Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies

Gallerini, Simone; Marsili, Luca; Marconi, Roberto

doi:10.1001/jamaneurol.2016.1161

Cited by 2 publications

(2 citation statements)

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“…OMS is a rare syndrome for which a diagnosis of definite PNS can be made without the presence of an antibody [16,[39][40][41]. Clinically, OMS is described by opsoclonus (conjugate fast and multidirectional saccades without intersaccadic pauses), non-epileptic myoclonus and, variably, ataxia.…”

Section: Opsoclonus-myoclonus Syndromementioning

confidence: 99%

“…Definite PNS has the highest score (≥8) and is characterized by a "high-risk" clinical phenotype and antibody, as well as confirmation of a specific cancer [16]. The only exception is represented by OMS associated with neuroblastoma/small cell lung cancer where no specific antibodies are recognized [16,39]. A recent retrospective, multicenter study showed how misdiagnosis of autoimmune encephalitis may be frequent, even at specialized centers [10].…”

Section: Diagnosismentioning

confidence: 99%

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Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

et al. 2023

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Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associated with antibodies against neural surface antigens, known as “intermediate- or low-risk” antibodies, are less frequently associated with cancer. In this narrative review, we will focus on PNS of the central nervous system (CNS). Clinicians should have a high index of suspicion with acute/subacute encephalopathies to achieve a prompt diagnosis and treatment. PNS of the CNS exhibit a range of overlapping “high-risk” clinical syndromes, including but not limited to latent and overt rapidly progressive cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome, paraneoplastic (and limbic) encephalitis/encephalomyelitis, and stiff-person spectrum disorders. Some of these phenotypes may also arise from recent anti-cancer treatments, namely immune-checkpoint inhibitors and CAR T-cell therapies, as a consequence of boosting of the immune system against cancer cells. Here, we highlight the clinical features of PNS of the CNS, their associated tumors and antibodies, and the diagnostic and therapeutic strategies. The potential and the advance of this review consists on a broad description on how the field of PNS of the CNS is constantly expanding with newly discovered antibodies and syndromes. Standardized diagnostic criteria and disease biomarkers are fundamental to quickly recognize PNS to allow prompt treatment initiation, thus improving the long-term outcome of these conditions.

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Section: Opsoclonus-myoclonus Syndromementioning

confidence: 99%