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Corneal Reflex

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621. Hereditary sensory and autonomic neuropathy, type 4

signs of the disease. The cardinal feature is absence of sweating on the trunk and extremities, with occasional patients producing some moisture on the forehead, tip of the nose and gluteal sulcus. The skin becomes thick and callused with lichenification of palms, areas of hypotrichosis on the scalp and dystrophic nails. Deep tendon reflexes are usually present. Pain insensitivity is profound resulting in self-mutilation, auto-amputation, and corneal scarring but some patients retain temperature

2007 Orphanet

622. Hereditary sensory and autonomic neuropathy, type 2

preponderance. Clinical description Disease onset is typically in infancy and is non-progressive. Initial symptoms (from birth to 3 years) include lack of crying with trauma, self-mutilation (tongue, lips), swallowing and feeding problems. Gastroesophageal reflux is common. Sensory dysfunction is manifested by reduced or absent pain and temperature perception, and depressed or absent deep tendon reflexes. Corneal reflexes are reduced or absent. Muscle strength is preserved and there is no atrophy. Sensation (...) to fine touch, position, vibration, taste, and gag reflexes may be diminished. Unrecognized injuries (e.g., burns, skin and corneal ulcers) and fractures of hands, feet, and limbs, sometimes resulting in osteomyelitis, as well as Charcot joints are frequent. Some patients have hearing loss. Autonomic involvement is limited to reduced lacrimation. Patients do not typically have orthostatic hypotension or sweating abnormalities. Etiology Causal mutations in several genes have been identified and include

2005 Orphanet

623. Lowe syndrome

The estimated prevalence is 1/500,000 and males are almost exclusively affected. Clinical description OCRL is a neonatal disorder characterized by ocular abnormalities (bilateral congenital discoid cataracts, glaucoma with or without buphthalmos, strabismus, hypermetropia and corneal and conjunctival cheloids), neurological involvement (developmental delay, seizures, hypotonia present at birth typically with absence of deep tendon reflexes), stereotypic behavior (temper tantrums, aggressiveness

2006 Orphanet

624. Familial dysautonomia

of information on the Orphanet website are accepted. For all other comments, please send your remarks via . Only comments written in English can be processed. Check this box if you wish to receive a copy of your message * " for="captcha" >Please reproduce the text below: * Familial dysautonomia Disease definition A rare hereditary sensory and autonomic neuropathy characterized by decreased pain and temperature perception, absent deep tendon reflexes, proprioceptive ataxia, afferent baroreflex failure (...) tears is normal until about seven months of age). No obvious dysmorphism is present at birth, but a characteristic facial expression develops over time. Pain and temperature perception are decreased, but not absent. Proprioception and vibration sense are markedly decreased. Deep tendon reflexes are absent. Feeding difficulties due to gastrointestinal dysmotility (oropharyngeal incoordination, abnormal esophageal peristalsis, erratic gastric emptying, gastroesophageal reflux) occur early and may

2005 Orphanet

625. What other possible causes of watering eye are there other than blocked tear ducts or infection which have been discounted?

caused by emotional or reflex stimulation of the lacrimal gland. Chronic lacrimation may deplete the lacrimal gland to such an extent that tear formation appears normal or reduced. Major causes: • corneal foreign body, abrasion or inflammation • entropion Less frequently: • crocodile tears • psychic stimulation - crying • cholinergic or anticholinesterase drugs • dacryoadenitis • lacrimal gland tumours.” [2] Concerning epiphora, GPNotebook states: “ This describes excessive tearing due to defective

2007 TRIP Answers

626. Alternative Dosing and Regimen of Replagal to Treat Fabry Disease

and 1 month after the last infusion. Safety evaluations are done periodically and include vital sign measurements, physical examination, blood and urine tests, review of drug side effects, electrocardiogram (ECG), Holder monitor (2 hour ECG), and QSART (NIH only). The QSART (quantitative sudomotor axon reflex test) measures the amount of sweat in a particular area of skin, mostly the forearm. For this test, a cup partly filled with a liquid is strapped on the arm. A weak electric current is turned (...) in plasma or serum or less than 8% of average mean normal in leukocytes). Subject must have one or more clinical manifestations of Fabry disease including neuropathic pain, angiokeratoma, corneal verticillata, cardiomyopathy, hypo- or anhydrosis, abdominal pain and/or diarrhea, serum creatinine greater than 1.0 mg/dl or proteinuria greater than 300 mg/24 hours. Subject must have voluntarily signed an Institutional Review Board (IRB) approved informed consent form after all relevant aspects of the study

