1/11. Optic disc edema in neonatal onset multisystem inflammatory disease (NOMID).PURPOSE: To inform ophthalmologists about neonatal onset multisystem inflammatory disease (NOMID), a rare condition with ophthalmologic manifestations. methods: We report a single case of NOMID with optic disc edema. RESULTS: A 28-month-old child with neonatal rash, arthropathy, central nervous system (CNS) involvement, and optic disc edema was diagnosed with NOMID. CONCLUSIONS: The finding of posterior uveitis or optic disc edema in a child with juvenile onset arthritis may allow the differentiation of NOMID from juvenile rheumatoid arthritis.- - - - - - - - - - ranking = 1keywords = multisystem inflammatory disease, inflammatory disease, multisystem (Clic here for more details about this article) |
2/11. Neurologic, ophthalmic, and neuropsychiatric manifestations of pediatric systemic lupus erythematosus.BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune, multisystem disorder with diverse manifestations. There are limited reports on the neuro-ophthalmic and neuropsychiatric findings in childhood SLE. CASE REPORT: An 8-year-old patient with suspected SLE with a history of skin rash, joint pain, transient hematologic abnormality, headaches, seizures, and psychosis, presented with papilledema secondary to acquired, obstructive hydrocephalus. DISCUSSION: SLE-related ocular and neuropsychiatric findings are reviewed. Proposed mechanisms for ocular and cerebral lupus include immune complex deposition, vascular thrombosis, and postinflammatory lesions. CONCLUSION: The prevalent neurologic and neuropsychiatric complications of pediatric SLE require close follow-up and interdisciplinary management.- - - - - - - - - - ranking = 0.00012183850278923keywords = multisystem (Clic here for more details about this article) |
3/11. Chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood.OBJECTIVE: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. DESIGN: Descriptive case-report study. SETTING: International collaborative study based on a questionnaire. RESULTS: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. CONCLUSION: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis.- - - - - - - - - - ranking = 1.0016616627768keywords = multisystem inflammatory disease, inflammatory disease, multisystem (Clic here for more details about this article) |
4/11. Long-term complications in Hunter's syndrome.This report describes the clinical and investigative findings in a 31-year-old man with Hunter's syndrome, showing evidence of severe multisystem involvement. Papilloedema has been present for at least 8 years with no evidence of progressive visual impairment. His most serious and life-threatening complications are laryngeal oedema and tracheal narrowing, which both compromise his respiratory function and constitute a major anaesthetic risk.- - - - - - - - - - ranking = 0.00012183850278923keywords = multisystem (Clic here for more details about this article) |
5/11. interferon-alpha 2a treatment of neuro-Behcet disease.Behcet disease is a multisystemic, chronic, recurrent inflammatory disorder characterized as a triad of hypopyon uveitis, oral aphthae, and genital ulcers. Neurologic involvement in Behcet disease (neuro-Behcet) is common. Neuro-Behcet disease typically manifests late after disease onset, rarely coincides with ocular involvement, and often heralds a poor prognosis for final vision and survival. We present a case of neuro-Behcet disease with neurologic onset concomitant with systemic and ocular involvement that was responsive to treatment with interferon-alpha 2a.- - - - - - - - - - ranking = 0.00012183850278923keywords = multisystem (Clic here for more details about this article) |
6/11. Optic perineuritis: clinical and radiographic features.BACKGROUND: Optic perineuritis is an uncommon variety of orbital inflammatory disease that is distinct from demyelinating optic neuritis. OBJECTIVE: To describe the clinical and radiographic features of idiopathic optic perineuritis, with particular emphasis on those features that help to distinguish this condition from optic neuritis. methods: We reviewed the medical records of 14 patients with optic perineuritis who were seen in 2 neuro-ophthalmology clinics. RESULTS: patients ranged in age from 24 to 60 years; 5 were older than 50 years. All patients had visual loss, eye pain, or both. The visual acuity was 20/20 or better in 8 of the 15 eyes. The results of visual field testing were normal in 2 eyes, and a paracentral scotoma or an arcuate defect was seen in 7. magnetic resonance imaging scans demonstrated circumferential enhancement around the optic nerve, sometimes with intraorbital extension. Response to corticosteroids was dramatic; however, 4 patients had a relapse with lowering of the dose. CONCLUSIONS: In contrast to those with optic neuritis, patients with optic perineuritis are often older at onset and are more likely to show sparing of central vision. magnetic resonance imaging scans demonstrate enhancement around, rather than within, the optic nerve. Response to corticosteroids is more dramatic than in patients with optic neuritis, and patients are more likely to experience recurrence after stopping treatment.- - - - - - - - - - ranking = 0.00041541569419415keywords = inflammatory disease (Clic here for more details about this article) |
