Bilateral macular retinitis as the presenting feature of subacute sclerosing panencephalitis.
ABSTRACT Two patients presented with retinitis as the initial clinical manifestation of subacute sclerosing panencephalitis (SSPE), a delayed neurologic complication of measles. In one patient, the ocular involvement preceded the neurologic symptoms by 4 weeks and in the other patient by 4 years. The diagnosis of SSPE was suspected when neuropsychiatric manifestations appeared and was confirmed by the typical panencephalitic electroencephalography changes, neuroimaging features of panencephalitis, and high titers of measles antibodies in serum and cerebrospinal fluid. Although SSPE is an untreatable illness, recognition of this unusual presentation is valuable to allow earlier diagnosis and institution of palliative measures.
- Canadian Journal of Ophthalmology 04/2012; 47(2):e1-2. · 1.30 Impact Factor
Article: Challenges in diagnosing SSPE.[Show abstract] [Hide abstract]
ABSTRACT: The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties. We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2 years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6 months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study. Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria. We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization.Child s Nervous System 12/2011; 27(12):2041-4. · 1.24 Impact Factor
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ABSTRACT: To review the literature on early visual manifestations of subacute sclerosing panencephalitis (SSPE) with regard to two patients who had visual problems preceding the onset of neurological symptoms. One patient had cortical visual disturbances and the other had visual loss due to retinal pigment epithelial changes. SSPE is a chronic encephalitis characterized by a history of measles infection and a progressive disease of the central nervous system that still occurs frequently in countries with insufficient measles immunization. Visual manifestations can occur as a result of involvement of the pathways that lead from the retina to the occipital cortex during the course of the disease, but are rare as a presenting sign. Fundus changes, especially macular retinitis and macular pigment disturbances, appear to be the most common ocular manifestations of SSPE. Ophthalmologists must be aware that SSPE can knock their door with ocular findings of SSPE, months or years before the onset of neurological symptoms.Current opinion in ophthalmology 11/2012; 23(6):466-71. · 2.64 Impact Factor