2004 Clinical Trials

627. Intra-arterial Microplasmin Administration in Patients With Acute Intracranial Vertebrobasilar Artery Occlusion

of the onset of neurological symptoms Age 18-75 (inclusive). Exclusion Criteria: Patients with coma > 6 hrs duration and complete loss of brain stem reflexes (corneal reflex, gag reflex, VOR, pupil reflexes) as measured at the last assessment before sedation/intubation Rapidly improving neurologic signs at any time before initiation of study drug administration. Known contrast agent-sensitivity Uncontrolled hypertension defined as a systolic blood pressure > 180 mm Hg or a diastolic blood pressure > 100 mm

2005 Clinical Trials

628. Recovery of Visual Acuity in People With Vestibular Deficits

of driving with resultant diminished independence and, ultimately, limited social interactions and increased isolation. Oscillopsia occurs because of inadequate vestibulo-ocular reflex (VOR) gain and suggests that compensation for the vestibular loss has not occurred. The purpose of this study was to examine the effect of an exercise intervention on visual acuity during head movement in patients with unilateral and bilateral vestibular hypofunction. We hypothesized that 1) patients performing vestibular (...) ] performance test Eye Movements: Scleral Search Coil [ Time Frame: pre- and post-treatment ] eye movements are measured by having the participant sit within an electromagnetic field while wearing a scleral coil (like a contact lens but only in contact with the sclea, not the cornea); te coil moves with eye movement and distorts the electrimagnetic field Eligibility Criteria Go to Information from the National Library of Medicine Choosing to participate in a study is an important personal decision. Talk

2006 Clinical Trials

629. Efficacy of T1675 Versus Placebo in Patients With Bilateral Treated Moderate Dry Eye Syndrome

, BUT and Schirmer, performed within the last 12 months before Inclusion Visit, for both eyes. Bilateral symptomatology suggestive of dry eye defined by: at least one of the following ocular symptoms suggestive of dry eye (burning, stinging, dryness feeling, sandy and/or gritty sensation, light sensitivity, reflex tearing, ocular fatigue) and Questioning on patient’s feeling (score >=3). Fulfilling the following criteria of dry eye syndrome in both eyes defined by: Keratoconjunctivitis defined by a lissamine (...) -GARCHER, Professor CHU of Dijon, France More Information Go to Layout table for additonal information ClinicalTrials.gov Identifier: Other Study ID Numbers: LT1675-PIICA-04/04 PHASE II First Posted: July 27, 2006 Last Update Posted: July 27, 2006 Last Verified: July 2006 Additional relevant MeSH terms: Layout table for MeSH terms Syndrome Keratoconjunctivitis Sicca Dry Eye Syndromes Disease Pathologic Processes Keratoconjunctivitis Conjunctivitis Conjunctival Diseases Eye Diseases Keratitis Corneal

2006 Clinical Trials

630. Efficacy and Safety Study for Ecabet Ophthalmic Solution for Treating Dry Eye Syndrome

that could interfere with study Diagnosis of Sjogren's syndrome, lacrimal obstruction, reflex, lid-related or contact lens-related dry eye syndrome(DES); significant anterior blepharitis or meibomianitis Contraindications or hypersensitivity to use of study meds or components Wear contact lenses Secondary dry eye to surgery Eye surgery (including laser) within 6 months Use of systemic or topical ophthalmic meds within 14 days Punctal plugs in one or both eyes in place for <45 days Permanent occlusion (...) -CS01 First Posted: September 20, 2005 Last Update Posted: March 14, 2013 Last Verified: March 2013 Additional relevant MeSH terms: Layout table for MeSH terms Syndrome Keratoconjunctivitis Sicca Dry Eye Syndromes Disease Pathologic Processes Keratoconjunctivitis Conjunctivitis Conjunctival Diseases Eye Diseases Keratitis Corneal Diseases Lacrimal Apparatus Diseases Pharmaceutical Solutions Ophthalmic Solutions Ecabet Anti-Infective Agents Anti-Ulcer Agents Gastrointestinal Agents Protease

