Cases reported "Vision Disorders"

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1/20. Intermittent visual loss and exophthalmos due to the Blue rubber bleb nevus syndrome.

    PURPOSE: To describe the occurrence of intermittent visual loss in a patient with orbital hemangioma due to the blue rubber bleb nevus syndrome. methods: Case report. RESULTS: A 70-year-old woman complained of intermittent blurred vision combined with pressure behind her left eye occurring after head bending or Valsalva maneuvers. Examination revealed 2 mm of enophthalmos on the left. After downward bending of the head the eye became exophthalmic by 12 mm and visual acuity decreased to finger counting. Neuro-ophthalmologic examination was otherwise normal. magnetic resonance imaging revealed an intraconal hemangioma. Bluish, soft, rubber-like, compressible and slowly refilling hemangiomas on the inferior lip, sublingually, on the hand, and vagina led to the diagnosis of BRBNS. CONCLUSIONS: Intermittent visual loss may be the presenting symptom of an orbital hemangioma in BRBNS. Recognition of this rare condition is important because of its potentially life-threatening complications.
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2/20. Parasellar syndromes.

    The parasellar compartments are located lateral to and on either side of the sella turcica. The cavernous sinuses are the most prominent anatomic feature of the parasella. Each sinus consists of a plexus of veins through which runs the intracavernous portion of the internal carotid artery. Ocular motor nerves three and four travel within the dural covering of the cavernous sinus to the superior orbital fissure, and cranial nerve six travels through the carotid sinus itself, giving rise to parasellar syndromes, which have distinctive clinical features. ophthalmoplegia occurs as a result of damage to these ocular motor nerves and variable involvement of oculosympathetic nerves. facial pain, dysesthesia, and paraesthesia are caused by damage to one or more of the divisions of the fifth cranial nerve, travelling in the dural wall of the cavernous sinus. Tumors, such as meningiomas, frequently cause parasellar syndromes, as do aneurysms of the intracavernous portion of the internal carotid artery, carotid-cavernous fistulas, and cavernous sinus thrombosis. Inflammatory conditions such as tolosa-hunt syndrome, ischemia to small vessels supplying the cavernous portion of the cranial nerves, and infections can cause this syndrome. magnetic resonance imaging is the investigation of choice and therapy is specific to the cause of the parasellar syndrome, but now includes more aggressive endoscopic and microsurgical intervention, and radiosurgery.
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3/20. Neurologic and cardiac progression of glycogenosis type VII over an eight-year period.

    Little is known about the progression of phosphofructokinase deficiency (glycogenosis type VII, Tarui's disease). We describe a 66-year-old woman who had this disease diagnosed in 1997. Initial manifestations had included simple partial seizures since 1977, anginal chest pain since 1982, and muscle cramps since 1983. To prevent recurrent myocardial infarction, anticoagulation therapy with phenprocumon was initiated. Cardiac involvement progressed over an 8-year period, manifesting as low-voltage electrocardiogram (ECG), ectopic supraventricular tachycardia, thickened mitral valve, mitral valve insufficiency, enlarged left atrium, left ventricular hypertrophy, and diastolic dysfunction. Progression of neurologic involvement manifested as complex partial seizures, double vision, reduced tendon reflexes, central facial palsy, bradydiadochokinesia, and distal weakness of the upper extremities. Discontinuance of oral anticoagulation after 19 years, initiation of enalapril therapy, and administration of carbamazepine markedly improved the patient's condition.
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4/20. alendronate-induced auditory hallucinations and visual disturbances.

    A 79-year-old Caucasian woman who had been taking alendronate 10 mg/day for over 2 years to prevent osteoporosis reported hearing "voices in her head" along with red-colored visual disturbances. These auditory hallucinations and visual disturbances began shortly after her regimen was changed from alendronate 10 mg/day to 70 mg once/week. Assessment of causality using the Naranjo and Jones algorithms revealed a "probable" and "highly probable" relationship, respectively, between this adverse drug event and the switch from daily to weekly alendronate therapy. Other bisphosphonates, such as etidronate and pamidronate, have caused both reversible and irreversible auditory, visual, and olfactory hallucinations beginning 2 hours--1 week after drug administration. The mechanism behind these adverse effects is unknown but is thought to be independent of calcium homeostasis. Clinicians should be aware of central nervous system toxicity as a rare but potential adverse effect associated with bisphosphonates.
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5/20. Atypical neuropsychological profile in a boy with 22q11.2 Deletion Syndrome.

