Clinical and Immunological Features of Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies

Armangué, Thaís; Sabater, Lidia; Torres-Vega, Estefanía; Martínez‐Hernández, Eugenia; Ariño, Helena; Petit‐Pedrol, Mar; Planagumà, Jesús; Bataller, Luís; Dalmau, Josep; Graus, Francesc

doi:10.1001/jamaneurol.2015.4607

Cited by 172 publications

(162 citation statements)

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“…Clinical presentation of OMS is characterized by the combination of rapid, involuntary, multidirectional, conjugate saccades of ocular movements without an intersaccadic interval (opsoclonus) and brief, shock-like involuntary movements of muscles (myoclonus) that predominantly involves trunk and limbs. Additional clinical features such as ataxia, tremors, dysarthria and psychiatric symptoms are usually observed in OMS [1,5]. In adults, patients with OMS can be paraneoplastic or idiopathic, the former conveys worse prognosis than the latter [5].…”

Section: Discussionmentioning

confidence: 99%

“…Additional clinical features such as ataxia, tremors, dysarthria and psychiatric symptoms are usually observed in OMS [1,5]. In adults, patients with OMS can be paraneoplastic or idiopathic, the former conveys worse prognosis than the latter [5]. Typical malignancies associated with POMS in adults are small cell lung carcinoma and breast cancer whereas neuroblastoma in pediatric population [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…Typical malignancies associated with POMS in adults are small cell lung carcinoma and breast cancer whereas neuroblastoma in pediatric population [1,3]. Moreover, other types of cancer such as ovarian teratoma, testicular seminoma, gastric, thymus, kidney and oropharyngeal carcinoma have been identified in association with POMS [5]. The pathophysiology of POMS remains ambiguous.…”

Section: Discussionmentioning

confidence: 99%

“…Immune mediated mechanism may play an important role where detection of antineuronal antibodies and neuronal surface antigens in serum and CSF of the patients were evident. Most of the POMS may not be associated with well characterized antibodies with the exception of breast carcinoma which is usually associated with anti-Ri antibodies [3,4,5,7]. Literatures have supported that disruption of the tonic inhibitory control of saccadic neurons by the omnipause neurons in the pontine reticular formation could be a possible cause of opsoclonus.…”

Section: Discussionmentioning

confidence: 99%

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Rare Paraneoplastic Opsoclonus Myoclonus Syndrome (POMS) in a Recurrent Breast Cancer Patient: Importance of Early Recognition of POMS and Initiation of Rehabilitation Intervention

Sh¹,

Tp²,

YeowLeng³

2017

JCR

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Background: Opsoclonus myoclonus syndrome is a rare condition characterized by the amalgamation of multidirectional saccades of eye movements, involuntary myoclonus predominantly affecting trunk and limbs and cerebellar ataxia. Typical malignancies associated with paraneoplatic opsoclonus myoclonus syndrome (POMS) in adults are small cell lung carcinoma and breast cancer with variable outcomes. Case Report: The authors report here a patient with stage IV recurrent right breast cancer, presenting to the hospital with unsteady gait, jerky movements of the limbs and body. She was clinically diagnosed with POMS with positive cerebrospinal fluid anti-Ri antibody. Medical treatment with intravenous methylprednisolone and early initiation of rehabilitation improved her balance, coordination and ataxia. This led to an overall improvement in her physical function. Conclusion: This case illustrates the importance of early recognition, prompt treatment of POMS and initiation of rehabilitation in achieving better functional outcomes.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Rare Paraneoplastic Opsoclonus Myoclonus Syndrome (POMS) in a Recurrent Breast Cancer Patient: Importance of Early Recognition of POMS and Initiation of Rehabilitation Intervention

Sh¹,

Tp²,

YeowLeng³

2017

JCR

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“…40 % of paraneoplastic origin and in 60 % idiopathic or presumably of parainfectious etiology (e. g., associated with HIV, mumps, CMV, EBV) [76]. In older patients ( > 50 years) with paraneoplastic OMS, lung cancer or breast cancer (typically with anti-Ri-Abs) are frequently detected.…”

Section: Opsoclonus-myoclonus Syndrome (Oms)mentioning

confidence: 99%

Management of Immune-Mediated Paraneoplastic Neurological Disorders

Ayzenberg

Gold

Kleiter

2017

Neurology International Open

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AbsTR Ac TParaneoplastic neurological disorders are rare and clinically heterogeneous diseases. They can affect both the central and peripheral nervous system as well as the neuromuscular junction and muscle. Neurological deficits develop in 2/3 of cases prior to cancer diagnosis. The diagnostic approach includes screening for antineuronal antibodies and a search for the underlying tumor. A prompt tumor therapy in combination with immunotherapy is the cornerstone in the management of these diseases. Due to lack of clinical trials, treatment recommendations are based on case series and expert opinions. Highdose corticosteroids, intravenous immunoglobulins and apheresis therapies are often used in the acute stage of the disease. These therapies should be started as early as possible, e. g., during tumor screening, in order to prevent irreversible damage. Long-term treatment is mostly immunosuppressive and depends on the specific paraneoplastic syndrome. Outcomes vary depending upon the prognosis of the underlying cancer and the nature of the antineuronal antibodies. Disorders with antibodies directed against antigens on the neuronal cell surface are highly sensitive to B cell-directed therapies and mostly associated with a favorable outcome. A thorough review of published data on actual treatment recommendation is provided along with discussion of currently not validated, but potentially effective new therapies. E264Ayzenberg I et al. Management of Immune-mediated Paraneoplastic … Neurology International Open 2017; 1: E264-E274 course, treatment response and prognosis of PNS [2,3]. Depending on tumor association, obligate and facultative paraneoplastic Abs are differentiated (Tab. 1).Clinically, the following PNS exist [3,4]: A. In the central nervous system: ▪ Encephalomyelitis (incl. brainstem encephalitis) ▪ Limbic encephalitis ▪ Subacute cerebellar degeneration ▪ Opsoclonus-myoclonus syndrome ▪ Optic neuritis/neuromyelitis optica ▪ Myelopathy/myelitis ▪ Stiff person syndrome ▶Table 1 Paraneoplastic antibodies: associated neurological syndromes and tumors. Target antigen clinical presentation# Association with tumorObligate paraneoplastic Abs (directed against intracellular antigens; tumor association > 95 %) In this review we discuss the diagnosis and management of PNS. With regard to facultative paraneoplastic syndromes which are more frequently of autoimmune origin, such as neuromyelitis optica or myasthenia gravis, the reader should refer to pertinent reviews [5,6]. Diagnostic and Pathogenetic Significance of Antibodies to Intracellular and Surface AntigensAntibodies (Abs) to intracellular antigens or so-called "classical" Abs are almost always tumor-associated (exception: GAD-Abs). They can be associated with various tumors and a variety of neurological conditions [7]. However, a direct pathogenetic significance of these Abs has not yet been demonstrated. Abs to intracellular antigens serve as useful diagnostic markers in the targeted tumor search in PNS. Here, cell-mediated immune responses play a major p...

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