Macular oedema as a sign of preventable neurological disease in a 17‐year‐old man

A 17-year-old man reported pain over his left temple on left gaze. He did not have any visual disturbance, but reported an occasional loss of consciousness and a floating sensation. His medical history included measles at 8 months of age. At the first examination, his visual acuity was 1.5 in both eyes (OU). The findings in the slit-lamp examination were normal but ophthalmoscopy showed a linear arcuate lesion in the macular area of the left eye with a dot haemorrhage at the temporal margin (Fig. 1A,B). The retina around the lesion was oedematous, and the oedema extended to a prominent arteriovenous crossing at the superotemporal arcade (Fig. 1). The superotemporal artery was narrow, and the veins of the superior and inferior temporal areas were dilated (Fig. 1A,B). Fundus photograph and optical coherence tomography (OCT) image of a patient with subacute sclerosing panencephalitis. (A) Fundus photographs: (a) right eye, showing a normal fundus; (b) left eye, showing a linear lesion (black arrow) and oedema (between black arrowhead) surrounding the macula with a haemorrhage at the temporal end. The dilated veins (white arrows) and the sheathed artery (grey arrow) can be seen. (B) OCT image showing a cross-section of the affected retina. The radial line and labels beside the OCT images indicate the direction of the cross-section corresponding to each image. The vesicles shown in the nerve fiber layer (b and e; grey arrow) are believed to be dilated vessels. The main lesion (white arrow with black rim) extends from the outer plexiform layer to the pigment epithelial layer but the choroid is intact. Haemorrhages are seen as acoustic shadows. The isointensity structure (c; grey arrow) is in the nerve fibre layer. (C) Retinal thickness map determined by OCT. The oedema extends close to the optic disc along the scar. Optical coherence tomography (OCT3; Zeiss, Dublin, California, USA) showed a localized cavity that extended from the outer plexiform layer to the retinal pigment epithelial layer (RPE) but the defect did not extend into the choroid (Fig. 1C, a–f). There was a small hole in the nerve fibre layer (Fig. 1B, b,d) that appeared to be the cross-section of a dilated vessel. OCT Ophthalmoscopic® (Ophthalmic Technologies Inc., Toronto, Ontario, Canada; Nidek Co. Ltd, Nagoya, Japan) scans parallel to the retinal surface showed a linear dark region that corresponded to the linear legion, and a hyper-intensity structure near the optic disc (Fig. 2A, d). (A) OD, optic disc; STA, supero-temporal artery; STV, supero-temporal vein: (a) scanning laser ophthalmoscope (SLO) image; (b–d) optical coherence tomography (OCT) Ophthalmoscopic® image parallel to the retinal surface; (e) colour photograph corresponding to the OCT image. All of these images were recorded 1 week after the first visit. All the images are the corresponding lesions to those in the OCT Ophthalmoscopic® images. (a–d) These images show a linear dark region (black and grey arrow) that corresponds with the fundus findings (black arrows). The nerve fibre layer defect (white arrow) is shown confluent to the linear lesion. Hyperintensity structure and possible obstructed artery are indicated by the small black arrow in (d) and (e), respectively. (B) Colour fundus photograph of the left eye: (a) 1 week later; (b) 2 months later; (c) 6 months later. The linear lesion remained after 6 months but the oedema had disappeared. (C) OCT image taken 2 months after the first visit. This is the cross-section of a 60-degree line, corresponding to label (c) in Fig 1B. The cavity (grey arrow) became larger and we could see the vessel inside the cavity. (D) The scanning laser ophthalmoscopic image of the linear lesion. There are dark bands, suggesting the retinal defect with a suspended vessel + fixing point. (a) Argon (488 nm, 20 degree2), (b) helium–neon (He–Ne 633 nm, 20 degree2), (c) diode (790 nm, 20 degree2, with ring aperture), (d) diode (790 nm, confocal) laser were used to obtain the image. Images (a) and (b) revealed that the tissue defect extended in the retinal surface and inner layer to the pigment epitherial layer. (c) Choroid image, which eliminated the involvement of the choroid. (d) Retinal outer layer and superficial layer are observed simultaneously. Beside the dark band, nerve fibre layer defects to the optic disc were detected. Full blood counts and differential white cell count were within normal limits. Screening for the 12 most frequent parasites in Japan by multiple dot enzyme-linked immunosorbent assay (ELISA) was negative, thus eliminating a migrating larva as the cause of the lesion. Angioid streaks and traumatic choroidal rupture were eliminated as the cause of the lesion because OCT showed that the changes were confined to the retina. The retinal oedema disappeared in 1 week but the arcuate lesion remained (Fig. 2B, a). A cross-sectional image of the linear lesion showed a cavity that extended from the nerve fibre layer to the RPE (Fig. 2C). Scanning laser ophthalmoscopy (SLO) recorded 1 week later showed the retinal defect around the vessels (Fig. 2D). Six months later, the patient’s family noticed abnormal involuntary movements and an intellectual deterioration of the patient. He soon developed severe convulsions and required respiratory assistance. An examination of the cerebrospinal fluid showed an elevation of anti-measles antibodies (IgG 12.8 mg/dl). Taking these findings together, a diagnosis of subacute sclerosing panencephalitis (SSPE) was made. At the final examination a year later, the patient’s fundus showed the linear scar but the venous congestion was not seen. The ocular manifestations of SSPE are: chorioretinitis with pigment clumping (De Laey et al. 1983; Zagami & Lethlean 1991), retinal vasculitis (Salmon et al. 1991) and serous macular detachment (De Laey et al. 1983). Retinal striae with oedema and pigment clumping (Zagami & Lethlean 1991) has also been reported. De Laey reported the results of a histopathological study of an eye belonging to a patient with SSPE (De Laey et al. 1983). The external plexiform layer was slightly oedematous, the RPE layer was thin with proliferation, and optic nerve showed hypercellularity in its supreficial temporal portion without any obvious ophthalmoscopic abnormalities. The linear lesion was detected as a rupture of the internal limiting membrane. In our patient, the alterations extended from the nerve fibre layer to the RPE layer. The external plexiform layer was thicker, and the RPE was partially obscured (Fig. 1B-f); it was too indistinct to say that it was depigmentation. The optic discs appeared normal but ocular pain with eye movements may be a sign of an occult inflammation of the optic disc. Zako et al.(2008) reported a marked decrease in foveal thickness in the OCT findings in a patient with SSPE. In our patient, the lesion was focal and the retinal thickness at the last examination was not decreased. The presence of focal retinitis and its acute changes may raise a suspicion of SSPE. This could facilitate early diagnosis and thus prevent the deterioration of the condition by treating this fatal disease at an early stage. This was demonstrated by Serdaroglu et al. (2005), who reported on a patient who they treated successfully thanks to an early diagnosis.

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