Paraneoplastic opsoclonus in adults

Edward J Dropcho MD (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released August 17, 1995; last updated May 19, 2015; expires May 19, 2018

Overview

Opsoclonus is one of the most dramatic syndromes in clinical neurology. In many children and adults, opsoclonus occurs as a remote effect of what is otherwise an occult neoplasm. Nearly all children with paraneoplastic opsoclonus have a neuroblastoma. Among adults, carcinoma of the lung or breast is most often associated with paraneoplastic opsoclonus, with reports of a wide variety of other tumors. Patients generally have some combination of myoclonus, ataxia, and generalized or multifocal encephalitis in addition to the ocular motor findings. Paraneoplastic opsoclonus is generally believed to have an autoimmune etiology; some patients have 1 of a number of serum antineuronal antibodies. Some patients show significant neurologic improvement with successful tumor therapy or immunotherapy, whereas others are left with permanent and severe neurologic disability despite treatment. The author reviews the clinical features, autoimmune aspects, and practical patient management of paraneoplastic opsoclonus.

Key points

 

• Small cell lung carcinoma and breast carcinoma are the tumors most often associated with paraneoplastic opsoclonus in adults.

 

• Most adults with paraneoplastic opsoclonus additionally have diffuse myoclonus and ataxia.

 

• Patients with paraneoplastic opsoclonus may have one of a number of serum "onconeural" antibodies, or may have no identifiable autoantibodies.

 

• The neurologic outcome of adults with paraneoplastic opsoclonus is variable: some patients improve dramatically with tumor treatment and/or immunotherapy, whereas others are left with severe permanent neurologic disability.

Historical note and terminology

The term "opsoclonus" was first used by Orzechowski in 1927 in reference to chaotic, repetitive, rapid eye movements observed in several patients with nonepidemic encephalitis. At the bedside, opsoclonus is generally defined as involuntary, chaotic saccades in all planes (horizontal, vertical, torsional), worsened by attempts at voluntary saccades or fixation. In electro-oculographic recordings, opsoclonus is characterized by bursts of saccadic oscillations without an intersaccadic interval; some saccades are disconjugate. Opsoclonus occurs in association with numerous disorders in patients of all ages. As part of a paraneoplastic syndrome, opsoclonus most often occurs in children with neuroblastoma (Dropcho 2002). Case reports of opsoclonus in adults with a variety of associated neoplasms date back more than 50 years. Paraneoplastic opsoclonus is best considered a physical sign, rather than a disease entity, because it occurs in a number of heterogeneous neurologic syndromes with differing immunologic abnormalities.

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