-
Journal of Clinical Neuroscience :... Jun 2019Visual snow is the perception of flickering dots throughout the entire visual field and occurs with other symptoms of dysfunctional central sensory processing. We...
Visual snow is the perception of flickering dots throughout the entire visual field and occurs with other symptoms of dysfunctional central sensory processing. We describe a patient who presented with visual snow, illusory visual motion, photopsia, and reduced night vision. He subsequently developed progressive cognitive impairment, myoclonus and ataxia. A diagnosis of sporadic Creutzfeldt-Jakob Disease was confirmed on post-mortem examination more than 49 months after symptom onset. The visual snow syndrome is typically benign, but our patient illustrates that occasionally it is the first manifestation of a serious brain disease. Careful application of the diagnostic criteria for the visual snow syndrome is important, particularly with the use of neuroimaging to exclude pathology in the occipital cortex.
Topics: Creutzfeldt-Jakob Syndrome; Humans; Male; Middle Aged; Vision Disorders
PubMed: 30910546
DOI: 10.1016/j.jocn.2019.03.023 -
Ophthalmology Mar 2004To report the clinical, electrophysiologic, and immunopathologic findings in a patient with progressive retinal degeneration associated with Waldenström's...
OBJECTIVE
To report the clinical, electrophysiologic, and immunopathologic findings in a patient with progressive retinal degeneration associated with Waldenström's macroglobulinemia.
DESIGN
Case report with immunohistochemical studies.
METHODS
A 46-year-old female with elevated serum immunoglobulin M (IgM) levels complained of photophobia and photopsias. Complete ophthalmic examinations including electrophysiologic testing and hematologic evaluations were performed over a 3-year period. Immunohistochemical studies to determine the presence of serum antiretinal antibodies were evaluated using confocal microscopy.
RESULTS
Examination of the patient's fundus remained normal, although there was deterioration in her visual acuity, visual field, and electroretinogram over the follow-up period. Waldenström's macroglobulinemia was diagnosed by bone marrow biopsy. Indirect immunohistochemistry revealed reactivity of the patient's serum against the photoreceptor-connecting cilium.
CONCLUSION
The paraneoplastic retinopathy associated with Waldenström's macroglobulinemia in this patient is presumed to result from antibodies of the IgM subtype reacting to proteins in the retinal photoreceptors.
Topics: Autoantibodies; Autoantigens; Electroretinography; Female; Fluorescent Antibody Technique, Indirect; Humans; Immunoglobulin M; Microscopy, Confocal; Middle Aged; Paraneoplastic Syndromes; Photophobia; Retina; Retinal Diseases; Visual Acuity; Visual Fields; Waldenstrom Macroglobulinemia
PubMed: 15019332
DOI: 10.1016/j.ophtha.2003.05.036 -
Future Oncology (London, England) Jun 2019ALK inhibitors are a new group of tyrosine kinase inhibitors, highly efficient in the treatment of non-small-cell lung carcinoma. However, these targeted therapies can... (Meta-Analysis)
Meta-Analysis Review
ALK inhibitors are a new group of tyrosine kinase inhibitors, highly efficient in the treatment of non-small-cell lung carcinoma. However, these targeted therapies can induce various adverse effects, including ocular toxicity. To date, few articles reporting ophthalmological side effects of ALK inhibitors have been published. In this review, we aim to describe the different side effects and to collect information regarding the causes behind the discrepancy between the reported rates of visual disorders. Frequent ocular side effects of ALK inhibitors included flashes, post-flashbulb effect, stripes, photopsia, accommodation disorder, presbyopia, reduced visual acuity and blurred vision. Optic neuropathy, vitreous floaters, diplopia, cataract and macular edema were also reported.