2005 Clinical Trials

631. Electrical stimulation of the rostral medial prefrontal cortex in rabbits inhibits the expression of conditioned eyelid responses but not their acquisition Full Text available with Trip Pro

Electrical stimulation of the rostral medial prefrontal cortex in rabbits inhibits the expression of conditioned eyelid responses but not their acquisition We have studied the role of rostral medial prefrontal cortex (mPFC) on reflexively evoked blinks and on classically conditioned eyelid responses in alert-behaving rabbits. The rostral mPFC was identified by its afferent projections from the medial half of the thalamic mediodorsal nuclear complex. Classical conditioning consisted of a delay (...) paradigm using a 370-ms tone as the conditioned stimulus (CS) and a 100-ms air puff directed at the left cornea as the unconditioned stimulus (US). The CS coterminated with the US. Electrical train stimulation of the contralateral rostral mPFC produced a significant inhibition of air-puff-evoked blinks. The same train stimulation of the rostral mPFC presented during the CS-US interval for 10 successive conditioning sessions significantly reduced the generation of conditioned responses (CRs) as compared

2007 Proceedings of the National Academy of Sciences of the United States of America

632. Oriental oculopalpebral dimensions: Quantitative comparison between Orientals from Japan and Brazil Full Text available with Trip Pro

palpebral surgery, and crossbreeding. Images were obtained with a digital camera, 30 cm from the frontal plane at pupil height, with the individual in a primary position and the eye trained on the camera lens. Images were transferred to computer and processed by the Scion Image program. Measurements were made of distance between medial canthi, distance between pupils (IPD), superior eyelid crease position, distance between the superior lid margin and corneal reflexes (MRD), horizontal width, height

2008 Clinical ophthalmology (Auckland, N.Z.)

633. Evoking Blinks with Natural Stimulation and Detecting Them with a Noninvasive Optical Device: A Simple, Inexpensive Method for Use with Freely Moving Animals Full Text available with Trip Pro

to deliver puffs of air to the cornea of freely moving rats during our studies of eyeblink conditioning. The stimulus evokes an unconditioned response that can be recorded without affecting the EMG signal. This allows a complete analysis of the unconditioned response which is important for studies examining reflex modification or the effect of drugs, genetic manipulations, or aging on the unconditioned blink reflex. We also describe an infrared reflective sensor that can be added to the tether

2008 Journal of neuroscience methods

634. Treating the Ocular Component of Allergic Rhinoconjunctivitis and Related Eye Disorders Full Text available with Trip Pro

the cornea, and research has revealed that involvement of different immune cell populations (mast cells, eosinophils, and lymphocytes) may cause these more severe symptoms. A variety of treatment options exist to control ocular allergy symptoms. Nonpharmacologic options include allergen avoidance and lubrication with saline, and if these fail to be sufficiently effective, symptom relief may be provided by medicinal agents that are either applied topically to the eye or taken orally. Recent evidence (...) suggests that nasal allergy treatments applied topically to the nose may also positively affect ocular allergy symptoms, which raises the interesting possibility that a parasympathetic nasal-ocular neural reflex pathway may be involved in the stimulation of allergic responses in the eye.Ocular allergy is underdiagnosed and has a significant impact on the life of the patient. It is vital to reach a better understanding of ocular allergic mechanisms and inflammation, which may lead to improved treatment.

