Neuro-Ophthalmologic Manifestations of Paraneoplastic Syndromes
STATE OF THE ART Neuro-Ophthalmologic Manifestations of Paraneoplastic Syndromes Melissa W. Ko, MD, Josep Dalmau, MD, PhD, and Steven L. Galetta, MD Abstract: Paraneoplastic syndromes with neuro- PARANEOPLASTIC CEREBELLAR ophthalmologic manifestations may involve the cen DEGENERATION tral nervous system, cranial nerves, neuromuscular Paraneoplastic cerebellar degeneration (PCD) is junction, optic nerve, uvea, or retina. Most of these a syndrome of subacute severe pancerebellar dysfunction disorders are related to immunologic mechanisms (Table 1). Initially, patients present with gait instability. presumably triggered by the neoplastic expression of Over several days to weeks, they develop truncal and limb neuronal proteins. Accurate recognition is essential to ataxia, dysarthria, and dysphagia. The cerebellar disease appropriate management. eventually stabilizes but leaves patients incapacitated. PCD is most commonly associated with cancers of (/Neuro-Ophthalmol 2008;28:58-68) the lung, ovary, and breast and with Hodgkin disease (7). Ocular motor manifestations include nystagmus, ocular dysmetria, saccadic pursuit, saccadic intrusions and he term "paraneoplastic neurologic syndrome" (PNS) oscillations, and skew deviation (8). Over the last 30 refers to dysfunction of the nervous system caused by T years, at least nine anti-neuronal antibodies have been a benign or malignant tumor via mechanisms other than associated with PCD. However, only about 50% of patients metastasis, coagulopathy, infection, or treatment side with suspected PCD test positive for anti-neuronal anti effects (1). Whereas reports of possible paraneoplastic bodies in serum or cerebrospinal fluid (CSF) (9). Anti-Yo neuropathies extend back to the 19th century, cerebellar syndromes associated with cancer were first described by and anti-Tr are the autoantibodies most commonly Brouwerin 1919 (2).
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