UNIQUE OPTICAL COHERENCE TOMOGRAPHY FINDINGS IN A CASE OF MACULAR RETINITIS CAUSED BY SUBACUTE SCLEROSING PANENCEPHALITIS Masayuki Hata, MD,* Akio Oishi, MD, PhD,* Yasuo Kurimoto, MD, PhD*†
Background: Subacute sclerosing panencephalitis is a rare neurodegenerative disease. The ophthalmologic findings, typically necrotizing retinitis, often precede other neurologic signs and symptoms. Ocular findings are helpful for early diagnosis, but the findings of optical coherence tomography (OCT) images in an active phase of retinitis have never been reported. We report unique findings of OCT images in a case of subacute sclerosing panencephalitis. Patient and Methods: A 13-year-old boy was referred to our hospital complaining of visual loss and other neurologic symptoms. Results: The fundus examination showed necrotizing retinitis. The OCT images showed empty spaces involving all layers of retina except the inner limiting membrane and hyperreflective lesion in inner retina. The diagnosis of subacute sclerosing panencephalitis was made from the blood and the spinal cerebral fluid test. Discussion: This is the first report on OCT images of necrotizing retinitis in an active stage of subacute sclerosing panencephalitis case. The unique findings of OCT images as in this case help to diagnose the rare devastating disease. RETINAL CASES & BRIEF REPORTS 6:435–437, 2012
article, we report unique OCT findings in an active case of SSPE.
From the *Department of Ophthalmology, Kobe City Medical Center General Hospital, Kobe, Japan; and †Department of Ophthalmology, Institute of Biomedical Research and Innovation Hospital, Japan.
Case Report
S
ubacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disorder caused by the measles virus. Ocular manifestations, typically necrotizing retinitis, are present in up to half of cases and often precede the other neurologic signs.1 In a previous report of a chronic case of SSPE, optical coherence tomography (OCT) images showed thinning of the retina.2 However, there is no report of OCT findings in the active stage of the disease. In this
A 13-year-old boy was referred to our hospital for evaluation of a progressive encephalopathy. In the previous month, he had experienced myoclonus of the limbs, convulsion, mental regression, and lack of interests in the environment. He had consulted another ophthalmologist complaining of bilateral sudden visual loss 1 month before and retinal hemorrhages were pointed out. At initial presentation, his best-corrected visual acuity was 0.4 in the right eye and 0.004 in the left eye. Fundus examination showed almost normal macula, peripheral retina, disk, and vessels in the right eye (Figure 1). However, the left eye presented retinal nerve fiber layer defect and small, red, lesion-like aneurysm in the macula (Figure 2A). Furthermore, OCT images revealed mild hyperreflective areas occupying mainly the inner retina (Figures 1C and 2, B–E). Of note, there were empty spaces involving all layers of retina, except the inner limiting membrane and plausible outer segment (Figure 2E). High titers of measles antibodies were found in the cerebral spinal fluid and in the serum (more than 1:50 in the cerebral spinal
There is no sponsoring agent or conflict of interest. Reprint requests: Masayuki Hata, MD, Department of Ophthalmology, Kobe City Medical Center General Hospital, 4-6 Minatojima Nakamachi, chuo-ku, Kobe 650-0046, Japan; e-mail: masayuki_ [email protected]
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RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 4
Fig. 1. A, A fundus photograph of the right eye appeared to be almost normal. B, A horizontal scan of the right eye also appeared to be normal. C, A vertical scan of the left eye illustrating mild hyperreflective area occupying mainly inner retina and defect of outer retina (blue arrow; black arrows, optical coherence tomography orientation).
fluid and 1:128 in the serum). On the basis of these findings, the diagnosis of SSPE was made.
Discussion The most common ophthalmologic manifestation of SSPE is a focal macular retinitis.1,3,4 Histopathologically, the inner nuclear layer and the ganglion cell layer are generally more severely affected than other retinal layers in the acute stage.5 The previous histopathologic case report also indicated an arcuate pattern of retinitis.6 Consistently, our patient showed retinal nerve fiber layer defect. There is limited information of OCT in a previous report; marked retinal thinning was observed in a chronic case.2 In our case with active SSPE, OCT images showed unique findings. There were high reflectance lesions, considered to be necrosis, in the inner retina of parafovea and the defect of full retina except the inner limiting membrane overlying the lesion. In view of OCT findings, interestingly, progressive outer retinal necrosis (PORN) sometimes shows
similar findings. Progressive outer retinal necrosis is also a necrotizing retinopathy caused by herpes viruses and characterized by early involvement of the outer retina.7 Although PORN clinically starts as an outer retinal process, previous OCT findings suggest that inner retina is also involved.8,9 Indeed, Narayanan and Kuppermann9 reported PORN OCT findings displaying mild hyperreflective lesions in the inner retina with hyporeflective lesions in the outer retina, which are quite similar to those seen in our case. Considering similarities, SSPE and PORN may share some pathologic process in retinal necrosis. It is sometimes difficult to diagnose SSPE especially when it lacks neurologic sign. The characteristic OCT findings, as seen in the present case, would be a useful help for early diagnosis because ocular manifestations generally precede the neurologic signs. In addition, the findings suggest that SSPE and PORN may have a common pathologic process. Key words: necrotizing retinitis, optical coherence tomography, progressive outer retinal necrosis, subacute sclerosing panencephalitis.