2007 Medscape General Medicine

635. Botulinum toxin for aberrant facial nerve regeneration: double-blind, placebo-controlled trial using subjective endpoints. (Abstract)

facial movements using videotaping and a physicians' grading scale.The videotape measurements of corneal light reflex to upper lid margin distance and vertical palpebral distance were made during adynamic and active facial expression. Thirty-six patients were studied (six in an open-label pilot study design and 30 in a multicenter, double-blind, placebo-controlled trial). Botulinum toxin type A injections consistently suppressed the degree of involuntary eyelid movement associated with smiling

2005 Plastic and reconstructive surgery

636. [Pharmacodynamics of two different propofol formulations]. (Abstract)

, based on measured arterial propofol plasma concentrations. Clinical pharmacodynamics were assessed by reaction on acoustic stimuli, eyelid reflex and corneal reflex.The drugs did not differ in pharmacodynamics with respect to EEG (EC(50) 2.1+/-0.6 for Diprivan and 2.1+/-0.5 microg/ml for Propofol Fresenius) and clinical signs. The pharmacodynamic model was characterized by a steep concentration effect relationship and a distinct hysteresis between propofol plasma concentration and effect (k(e0) 0.12

2006 Der Anaesthesist Controlled trial quality: uncertain

637. Treatment of Lower Eyelid Malposition with Dermis Fat Grafting. (Abstract)

immunodeficiency virus-associated lipodystrophy were treated with midfacial lifting combined with dermis fat posterior lamellar spacer grafting.Preoperative and postoperative measurements of eyelid position, margin-to-reflex distance (defined as the distance from the upper eyelid to the central corneal light reflex and the distance from the lower eyelid to the corneal light reflex), lagophthalmos, corneal staining, presence of ocular surface symptoms, and patient satisfaction.All patients who underwent dermis

2007 Ophthalmology

638. Cannabinoid antagonist AM 281 reduces mortality rate and neurologic dysfunction after cecal ligation and puncture in rats. (Abstract)

-iminoethyl)-lysine (4 mg/kg), was injected intraperitoneally immediately after CLP to produce the CLP + L-N6-(1-iminoethyl)-lysine group to exclude the influence of depressed hemodynamics on neurologic impairment.It was found that administration of AM 281 could prevent the hemodynamic changes induced by sepsis. Reflex responses, including the pinna, corneal, paw or tail flexion, and righting reflexes, and the escape response significantly decreased in the CLP and CLP + L-N6-(1-iminoethyl)-lysine groups (...) at 48 hrs after the surgery. In contrast, no changes in these reflex responses were found between the CLP + AM 281 and control and sham groups. In addition, no effects of the administration of AM 281 on neurologic function and mortality rate in the control group were found. Tissue caspase-3 levels were elevated at 48 hrs after CLP in the CLP alone group (means +/- sd: control, 3.9 +/- 0.4; sham, 4.2 +/- 0.4; CLP, 7.1 +/- 1.0 [p < .01]; CLP + AM 281, 4.0 +/- 0.5 densitometric units). In addition

2005 Critical Care Medicine

639. Strabismus in senile cataract patients. (Abstract)

was measured by the prism and cover test or prism and corneal reflex test.Mean preoperative deviation of the study group was 17.8 +/- 9.7 prism diopters (PD) of exophoria/tropia. In this group, 70.2% had exophoria and 27.7% had exotropia. In the control group exophoria was found in 75% of the persons whereas none of them had any heterotropia. Mean deviation in the control group was 4.7 +/- 5.1 PD of exophoria. Postoperatively, the angle of exophoria/tropia improved to 12.8 +/- 8.5 PD, which was different

2005 JAAPOS - Journal of the American Association for Pediatric Ophthalmology and Strabismus

640. Pediatric Fabry disease. Full Text available with Trip Pro

age: 12.3 +/- 3.5 years) and 21 age-matched control subjects.Quality of life (measured with the Child Health Questionnaire) and sweating (assessed with the quantitative sudomotor axon reflex test).Quality of life scores for pediatric patients <10 years of age with Fabry disease, compared with published normative values, were 55 +/- 17 vs 83 +/- 19 for bodily pain and 62 +/- 19 vs 80 +/- 13 for mental health. Bodily pain scores for patients > or =10 years of age were 54 +/- 22 vs 74 +/- 23. Sweat (...) volume in the Fabry disease group was 0.41 +/- 0.46 microL/mm2, compared with 0.65 +/- 0.44 microL/mm2 in the control group. Renal function, urinary protein excretion, and cardiac function and structure were normal for the majority of patients. The 3 patients with residual alpha-galactosidase A activity > or =1.5% of normal values were free of cornea verticillata and had normal serum and urinary globotriaosylceramide levels. All other children had glycolipid levels comparable to those of adult

2005 Pediatrics

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