7/11. Cranial hypertension as first manifestation of Behcet's disease: a case report.Behcet's disease (BD) is a chronic, multisystem and relapsing vasculitis of unknown etiology. central nervous system (CNS) involvement is reported in 30% of cases, but it is the first symptom of the disease in only 5% of subjects. Neurological manifestations may appear as a parenchymal CNS pattern (the commonest), an intracranial hypertension-like pattern, or a meningitis-like pattern. We describe a 30-year-old Algerian man with BD who developed, as first symptom, a typical intracranial hypertension picture with headaches, bilateral papilledema and raised cerebrospinal fluid (CSF) pressure. magnetic resonance angiography (MRA) revealed a cerebral venous sinus thrombosis (VST). After 1 month of treatment with anticoagulants, prednisone, colchicine and chlorambucil, MRA showed complete recanalization of the cerebral venous sinus and the patient made a full recovery. BD should be routinely looked for in adult patients, especially males in their third and fourth decades, who present with intracranial hypertension syndrome. We therefore advocate the use of MRA for unexplained neurological symptoms in BD, since without it cerebral VST may easily be missed.- - - - - - - - - - ranking = 0.00012183850278923keywords = multisystem (Clic here for more details about this article) |
8/11. The pseudopapilledema of neonatal-onset multisystem inflammatory disease.PURPOSE: To report a rare case of neonatal-onset multisystem inflammatory disease with serial photographs to characterize the optic nerve findings. DESIGN: Observational case report. methods: A 6-year-old girl with neonatal-onset multisystem inflammatory disease, who had received systemic corticosteroid therapy for 5 years, had bilateral fibrillar opacities that surrounded the optic disks and extended into the peripapillary nerve fiber layer and vessels. A magnetic resonance imaging examination and lumbar puncture revealed elevated intracranial pressure. RESULTS: The elevated intracranial pressure returned to normal following a corticosteroid taper. optic disk photographs, taken 4 years earlier, were subsequently obtained. The optic disk appearance had remained unchanged over the 4-year period, consistent with a pseudopapilledema. CONCLUSION: The optic disk appearance is not consistent with papilledema from increased intracranial pressure. The optic disk findings, in conjunction with the underlying inflammatory syndrome, suggest an infiltrative etiology for the atypical optic nerve findings in neonatal-onset multisystem inflammatory disease.- - - - - - - - - - ranking = 1.4keywords = multisystem inflammatory disease, inflammatory disease, multisystem (Clic here for more details about this article) |
9/11. Papilloedema in Behcet's disease: value of MRI in diagnosis of dural sinus thrombosis.Behcet's disease is a multisystem disease characterised by the clinical triad of oral ulcers, genital ulcers and uveitis. Nervous system involvement is frequent and occasionally precedes other manifestations. Behcet's disease is not frequently considered in the differential diagnosis of papilloedema. We report four cases of Behcet's disease in which papilloedema occurred with or without dural sinus thrombosis. MRI is of great value in the investigation of such patients as it can demonstrate venous sinus thrombosis non-invasively or suggest the diagnosis by showing the associated parenchymal lesions secondary to small vessel pathology.- - - - - - - - - - ranking = 0.00012183850278923keywords = multisystem (Clic here for more details about this article) |
10/11. Infiltrative orbitopathy, optic disk edema, and POEMS.The polyneuropathy, organomegaly, edema, monoclonal protein, and skin changes (POEMS) syndrome is a multisystem disorder of unknown etiology. neurologic manifestations include polyneuropathy, optic disk edema, and intracranial hypertension. We studied a patient with poems syndrome who had an infiltrative orbitopathy.- - - - - - - - - - ranking = 0.00012183850278923keywords = multisystem (Clic here for more details about this article) |
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