Opsoclonus: clinical and immunological features.

Abstract

BACKGROUND:

Opsoclonus is felt to be a saccadic oscillation disorder but the neuroanatomical substrate for generating the abnormal eye movements is poorly understood.

METHODS:

We recorded eye movements and studied serum samples from 7 patients who presented with opsoclonus and with either myoclonus or generalized tremor. Anti neuronal antibodies were detected by immunohistochemestry using rat and human cerebellar sections.

RESULTS:

In all patients but one the opsoclonus resolved within 2weeks (after immunosuppression in 4, resection of the underlying neoplasm in 1 and spontaneously in 1). Opsoclonus was arrhythmic and multidirectional with a wide frequency range (4-10Hz). No known paraneoplastic antibodies were found in the initial commercial screen. Three patients had antiPurkinje cell antibodies with a characteristic punctate staining in the molecular layer.

CONCLUSIONS:

The clinical and immunological findings are consistent with the hypothesis, that in some patients, opsoclonus results from antibodies directed at the parallel fiber-Purkinje cell synapse. The antibodies block parallel fiber input to Purkinje cells allowing spontaneous oscillating activity generated in the inferior olives to be passed on to the oculomotor nuclei via the flocculus.

• Authors: Joanna C Jen, Ivan Lopez, Robert W Baloh
• Journal: Journal of the neurological sciences (J Neurol Sci) Vol. 320 Issue 1-2 Pg. 61-5 (Sep 15 2012) ISSN: 1878-5883 [Electronic] Netherlands
• PMID: 22818114 (Publication Type: Journal Article)
• Copyright: Copyright © 2012 Elsevier B.V. All rights reserved.

Chemical References

• Autoantibodies
• Receptors, Glutamate
• glutamate receptor delta 2

Topics

• Adult
• Animals
• Autoantibodies (blood, metabolism)
• COS Cells
• Cell Line, Transformed
• Cerebellum (immunology, metabolism)
• Chlorocebus aethiops
• Eye Movements (immunology, physiology)
• Female
• Humans
• Male
• Middle Aged
• Opsoclonus-Myoclonus Syndrome (blood, diagnosis, immunology)
• Purkinje Cells (metabolism)
• Rats
• Rats, Sprague-Dawley
• Receptors, Glutamate (genetics, metabolism)