Berker Nilufer, Batman Cosar, Guven Alev, Ozalp Solmaz, Aslan Ozlem, Zilelioglu Orhan
Department of Ophthalmology, Ankara Social Insurance Hospital, Turkey.
Am J Ophthalmol. 2004 Nov;138(5):879-81. doi: 10.1016/j.ajo.2004.05.068.
To report a case of subacute sclerosing panencephalitis in which the initial clinical presentations were optic atrophy and macular degeneration.
Observational case report.
A 9-year-old boy with visual loss attributable to optic atrophy and macular degeneration had a diagnostic examination.
The visual acuity levels of both eyes were light perception at presentation. Fundus examination revealed bilateral optic atrophy and macular degenerative changes including retinal pigment epithelial atrophy, macular scarring, and epiretinal membrane formation. The antimeasles IgG titers were high both in serum and cerebrospinal fluid samples. Neurologic symptoms of myoclonus, ataxia, motor, and mental disabilities occurred 4 months after the onset of first visual disturbances and 2 weeks after the patient applied to our clinic.
Children with optic atrophy and macular degenerative lesions should be examined for sclerosing panencephalitis, even if the classical neurologic symptoms of this disease are not present.
报告一例亚急性硬化性全脑炎病例,其最初临床表现为视神经萎缩和黄斑变性。
观察性病例报告。
对一名因视神经萎缩和黄斑变性导致视力丧失的9岁男孩进行诊断性检查。
初诊时双眼视力仅为光感。眼底检查发现双侧视神经萎缩以及黄斑退行性改变,包括视网膜色素上皮萎缩、黄斑瘢痕形成和视网膜前膜形成。血清和脑脊液样本中的抗麻疹IgG滴度均较高。在首次出现视觉障碍4个月后以及患者就诊于我院2周后,出现了肌阵挛、共济失调、运动和精神障碍等神经症状。
即使不存在该疾病的典型神经症状,对视神经萎缩和黄斑退行性病变的儿童也应进行硬化性全脑炎检查。