Cerebellar degeneration and ocular myasthenia gravis in a patient with recurring ovarian carcinoma

Abstract

Myasthenia gravis (MG) and paraneoplastic cerebellar degeneration (PCD) are immune-mediated syndromes that can represent paraneoplastic disorders. We report a patient with history of ovarian carcinoma that presented with ptosis, diplopia and gait ataxia. Neurophysiological examination and laboratory tests revealed the presence of MG and PCD. An integrated FDG-PET/contrast-enhanced CT scan showed tumor recurrence. This is to the best of our knowledge the first association of MG and PCD with recurring ovarian carcinoma.

A Factor Found in the Igg Fraction of Serum of Patients With Paraneoplastic Bilateral Diffuse Uveal Melanocytic Proliferation Causes Proliferation of Cultured Human Melanocytes

“…12 Some paraneoplastic syndromes have been attributed to factors produced by the cancer, whereas others have been strongly associated with the production of antibodies by the immune system, including ocular myasthenia gravis, carcinoma-associated retinopathy, and central neurologic paraneoplastic syndromes, including paraneoplastic cerebellar degeneration. [11][12][13] The latter is most commonly associated with anti-Purkinje cell antibodies. It is believed that certain antigens produced by the ovarian cancer induce the production of antibodies that then cross-react with the cells that normally express those antigens.…”

“…2,11 The associations are mainly descriptive, and the pathophysiology is not well understood. The most commonly associated paraneoplastic syndrome is dermatomyositis.…”

Myasthenia as a paraneoplastic manifestation of ovarian Cancer

“…These findings were subsequently confirmed by imaging tests. Caliandro et al ( 2010 ) was the first to describe the concomitance between myasthenia and ovarian tumors. However, establishing the causality of the conditions was more unlikely given that myasthenia only developed during the recurrence of ovarian cancer.…”

“…Myasthenia gravis (MG) is an immune-mediated neurological disease that affects neuro-muscular transmission. Approximately 10% of MG cases consist in paraneoplastic disorders ( Dalmau & Rosenfeld, 2010 ; Toothaker & Rubin, 2009 ), mainly in patients with thymoma ( Caliandro et al, 2010 ; Toothaker & Rubin, 2009 ) but rarely with other tumors ( Dalmau & Rosenfeld, 2010 ). Antibodies active against acetylcholine receptors (AChR-Abs) are typically detected; however, 10–20% of the carriers do not exhibit this marker.…”

“…Antibodies active against acetylcholine receptors (AChR-Abs) are typically detected; however, 10–20% of the carriers do not exhibit this marker. Nerve stimulus tests, such as repetitive nerve stimulation (RNS) and single-fiber electromyography (SF-EMG), are important in the diagnostic confirmation of this entity ( Caliandro et al, 2010 ).…”