Omnipause Neurons in Two Cases of Opsoclonus Associated With Oat Cell Carcinoma of the Lung

Abstract

Opsoclonus is an involuntary eye movement disorder in which there are chaotic, usually conjugate, multidimensional saccadic eye movements. In this paper 2 cases of opsoclonus are reported, as a paraneoplastic phenomenon in association with oat cell carcinoma of the lung. It has previously been hypothesized that opsoclonus results from dysfunction of a group of premotor neurons in the brainstem called omnipause neurons. We describe the location of these cells in man by homology with animal studies, and describe the light microscopic appearance of these neurons in the 2 cases of opsoclonus. Although these neurons appeared normal it is still possible that their function was disturbed as a result of metabolic or neurotransmitter abnormalities.

Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma

“…Anxiety, nervousness and irritability have been reported in children with opsoclonus-myoclonus, but have been infrequently described in adult patients with paraneoplastic opsoclonus-myoclonus [13]. Restlessness or agitation was reported in three patients with a small cell lung cancer and opsoclonus-myoclonus [1,15]. A review, published before the discovery of the anti-Ri antibodies, summarizing 11 adult patients with paraneoplastic opsoclonusmyoclonus mentions nervousness in a female patient with a breast carcinoma [6].…”

Opsoclonus-Myoclonus Syndrome as a Paraneoplastic Manifestation of Renal Cell Carcinoma

“…All paraneoplastic cases reported in the literature occurred in patients over 40 [2][3][4][5][6][7][8][9]. Different types of tumor involving a wide variety of organs have been reported; breast and lung cancer (small cell lung cancer and adenocarcinoma) represent 70% of the described cases [2,9,10]. Most of these patients die within a few months.…”

“…Paraneoplastic manifestations are present in up to 20% of patients with renal cell carcinoma (RCC) (table 1) [1][2][3][4][5][6][7]10]. Opsoclonus is a rare ocular dyskinesia, characterized by involuntary, repetitive and rapid conjugated ocular saccades that are irregular in amplitude and fre- quency and occur in all directions without an intersaccadic interval.…”

Ocular flutter and truncal ataxia may be associated with enterovirus infection

“…Zee and Robinson [10] proposed a hypothetical explanation in which pause cells in the pontine tegmentum are believed to lack proper inhibition over pontine burst neurons. The fact that histological analysis of two patients who had suffered from opsoclonus has failed to show a loss of pause cells [13] suggests that a disorder of inputs to pause cells or burst neurons is a more likely cause of saccadic oscillations. In this regard cerebellar dysfunction, especially of the dorsal vermis and its outputs via the fastigial nucleus, might play a causal role in the pathophysiology of opsoclonus and ocular flutter.…”