Opsoclonus: Clinical and immunological features

Jen, Joanna C.; López, Iván; Baloh, Robert W.

doi:10.1016/j.jns.2012.06.017

Cited by 27 publications

(15 citation statements)

References 22 publications

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“…Another hypothesis of how opsoclonus might be generated suggests that antibodies block PF to PuC synapses, thus reducing inhibition on the flocculus. This allows spontaneous oscillatory activity in the IOs to be passed to the ocular motor nuclei ( 17 ). This hypothesis has already been tested in the context of oculopalatal tremor (OPT) ( 39 , 40 ).…”

Section: Resultsmentioning

confidence: 99%

“…Mutant mice with a modified glutamate receptor on PuC show clustered PuC action potentials, likely induced by climbing fiber activation, and opsoclonus-like eye movements ( 16 ). Jen et al ( 17 ) found antibodies to PuC in patients with opsoclonus. They proposed that those antibodies blocked the parallel fiber (PF) input to PuC, allowing spontaneous oscillations generated in the inferior olives (IO) to be passed to the oculomotor vermis through the flocculus.…”

Section: Introductionmentioning

confidence: 99%

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A GABAergic Dysfunction in the Olivary–Cerebellar–Brainstem Network May Cause Eye Oscillations and Body Tremor. II. Model Simulations of Saccadic Eye Oscillations

Optican

Pretegiani

2017

Front. Neurol.

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Eye and body oscillations are shared features of several neurological diseases, yet their pathophysiology remains unclear. Recently, we published a report on two tennis players with a novel presentation of eye and body oscillations following self-administration of performance-enhancing substances. Opsoclonus/flutter and limb tremor were diagnosed in both patients. Common causes of opsoclonus/flutter were excluded. High-resolution eye movement recordings from one patient showed novel spindle-shaped, asymmetric saccadic oscillations (at ~3.6 Hz) and ocular tremor (~40-60 Hz). Based on these findings, we proposed that the oscillations are the result of increased GABAA receptor sensitivity in a circuit involving the cerebellum (vermis and fastigial nuclei), the inferior olives, and the brainstem saccade premotor neurons (excitatory and inhibitory burst neurons, and omnipause neurons). We present a mathematical model of the saccadic system, showing that the proposed dysfunction in the network can reproduce the types of saccadic oscillations seen in these patients.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A GABAergic Dysfunction in the Olivary–Cerebellar–Brainstem Network May Cause Eye Oscillations and Body Tremor. II. Model Simulations of Saccadic Eye Oscillations

Optican

Pretegiani

2017

Front. Neurol.

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“…Aunque la patogénesis no está bien dilucidada, se cree que es de origen autoinmune, dada la presencia de autoanticuerpos descritos tanto en adultos como en niños (8)(9)(10). El 50 % de los casos de síndrome opsoclonus mioclonus ataxia en la población pediátrica están asociados con neuroblastoma (11).…”

Section: Discussionunclassified

Síndrome de Kinsbourne: reporte de un caso

Paredes-Ebratt

Espinosa

2017

iatreia

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El síndrome de Kinsbourne, conocido también como "síndrome de opsoclonus-mioclonus" o "síndrome de los ojos danzantes", es una enfermedad rara caracterizada por movimientos oculares rápidos, irregulares, multidireccionales (opsoclonus), movimientos mioclónicos en tronco, cara y/o extremidades y ataxia; se presenta entre los 6 y 36 meses de edad. Su etiología puede ser paraneoplásica (neuroblastoma), no paraneoplásica (infecciosa) o idiopática. Independientemente de la causa, se han utilizado inmunosupresores para reducir la formación de anticuerpos posiblemente involucrados en su fisiopatología.Presentamos el caso de una niña lactante de 21 meses con este síndrome secundario a un cuadro respiratorio viral. Tuvo ataxia, opsoclonus, mioclonías de miembros superiores, irritabilidad y alteración en el patrón de sueño. Se descartó el diagnóstico de neuroblastoma. El tratamiento inicial se hizo con pulsos de metilprednisolona seguida de prednisolona oral. Sus síntomas desaparecieron progresivamente y recuperó la marcha; actualmente está asintomática y sin secuelas. En estos pacientes se debe descartar siempre un síndrome paraneoplásico.

