in children. The existing literature describes it as a benign, self-remitting condition. However, recent evidence discloses the persistence of long-course neuropsychiatric and cognitive complications in adulthood, which imposes to redefine the concept of 'benignity' of such condition. In addition, therapies are mostly empirical and non-evidence based. AREAS COVERED: Here, we conducted an electronic exploration of the PubMed database and selected 165 relevant studies directly correlated to SC treatment. Critical data from selected articles were synthesized to provide an update on pharmacotherapy in SC, which basically consists of three pillars: antibiotic, symptomatic and immunomodulant treatments. Moreover, since SC mostly affects females with recurrences occurring in pregnancy (chorea gravidarum), we focused on the management in pregnancy. EXPERT OPINION: SC is still a major burden in developing countries. The first therapeutic strategy should be the primary prevention of group A beta-hemolytic streptococcal (GABHS) infection. Secondary antibiotic prophylaxis should be performed in every SC patient as the World Health Organization (WHO) guidelines recommend. Symptomatic or immunomodulant treatments are administered according to clinical judgment. However, a greater effort to understand SC physiopathology is needed, together with larger trials, to outline appropriate therapeutic indications.