Fig. 2. A, A fundus photograph of the left eye illustrating nerve fiber bundle defect and red spots that resemble aneurysm in the macula. B, A horizontal scan of the left eye illustrating hyperreflective area occupying mainly the inner retina and sub- or intraretinal lesion (arrowhead, [D] magnified image). C, A vertical scan of the left eye illustrating mild hyperreflective area occupying the inner retina and focal empty spaces involving all layers of the retina except the inner limiting membrane (blue arrow, E, magnified image).
UNIQUE OCT FINDINGS IN SSPE
References 1. Hiatt RL, Grizzard HT, McNeer P, Jabbour JT. Ophthalmologic manifestations of subacute sclerosing panencephalitis (Dawson’s encephalitis). Trans Am Acad Ophthalmol Otolaryngol 1971; 75:344–350. 2. Zako M, Kataoka T, Ohno-Jinno A, et al. Analysis of progressive ophthalmic lesion in a patient with subacute sclerosing panencephalitis. Eur J Ophthalmol 2008;18: 155–158. 3. Nguyen NQ, Lee AG, McClure CD, Miller G. Subretinal lesions in subacute sclerosing panencephalitis. J AAPOS 1999;3: 252–254. 4. Green SH, Wirtschafter JD. Ophthalmoscopic findings in subacute sclerosing panencephalitis. Br J Ophthalmol 1973;57: 780–787.
437 5. De Laey JJ, Hanssens M, Colette P, et al. Subacute sclerosing panencephalitis: fundus changes and histopathologic correlations. Doc Ophthalmol 1983;56:11–21. 6. Park DW, Boldt HC, Massicotte SJ, et al. Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings. Am J Ophthalmol 1997;123:533–542. 7. Kuppermann BD, Quiceno JI, Wiley C, et al. Clinical and histopathologic study of varicella zoster virus retinitis in patients with the acquired immunodeficiency syndrome. Am J Ophthalmol 1994;118:589–600. 8. Almony A, Dhalla MS, Feiner L, Shah GK. Macular optical coherence tomography findings in progressive outer retinal necrosis. Can J Ophthalmol 2007;42:881. 9. Narayanan R, Kuppermann BD. Optical coherence tomography in progressive outer retinal necrosis. Ophthalmic Surg Lasers Imaging 2006;37:506–507.
Background: Subacute sclerosing panencephalitis is a rare neurodegenerative disease. The ophthalmologic findings, typically necrotizing retinitis, often precede other neurologic signs and symptoms. Ocular findings are helpful for early diagnosis, but the findings of optical coherence tomography (OCT) images in an active phase of retinitis have never been reported. We report unique findings of OCT images in a case of subacute sclerosing panencephalitis. Patient and Methods: A 13-year-old boy was referred to our hospital complaining of visual loss and other neurologic symptoms. Results: The fundus examination showed necrotizing retinitis. The OCT images showed empty spaces involving all layers of retina except the inner limiting membrane and hyperreflective lesion in inner retina. The diagnosis of subacute sclerosing panencephalitis was made from the blood and the spinal cerebral fluid test. Discussion: This is the first report on OCT images of necrotizing retinitis in an active stage of subacute sclerosing panencephalitis case. The unique findings of OCT images as in this case help to diagnose the rare devastating disease. RETINAL CASES & BRIEF REPORTS 6:435–437, 2012
article, we report unique OCT findings in an active case of SSPE.
From the *Department of Ophthalmology, Kobe City Medical Center General Hospital, Kobe, Japan; and †Department of Ophthalmology, Institute of Biomedical Research and Innovation Hospital, Japan.
Case Report
S
ubacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disorder caused by the measles virus. Ocular manifestations, typically necrotizing retinitis, are present in up to half of cases and often precede the other neurologic signs.1 In a previous report of a chronic case of SSPE, optical coherence tomography (OCT) images showed thinning of the retina.2 However, there is no report of OCT findings in the active stage of the disease. In this
A 13-year-old boy was referred to our hospital for evaluation of a progressive encephalopathy. In the previous month, he had experienced myoclonus of the limbs, convulsion, mental regression, and lack of interests in the environment. He had consulted another ophthalmologist complaining of bilateral sudden visual loss 1 month before and retinal hemorrhages were pointed out. At initial presentation, his best-corrected visual acuity was 0.4 in the right eye and 0.004 in the left eye. Fundus examination showed almost normal macula, peripheral retina, disk, and vessels in the right eye (Figure 1). However, the left eye presented retinal nerve fiber layer defect and small, red, lesion-like aneurysm in the macula (Figure 2A). Furthermore, OCT images revealed mild hyperreflective areas occupying mainly the inner retina (Figures 1C and 2, B–E). Of note, there were empty spaces involving all layers of retina, except the inner limiting membrane and plausible outer segment (Figure 2E). High titers of measles antibodies were found in the cerebral spinal fluid and in the serum (more than 1:50 in the cerebral spinal
There is no sponsoring agent or conflict of interest. Reprint requests: Masayuki Hata, MD, Department of Ophthalmology, Kobe City Medical Center General Hospital, 4-6 Minatojima Nakamachi, chuo-ku, Kobe 650-0046, Japan; e-mail: masayuki_ [email protected]
435
436
RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 4
Fig. 1. A, A fundus photograph of the right eye appeared to be almost normal. B, A horizontal scan of the right eye also appeared to be normal. C, A vertical scan of the left eye illustrating mild hyperreflective area occupying mainly inner retina and defect of outer retina (blue arrow; black arrows, optical coherence tomography orientation).