Topics: Anaplastic Lymphoma Kinase; Antineoplastic Agents; Carcinoma, Non-Small-Cell Lung; Eye Diseases; Humans; Lung Neoplasms; Molecular Targeted Therapy; Orbital Diseases; Protein Kinase Inhibitors
PubMed: 31140869
DOI: 10.2217/fon-2018-0608 -
Brain and Nerve = Shinkei Kenkyu No... Apr 2010Paraneoplastic retinopathy including cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), and paraneoplastic optic neuropathy (PON) are visual... (Review)
Review
Paraneoplastic retinopathy including cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), and paraneoplastic optic neuropathy (PON) are visual disorders associated with systemic cancer. Patients with CAR typically present with progressive loss of vision and photopsia, which are related to dysfunction of both cones and rods in photoreceptors. The triad of photosensitivity, ring scotoma, and a reduced caliber of the retinal arteriole along with undetectable signals in electroretinogram (ERG) are specific manifestations of CAR. CAR is associated most commonly with small-cell lung cancer (SCLC) and occasionally with gynecologic tumors, and it is usually caused by autoantibodies against recovering, which is a calcium-binding photoreceptor protein that participates in the transduction of light. MAR is characterized by shimmering, flickering, or pulsating photopsias, and usually occurs in the patients with a cutaneous melanoma. MAR differs from CAR in terms of visual acuity and color vision and is associated with a characteristic pattern detected in ERG. Autoantibodies against the bipolar cells of the retina have been identified in patients with MAR. Patients with PON frequently present with progressive visual loss and optic disc edema, or with other paneoplastic neurologic syndromes related to SCLC, such as paraneoplastic encephalomyelitis or retinitis, ophthalmoplegia, and subacute cerebellar syndrome. Autoantibodies against collapsin-responsive mediator protein-5 (CRMP-5, also called anti-CV2) are considered to be as the causative factor. Treatments with corticosteroids, plasma exchange, and intravenous immune globulin as well as treatment of the tumor itself, occasionally improves these paraneoplastic visual syndromes. However, the prognosis depends on their underlying malignancy.
Topics: Autoantibodies; Diagnosis, Differential; Humans; Hydrolases; Lung Neoplasms; Melanoma; Microtubule-Associated Proteins; Nerve Tissue Proteins; Optic Nerve Diseases; Paraneoplastic Syndromes, Nervous System; Prognosis; Recoverin; Retinal Diseases; Small Cell Lung Carcinoma
PubMed: 20420177
DOI: No ID Found -
Survey of Ophthalmology 1993Central retinal vein occlusion (CRVO) is usually seen in older adults and is often associated with systemic vascular disease. CRVO can be seen in young adults, and... (Review)
Review
Central retinal vein occlusion (CRVO) is usually seen in older adults and is often associated with systemic vascular disease. CRVO can be seen in young adults, and although it is occasionally associated with a systemic disease, in the majority of cases it occurs in an otherwise healthy patient with no known systemic disease or ocular problem. Inflammation of the central retinal vein has been proposed as a cause of the occlusion in young adults and for that reason it has been called papillophlebitis. The appearance of unilateral optic disc edema, dilatation, and tortuosity of the major retinal veins with a variable amount of retinal hemorrhage in young, healthy adults with complaints of blurred vision or photopsias has been called, in addition to papillophlebitis, benign retinal vasculitis, optic disc vasculitis, nonischemic CRVO, big blind spot syndrome, and presumed phlebitis of the optic disc. An approach to the diagnostic evaluation of the young adult with CRVO is presented. Although most eyes recover vision to better than 20/40, about one-fifth have significant visual loss, and many suffer ocular sequelae. Many treatment modalities have been tried for this entity, but no conclusive evidence exists that any treatment alters its natural history.
Topics: Adolescent; Adult; Age Factors; Female; Fluorescein Angiography; Fundus Oculi; Humans; Male; Prognosis; Retinal Vein Occlusion; Sex Factors
PubMed: 8516752
DOI: 10.1016/0039-6257(93)90138-w -
IDCases 2020A healthy 47-year-old immunocompetent man from Northern Canada presented for ophthalmologic assessment after experiencing one month of right-sided photopsias, floaters,...
