Journal français d'ophtalmologie
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Case Reports
[Devic's neuromyelitis optica: diagnosis after 10 years of bilateral severe relapsing optic neuritis].
Neuromyelitis optica, also known as Devic's disease, is a severe idiopathic inflammatory demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis have now been recognized. ⋯ This case is an illustration of the new criteria in the diagnosis of NMO, underscoring the importance of the positive serum NMO-Ig G antibody to distinguish multiple sclerosis from NMO. It also emphasizes that asymptomatic brain lesions are common in NMO on brain MRIs and symptomatic brain lesions do not exclude its diagnosis.
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Case Reports
[Ophthalmologic disease in sarcoid-like granulomatosis and true sarcoidosis in immunodeficiency. Four case reports].
Granulomatosis lesions occurring after diagnosis of primary or secondary immunodeficiency are not accidental and have been described in a small number of patients suffering from various diseases: common variable immunodeficiency (CVID), malignancy (lymphoma and solid tumors), and acquired immunodeficiency syndrome (AIDS). Two types of granulomatosis can appear: true sarcoidosis and sarcoid-like reaction. We report four patients, two with CVID and two with malignancy, in whom clinical granulomatosis appeared a few months to a few years after diagnosis of immunodeficiency. ⋯ The underlying pathophysiology responsible for the association between granuloma formation and immunodeficiency in the same patient remains obscure. It may be quite difficult to distinguish true sarcoidosis and sarcoid-like reaction. It is possible that these two entities are the clinical extremes of a common pathological process.
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We report three cases of optic disc drusen in children. The children were 5 months, 10 years, and 11 years old. Two of them were male. ⋯ Optic disc calcification on CT scan was identified in only one case. In children, optic disc drusen are often buried, leading to pseudopapilledema appearance of the optic disc. Ultrasonography is highly sensitive in detecting optic disc drusen, making more expensive investigations unnecessary.
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Historical Article
[History of the evaluation of medicines aiming for marketing authorization].
The European Directive on Medicines Evaluation and Marketing Authorization were issued in 1975. For more than 30 years, Marketing Authorization criteria have been defined as pharmaceutical and biological quality, therapeutic efficacy, and safety. The application comes from the pharmaceutical company and must include the full data on drug development. French procedures have always included practical assessment of the drug by health practitioners: clinicians, pharmacists, biologists, and specialists in biostatistics.
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A case of Susac syndrome in a child is reported with a review of the literature. A 14-year-old girl presented with headache, left hemiparesis, sphincter deficit, and cognitive deficits. The assessment consisted of neurological and ocular examination, imagery by cerebral magnetic resonance, lumbar puncture, and a biological and immunological assessment. ⋯ However, sensory and neurologic sequelae may be present. Treatment is not well codified and may include steroids, immunosuppressant drugs, and immunoglobulin. Susac syndrome should be considered in children when evaluating patients with branch retinal artery occlusion and encephalopathy.