fluid and 1:128 in the serum). On the basis of these findings, the diagnosis of SSPE was made.
Discussion The most common ophthalmologic manifestation of SSPE is a focal macular retinitis.1,3,4 Histopathologically, the inner nuclear layer and the ganglion cell layer are generally more severely affected than other retinal layers in the acute stage.5 The previous histopathologic case report also indicated an arcuate pattern of retinitis.6 Consistently, our patient showed retinal nerve fiber layer defect. There is limited information of OCT in a previous report; marked retinal thinning was observed in a chronic case.2 In our case with active SSPE, OCT images showed unique findings. There were high reflectance lesions, considered to be necrosis, in the inner retina of parafovea and the defect of full retina except the inner limiting membrane overlying the lesion. In view of OCT findings, interestingly, progressive outer retinal necrosis (PORN) sometimes shows
similar findings. Progressive outer retinal necrosis is also a necrotizing retinopathy caused by herpes viruses and characterized by early involvement of the outer retina.7 Although PORN clinically starts as an outer retinal process, previous OCT findings suggest that inner retina is also involved.8,9 Indeed, Narayanan and Kuppermann9 reported PORN OCT findings displaying mild hyperreflective lesions in the inner retina with hyporeflective lesions in the outer retina, which are quite similar to those seen in our case. Considering similarities, SSPE and PORN may share some pathologic process in retinal necrosis. It is sometimes difficult to diagnose SSPE especially when it lacks neurologic sign. The characteristic OCT findings, as seen in the present case, would be a useful help for early diagnosis because ocular manifestations generally precede the neurologic signs. In addition, the findings suggest that SSPE and PORN may have a common pathologic process. Key words: necrotizing retinitis, optical coherence tomography, progressive outer retinal necrosis, subacute sclerosing panencephalitis.
Fig. 2. A, A fundus photograph of the left eye illustrating nerve fiber bundle defect and red spots that resemble aneurysm in the macula. B, A horizontal scan of the left eye illustrating hyperreflective area occupying mainly the inner retina and sub- or intraretinal lesion (arrowhead, [D] magnified image). C, A vertical scan of the left eye illustrating mild hyperreflective area occupying the inner retina and focal empty spaces involving all layers of the retina except the inner limiting membrane (blue arrow, E, magnified image).
UNIQUE OCT FINDINGS IN SSPE
References 1. Hiatt RL, Grizzard HT, McNeer P, Jabbour JT. Ophthalmologic manifestations of subacute sclerosing panencephalitis (Dawson’s encephalitis). Trans Am Acad Ophthalmol Otolaryngol 1971; 75:344–350. 2. Zako M, Kataoka T, Ohno-Jinno A, et al. Analysis of progressive ophthalmic lesion in a patient with subacute sclerosing panencephalitis. Eur J Ophthalmol 2008;18: 155–158. 3. Nguyen NQ, Lee AG, McClure CD, Miller G. Subretinal lesions in subacute sclerosing panencephalitis. J AAPOS 1999;3: 252–254. 4. Green SH, Wirtschafter JD. Ophthalmoscopic findings in subacute sclerosing panencephalitis. Br J Ophthalmol 1973;57: 780–787.
437 5. De Laey JJ, Hanssens M, Colette P, et al. Subacute sclerosing panencephalitis: fundus changes and histopathologic correlations. Doc Ophthalmol 1983;56:11–21. 6. Park DW, Boldt HC, Massicotte SJ, et al. Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings. Am J Ophthalmol 1997;123:533–542. 7. Kuppermann BD, Quiceno JI, Wiley C, et al. Clinical and histopathologic study of varicella zoster virus retinitis in patients with the acquired immunodeficiency syndrome. Am J Ophthalmol 1994;118:589–600. 8. Almony A, Dhalla MS, Feiner L, Shah GK. Macular optical coherence tomography findings in progressive outer retinal necrosis. Can J Ophthalmol 2007;42:881. 9. Narayanan R, Kuppermann BD. Optical coherence tomography in progressive outer retinal necrosis. Ophthalmic Surg Lasers Imaging 2006;37:506–507.
