No abstract available.
Dexamethasone ; Paraneoplastic Syndromes, Ocular ; Plasma

Cancer associated retinopathy (CAR) syndrome is a very rare ocular manifestation of paraneoplastic syndrome, and is characterized clinically by progressive visual impairment. Immune cross-reactivity between antigens in the cancerous tissue and antigens in the retina may play an important role in its pathogenesis, and most of cases are associated with lung carcinoma, particularly small cell lung cancer. The clinical triad of CAR is described as photosensitivity, ring scotomata, and an attenuated retinal arterial caliber. Here, we report a 61-year old male patient with CAR syndrome, who had small cell lung carcinoma in the stage of limited disease, with a brief review of the relevant literature.
Humans ; Lung ; Male ; Middle Aged ; Paraneoplastic Syndromes ; Paraneoplastic Syndromes, Ocular* ; Retina ; Retinaldehyde ; Small Cell Lung Carcinoma* ; Vision Disorders

Ocular flutter is a relatively rare eye movement disorder that refers to occasional bursts of involuntary horizontal oscillation around the point of fixation, characterized by rapid, repetitive, horizontal, symmetrical and sinusoidal oscillation without inter- saccadic interval. It is known to be associated with disorders of the cerebellum and brainstem resulting from various pathological conditions, but it's anatomical and pathophysiological basis remains unclear. Here, we present the case of a patient who had severe ocular flutter associated with Merkel cell carcinoma.
Brain Stem ; Carcinoma, Merkel Cell* ; Cerebellum ; Humans ; Ocular Motility Disorders ; Paraneoplastic Syndromes

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.
Brain ; Dizziness ; Humans ; Magnetic Resonance Imaging ; Myoclonus ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Paraneoplastic Syndromes, Nervous System ; Prevalence

Autoimmune retinopathy (AIR) is an immune-mediated retinopathy, resulting from an immunologic process caused by the aberrant recognition of retinal antigens as autoantigens. The diagnosis of AIR involves the detection of antiretinal antibodies with concurrent clinical and electrophysiological evidence of retinopathy. A 40-year-old patient presented with progressive loss of bilateral vision over several months. A fundus examination was unremarkable. Spectral domain optical coherence tomography revealed a blurred photoreceptor ellipsoid zone at the subfoveal region in both eyes with more prominent disruption in the left eye. Full-field electroretinography (ERG) showed relatively normal rod and cone responses in the right eye, and decreased photopic bwaves with minimal attenuation of a-waves in the left eye. Multifocal ERG demonstrated slightly reduced amplitude of the inner segment ring in the right eye and decreased amplitudes and delayed latencies of all modalities in the left eye. The patient was suspected to have AIR and it was supported by positive Western blots for 23-kDa protein, enolase (46-kDa), aldolase (40-kDa), 62-kDa and 78-kDa proteins and by immunohistochemical staining of human retinal bipolar and ganglion cells. Despite the immunosuppressive treatment, the destruction of the retinal photoreceptors progressed, and immunosuppressive interventions produced very little visual improvement. We report on what is, to the best of our knowledge, the very first case of serologically confirmed nonparaneoplastic AIR in Korea.
Autoantibodies/*blood/immunology ; Autoantigens ; Autoimmune Diseases/*immunology ; Electroretinography ; Humans ; Immunologic Factors ; Paraneoplastic Syndromes/*immunology ; Paraneoplastic Syndromes, Ocular ; Phosphopyruvate Hydratase ; Recoverin ; Republic of Korea ; Retina/*immunology ; Retinal Diseases/*immunology ; Tomography, Optical Coherence