A healthy 47-year-old immunocompetent man from Northern Canada presented for ophthalmologic assessment after experiencing one month of right-sided photopsias, floaters, and a right lower nasal quadrant visual field defect. Optic disc swelling, vitritis, chorioretinitis, peripheral retinal infiltrates and hemorrhages were noted in the right eye. A broad right inferior arcuate and nasal visual field defect were also present. Fluorescein angiography of the right retina showed dilated disc vessels and staining of the optic disc. Treponemal antibody testing, using chemiluminescent microparticle immunoassay, was highly positive; this was followed by a Venereal Disease Research Laboratory (VDRL) test with a titre of 1:32 and confirmed by Treponema pallidum particle agglutination (TP-PA) test. Testing did not demonstrate any co-infections. Cerebrospinal fluid (CSF) analysis revealed strong reactivity (4+) to the Treponemal antibody by immunofluorescence antibody absorbed (FTA-ABS) test and non-reactivity by CSF VDRL test. Syphilis PCR of CSF was negative. A diagnosis of neurosyphilis was made. He was treated with ceftriaxone 2 grams IV q24h for 14 days. The vitritis gradually improved. Familiarity with syphilis diagnostics is becoming increasingly important, especially given its recent resurgence amongst several at risk groups. This patient's case highlights that non-reactive CSF VDRL is not a reliable test in the context of positive serum results and a compatible clinical picture. CSF Treponemal tests such as TP-PA and FTA-ABS offer higher sensitivity than non-treponemal tests such as VDRL in the context of CNS involvement and ocular syphilis.
PubMed: 32528847
DOI: 10.1016/j.idcr.2020.e00840 -
Advances in Experimental Medicine and... 2018Autoimmune retinopathy (AIR) was often mistaken for retinitis pigmentosa (RP), due to an overlap of clinical findings, but increasingly has been recognized as a unique...
Autoimmune retinopathy (AIR) was often mistaken for retinitis pigmentosa (RP), due to an overlap of clinical findings, but increasingly has been recognized as a unique entity in the last decade. AIR has distinctive features: sudden onset of photopsias and scotomata in patients with no family history of RP, followed by visual field and central vision loss. Initially, retina exams are normal with no sign of pigment deposits or retinal degeneration. A family history of autoimmune diseases (all types) occurs in 60% of patients. One hallmark of AIR has been the presence of anti-retinal autoimmune antibodies (ARAs) in patients' sera, but patients can continue to have ARAs even when the disease has been quiescent for years. The accumulation of ARAs represents a breakdown of retinal immune tolerance with many different immunoreactive bands found at different reference weights in AIR patients. We began investigating cellular immunity using flow cytometry and found abnormal distributions (>2 StDev) of increased memory lymphocytes and NK cells and decreased regulatory B cell subsets in many AIR patients compared to normal controls. Culture of patient lymphocytes with small amounts (25 μg) of recoverin protein for 6 days led to significant elevations of interferon gamma (IFNγ) and in some cases tumor necrosis factor alpha (TNFα) production. We found the IFNγ/IL-10 ratio in response to recoverin was elevated in patients with more active disease (defined by visual field contraction between visits), but in some patients, there also appeared to be independent factors influencing severity, suggesting other autoimmune mechanisms were at play. These cellular immune parameters may provide improved markers for active AIR.
Topics: Autoantibodies; Autoimmune Diseases of the Nervous System; Blotting, Western; Cells, Cultured; Diagnosis, Differential; Gene Expression Profiling; Humans; Immunity, Cellular; Immunologic Memory; Interferon-gamma; Killer Cells, Natural; Lymphocyte Subsets; RNA, Messenger; Receptor, Transforming Growth Factor-beta Type I; Receptors, CCR7; Recoverin; Retinitis; Retinitis Pigmentosa; Tumor Necrosis Factor-alpha
PubMed: 29721944
DOI: 10.1007/978-3-319-75402-4_24 -
Journal of Neuro-ophthalmology : the... Mar 2021
Topics: Adult; Drug-Related Side Effects and Adverse Reactions; Female; Humans; Immune Checkpoint Inhibitors; Magnetic Resonance Imaging; Melanoma; Nerve Fibers; Nivolumab; Optic Neuritis; Papilledema; Retinal Ganglion Cells; Skin Neoplasms; Tomography, Optical Coherence; Vision Disorders; Visual Acuity; Visual Fields; Melanoma, Cutaneous Malignant
PubMed: 32108668
DOI: 10.1097/WNO.0000000000000909 -
Supplements To Clinical Neurophysiology 2006
Review
Topics: Hallucinations; Humans; Migraine with Aura; Perceptual Distortion; Phosphenes; Psychotic Disorders
PubMed: 16893099
DOI: 10.1016/s1567-424x(09)70018-2 -
Indian Journal of Ophthalmology Jul 2021
Topics: Cataract; Cataract Extraction; Humans; Retinal Diseases; Vision Disorders
PubMed: 34146030
DOI: 10.4103/ijo.IJO_545_21