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Opsoclonus myoclonus syndrome (OMS), also known as opsoclonus-myoclonus-ataxia (OMA), is a rare neurological disorder of unknown cause which appears to be the result of an autoimmune process involving the nervous system. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year.
Opsoclonus consists of rapid, involuntary, multivectorial (horizontal and vertical), unpredictable, conjugate fast eye movements without inter-saccadic intervals. [1] It is also referred to as saccadomania or reflexive saccade. The movements of opsoclonus may have a very small amplitude, appearing as tiny deviations from primary position.
Kinsbourne was the first to identify and systematically describe the infant disorder "opsoclonus myoclonus" syndrome, sometimes called Kinsbourne syndrome in his honor. He also was first to describe "hiatus hernia" with contortions of the neck, subsequently called Sandifer Syndrome in honor of his mentor, Dr. Paul Sandifer.
Myoclonus is a brief, involuntary, irregular (lacking rhythm) twitching of a muscle, a joint, or a group of muscles, different from clonus, which is rhythmic or regular. Myoclonus (myo-"muscle", clonus "spasm") describes a medical sign and, generally, is not a diagnosis of a disease.
Symptomatic features of paraneoplastic syndrome cultivate in four ways: endocrine, neurological, mucocutaneous, and hematological.The most common presentation is a fever (release of endogenous pyrogens often related to lymphokines or tissue pyrogens), but the overall picture will often include several clinical cases observed which may specifically simulate more common benign conditions.
Opsoclonus-myoclonus syndrome (OMS) is a condition that develops in children as a result of anti-Hu antibodies. The illness afflicts younger children, with one study showing an age range of about 2 months to 10 years, with the majority of cases falling between 6 months to 3 years. The first symptoms are nonspecific.
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Opsoclonus myoclonus syndrome: Central nervous system, eye movement control Anti-neuronal antibodies Possible [105] Optic neuritis: Optic nerve Various Confirmed [106] Scleritis: Sclera: Various Possible [107] Susac's syndrome: Retina, cochlea, and brain None specific Probable [108] Sympathetic ophthalmia: Uveal tract: Various Probable Rare [109]