PURPOSE: To report a case of cancer-associated retinopathy developed in a patient with small cell lung cancer, which is a kind of paraneoplastic syndrome. METHODS: A 78-year-old woman presented complaining of decreased visual acuity and visual field that had developed about 15 days previously. She was diagnosed with small cell lung cancer 1.5 years ago and underwent 3 cycles of chemotherapy. At presentation, the best-corrected visual acuity was hand motion in both eyes and there was no afferent pupillary defect. Slit-lamp biomicroscopic examination revealed no specific abnormality in the anterior segment of either eye, and intraocular pressure was normal. Posterior segment examination demonstrated remarkable arteriolar narrowing in both eyes, but there was little doubt about the presence of an optic nerve lesion such as optic disc edema or pallor. RESULTS: Fluorescein angiography and brain magnetic resonance imaging (MRI) revealed no significant abnormalities. However, electroretinograms (ERG) demonstrated marked reduction in the a and b waves. Visual evoked response was delayed for the latency period. She was treated with systemic steroid, after which her visual acuity gradually improved.
Aged ; Brain ; Drug Therapy ; Edema ; Evoked Potentials, Visual ; Female ; Fluorescein Angiography ; Hand ; Humans ; Intraocular Pressure ; Latency Period (Psychology) ; Magnetic Resonance Imaging ; Optic Nerve ; Pallor ; Paraneoplastic Syndromes ; Paraneoplastic Syndromes, Ocular* ; Pupil Disorders ; Small Cell Lung Carcinoma* ; Visual Acuity ; Visual Fields

BACKGROUND: While tonic pupils have been attributed to various diseases, including syphilis, herpes zoster, orbital trauma, temporal arteritis, endometriosis, and paraneoplastic syndromes, obstructive hydrocephalus has not been implicated. CASE REPORT: A 36-year-old woman visited a neurology department with a 7-day history of throbbing headache and blurred vision in both eyes. She had early dorsal midbrain syndrome mimicking an Adie's tonic pupil, and cholinergic supersensitivity was demonstrated using topical 0.125% pilocarpine. Brain MRI revealed obstructive hydrocephalus at the level of the aqueduct of Sylvius, and her symptoms resolved 4 days after surgery. CONCLUSIONS: We report a patient with early dorsal midbrain syndrome that was initially believed to represent a tonic pupil on the basis of pharmacologic testing. The findings in our patient suggested that early dorsal midbrain syndrome mimicking an Adie's tonic pupil can be caused by obstructive hydrocephalus compressing the Edinger-Westphal nucleus.
Adult ; Brain ; Cerebral Aqueduct ; Endometriosis ; Eye ; Female ; Giant Cell Arteritis ; Headache ; Herpes Zoster ; Humans ; Hydrocephalus ; Mesencephalon ; Neurology ; Orbit ; Paraneoplastic Syndromes ; Pilocarpine ; Syphilis ; Tonic Pupil ; Vision, Ocular

PURPOSE: The dancing eye syndrome is a rare neurological condition of unknown etiology characterized by multidirectional chaotic eye movement(opsoclonus), myoclons and ataxia. In children, it could be a paraneoplastic syndrome in association with neuroblastoma. Long-term neurological sequelae and decreased visual acuity are major problem in these patients. This study was done retrospectively to evaluate the clinical course and neurologic sequelae of the dancing eye syndrome and we also investigated in association with neuroblastoma. METHODS: We retrospectively reviewed the medical records of 5 childrens who were admitted to Pediatric Neurology, College of Medicine, Yonsei University for dancing eye syndrome from 1990 to 2001. RESULTS: The range of age was from 4 months to 2 years 9 months, 4 of them were female and the rest one was male. The mean follow up duration was 4 years 6 months. The associated diseases were hypoxic ischemic encephalopathy(2 cases), congenital aniridia(1 case). Neuroblastoma was identified in only 1 children, stage 2A. Four of them had recurrence of opsoclonus and visual acuity were getting worse(near blindness in 1 case). Four patients had a long-term neurological sequelae. The long-term neurological sequelae was dysarthria, learning disorder, seizure, ataxia. CONCLUSION: MIBG(Metaiodobenzylguanidine) scan and abdominal sonography are highly effective in the detection of neuroblastoma. Steroid therapy seems to be effective in opsoclonus in acute stage but did not necessarily have a good long term neurological outcome as it recurred. In conclusion, major problem in dancing eye syndrome is not opsoclonus and myoclonus in acute stage but decreased visual acuity and long-term neurological sequelae.
Ataxia ; Blindness ; Child ; Dancing* ; Dysarthria ; Female ; Follow-Up Studies ; Humans ; Learning Disorders ; Male ; Medical Records ; Myoclonus ; Neuroblastoma ; Neurology ; Ocular Motility Disorders ; Paraneoplastic Syndromes ; Recurrence ; Retrospective Studies ; Seizures ; Visual Acuity