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“…13,14 In general, the presence of opsoclonus is a clue to the opsoclonus-myoclonus syndrome, which is highly indicative of a paraneoplastic or other autoimmune etiology. [15][16][17] The presence and extent of other neurologic signs and symptoms such as cognitive impairment, seizures, psychiatric symptoms, neuropathy, movement abnormalities (e.g., myoclonus, tremors, dystonia), hearing impairment, ocular abnormalities such as uveitis or chorioretinitis, and autonomic features vary across the specific antibody syndromes and are described in detail in the Specific Autoimmune Disorders section.…”

Section: Clinical Presentation Of Autoimmune Vestibulocerebellar Disomentioning

confidence: 99%

Autoimmune Vestibulocerebellar Syndromes

2020

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Autoimmune disorders affecting the vestibular end organs, vestibular pathways, vestibular nuclei, and vestibulocerebellum are often underrecognized as a cause of chronic dizziness and ataxia. Autoantibodies specific for cell-surface, synaptic, and intracellular neural antigens serve as biomarkers of these disorders. This article describes the epidemiology, clinical presentation, diagnostic considerations, imaging findings, treatment, and prognosis of autoimmune disorders, in which the vestibulocerebellar syndrome is the main or presenting clinical presentation. Antibodies specific for intracellular antigenic targets described in the article are PCA-1 (Purkinje cell cytoplasmic antibody type 1, also known as anti-Yo), ANNA-1 (antinuclear neuronal antibody type 1, also known as anti-Hu), ANNA-2 (antinuclear neuronal antibody type 2, also known as anti-Ri), Ma1/2 (anti-Kelch-like 11/12 antibody), Kelch-like 11, amphiphysin, CV2 (collapsin response 2, also known as collapsin response mediator protein-5 [CRMP5]), VGCC (voltage-gated calcium channel), GAD65 (glutamic acid decarboxylase 65-kDa isoform), AP3B2 (adaptor protein 3B2, also known as anti-Nb), MAP1B (microtubule-associated protein 1B antibody, also known as anti-PCA-2), and neurochondrin antibodies. Antibodies targeting cell-surface or synaptic antigenic targets described in the article include DNER (delta/notchlike epidermal growth factor related receptor; antigen to anti-Tr), CASPR2 (contactin-associated proteinlike 2), septin-5, Homer-3, and mGluR1 (metabotropic glutamate receptor 1). The vestibulocerebellar presentation is largely indistinguishable among these conditions and is characterized by subacute onset of cerebellar symptoms over weeks to months. The diagnosis of autoimmune vestibulocerebellar syndromes is based on a combination of clinical and serological features, with a limited role for neuroimaging. Subtle eye movement abnormalities can be an early feature in many of these disorders, and therefore a meticulous vestibulo-ocular examination is essential for early and correct identification. Cancer occurrence and its type are variable and depend on the autoantibody detected and other cancer risk factors. Treatment comprises immunotherapy and cancer-directed therapy. Acute immunotherapies such as intravenous immunoglobulin, plasma exchange, and steroids are used in the initial phase, and the use of long-term immunosuppression such as rituximab may be necessary in relapsing cases. Outcomes are better if immunotherapy is started early. The neurologic prognosis depends on multiple factors.

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Opsoclonus: Clinical and immunological features

Cited by 27 publications

References 22 publications

A GABAergic Dysfunction in the Olivary–Cerebellar–Brainstem Network May Cause Eye Oscillations and Body Tremor. II. Model Simulations of Saccadic Eye Oscillations

A GABAergic Dysfunction in the Olivary–Cerebellar–Brainstem Network May Cause Eye Oscillations and Body Tremor. II. Model Simulations of Saccadic Eye Oscillations

Síndrome de Kinsbourne: reporte de un caso

Autoimmune Vestibulocerebellar Syndromes

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