Abstract

Paraneoplastic neurologic syndromes (PNSs) are a heterogeneous group of disorders caused by the remote effects of cancer with immune-mediated pathogenesis. Anti-Ma2 antibody was defined as one of the well-characterized onconeural antibodies that could help establish a definite PNS diagnosis. We aimed to report and explore patients with anti-Ma2 antibody-associated paraneoplastic neurologic syndrome (Ma2-PNS) who frequently exhibit sensorimotor neuropathy (SMN) using a new method of factor analysis of mixed data (FAMD). Clinical data from a case series of eight patients with definite diagnoses were retrospectively reviewed. FAMD conducted further analyses with a comprehensive visualization in R software. Our cohort, with a predominance of females (5/8), presented more frequently with SMN (4/8), followed by limbic encephalitis (LE) (3/8). Two patients with LE were found to have a testicular germ-cell tumor and a thymoma, respectively. In addition, a patient who developed chronic SMN was diagnosed with multiple myeloma (MM) involving multiple organs. FAMD exhibited the overall features into a two-dimensional coordinate and located each individual into their corresponding position with high relevance. It provided a clue for determining their potential relationships and predictors. Our findings indicated that Ma2-PNS could frequently involve the peripheral nervous system, MM might be one of its associated cancers with a presentation of chronic SMN, and FAMD might be a clinically valuable tool.

Highlights

  • Paraneoplastic neurologic syndromes (PNSs) are a heterogeneous group of disorders caused by the remote effects of cancer with immune-mediated pathogenesis [1,2]

  • Except for 3 patients with a shorter disease course ranging from 19 days to 2 months, 5 patients had a chronic course lasting more than half a year

  • A 64-year-old female patient admitted to the hospital 2 years earlier for myasthenia gravis, and thymoma with lymphatic metastasis underwent thymoma resection and chemotherapy at that time

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Summary

Introduction

Paraneoplastic neurologic syndromes (PNSs) are a heterogeneous group of disorders caused by the remote effects of cancer with immune-mediated pathogenesis [1,2]. They could affect any part of the nervous system from the cerebral cortex to neuromuscular junctions and muscle. Classical syndromes indicated the neurological syndromes associated frequently with cancer, including encephalomyelitis, limbic encephalitis (LE), subacute cerebellar degeneration, opsoclonusmyoclonus, subacute sensory neuronopathy, chronic gastrointestinal pseudo-obstruction, Lambert–Eaton myasthenic syndrome, and dermatomyositis. Anti-Ma2 antibody was defined as one of the well-characterized onconeural antibodies that could help establish a definite PNS diagnosis [3]. Considering the complex and variable performance of this disease, a novel statistical method must explore the underlying relationships among each feature

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