    In this article the general and specific cognitive impairments of the boy R.H. with a de novo deletion 22q11.2 are described. His full-scale IQ was 73, and he obtained only slightly better verbal than non-verbal subtest scores. Neuropsychological assessment revealed specific impairments in perceptual categorization of objects presented suboptimal, matching of unfamiliar faces, and verbal learning and memory. In contrast, he performed in accordance with his intelligence level on other visual perceptual tasks, on non-verbal learning and memory tasks, and on attention tasks. Voxel-wise statistical comparison of a high-resolution T1-weighted magnetic resonance image of R.H's brain with similar images obtained from 14 normal control children revealed as major abnormalities a reduction of the right inferior parietal and superior occipital lobe, and a bilateral reduction of deep white matter behind the inferior frontal gyrus. These cognitive impairments and MRI abnormalities are not commonly described in 22q11.2 Deletion Syndrome and may indicate a larger heterogeneity in the neurocognitive phenotype than currently evidenced. At least in this boy the microdeletion seems to have interfered with the development and functioning of particular neural subsystems, while the structure and functioning of other subsystems was left intact.
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6/20. Purtscher's retinopathy after fracture dislocation of shoulder joint.

    Purtscher described sudden blindness in patients with severe head trauma due to a remote retinopathy, characterised by bilateral retinal haemorrhages, cotton wool spots, and optic disc swelling seen on fundoscopy. A similar retinopathy has been reported in compressive chest trauma, long bone fractures, and acute pancreatitis. It is less well recognised that Purtscher's retinopathy can occur unilaterally and following less severe trauma. We present a case of unilateral remote traumatic retinal angiopathy following a fracture dislocation of the shoulder joint.
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7/20. Hyperbaric oxygen treatment in Purtscher's retinopathy induced by chest injury.

    We present a patient who had Purtscher's retinopathy in both eyes after sustaining a chest contusion. After multiple sessions of hyperbaric oxygen (HBO) therapy were given, visual function and retinal appearance improved. This case suggests that HBO therapy may be a treatment option for Purtscher's retinopathy.
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keywords = chest
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8/20. Monocular accommodative fluctuations associated with temporomandibular joint syndrome.

    A young scientist reported severe headaches emanating from behind one eye with an associated intermittent monocular blur. The monocular near cross-cylinder test revealed an accommodative fluctuation of one eye alone with a range of approximately 3 D. Referral to a neuro-ophthalmologist failed to produce a diagnosis for the disorder. A subsequent routine visit to a dentist provided a diagnosis of Costen's temporomandibular joint (TMJ) syndrome, a condition that is commonly misdiagnosed as a variety of neurological and vascular disorders.
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9/20. Benign essential blepharospasm.

    A 57-year-old female had vague visual complaints of pressure behind her right eye during an ocular examination. One month later the patient was visually impaired due to uncontrollable spasms of both eyelids. A diagnosis of benign essential blepharospasm (BEB) was made, and the patient was eventually treated with botulinum toxin to temporarily relieve her symptoms. I have reviewed and summarized the literature relating to the characteristics and management of this often misdiagnosed and misunderstood ocular muscular condition.
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10/20. Computed tomography of the visual pathways.

    Many articles have discussed the anatomy and pathology of the orbits. Although this area is exquisitely visualized via computed tomography, the bulk of the visual apparatus lies behind the optic canal. A variety of pathologic conditions will manifest themselves by changes in the visual fields. This article demonstrates the normal anatomy of the visual pathways within the brain by CT scanning and correlates various changes that cause visual field defects.
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