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Year : 1999  |  Volume : 47  |  Issue : 4  |  Page : 250-252

Subacute sclerosing panencephalitis presenting as optic neuritis

Centre for Ophthalmic Sciences, All Institute of Medical Sciences, New Delhi, India

Correspondence Address:
R Tandon
Centre for Ophthalmic Sciences, All Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

PMID: 10892484

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How to cite this article:
Tandon R, Khanna S, Sharma M C, Seshadri S, Menon V. Subacute sclerosing panencephalitis presenting as optic neuritis. Indian J Ophthalmol 1999;47:250-2

How to cite this URL:
Tandon R, Khanna S, Sharma M C, Seshadri S, Menon V. Subacute sclerosing panencephalitis presenting as optic neuritis. Indian J Ophthalmol [serial online] 1999 [cited 2023 Dec 10];47:250-2. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1999/47/4/250/14901

Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disease affecting both grey and white matter of the brain in children and young adults. One such case which involved the visual system is described here.

SSPE usually begins with personality or behavioural changes, followed over a variable period by dementia, seizures, myoclonus, quadriparesis and eventually death. There is a high incidence of visual system abnormalities during the course of the illness, often appearing before the central nervous system manifestations. Intranuclear inclusions of the measles virus have been found in retinal cells and in the optic nerve of patients with SSPE.[1]

We report herein the case of a young girl who initially presented with bilateral papillitis and subsequently developed macular retinitis. These ophthalmic manifestations were followed over a few days by a rapid downhill course of neurologic complications. The diagnosis of SSPE was suspected clinically only after the neurologic manifestations developed and were confirmed by a brain biospy. During a retrospective review of the literature we realized that our patient's initial presentation was accompanied by typical fundus changes described clearly in SSPE in western literature. Though SSPE is a disease entity which has been well reported from India there has been no mention of the ocular complications in these patients.[2],[3]

This report illustrates and reviews the typical ocular findings of SSPE not previously reported in Indian literature and emphasizes that since ocular complications may precede clinical neurologic involvement, SSPE should be considered in the differential diagnosis of optic atrophy, papillitis, and retinitis of unknown aetiology in children.

  Case Report Top

An 18-year-old girl presented to our hospital with sudden blurring of vision in her right eye for 15 days. This was associated with mild pain on ocular movements in the right eye. She had no complaints in her left eye and no systemic disorder. Her past medical history was initially noncontributory. Retrospectively, she was found to have demonstrated poor scholastic performance for the past 3 years. She had had measles at the age of 18 months.

On ophthalmic examination, her best corrected visual acuity in right eye was 1/60 and left eye was 6/12. Colour vision testing using Ishihara test plates revealed red-green blindness both eyes. The anterior segment was normal in both eyes except for a right afferent pupillary defect. Fundus examination revealed clear media bilaterally. Right fundus showed mild disc pallor with full cup and blurred margins suggestive of papillitis developing secondary optic atrophy [Figure - 1]. The left eye showed a hyperemic disc with blurred margins and slightly engorged veins suggestive of papillitis.

We diagnosed bilateral papillitis (right developing secondary optic atrophy, left early papillitis) and started the patient on intravenous pulsed steroids. After 3 days of 100mg dexamethasone intravenously daily her vision improved to 6/60 in the right eye and remained unchanged in the left eye. Two days later her vision in the left eye dropped to 6/60. A repeat examination revealed retrolental cells in her left eye with a whitish ground-glass-like, ill-defined lesion in the macula [Figure - 2]. The right macula was also discoloured, ith dull foveal reflex.

At this stage, Toxoplasmosis was considered as an aetiologic possibility and the patient was put on Co-trimoxazole (160 mg Trimethoprin with 800 mg sulphamethoxazole) orally twice daily and Prednisolone 60mg once daily. Indirect hemaglutination test (IHA) for Toxoplasma and Venereal disease research laboratory (VDRL) test for syphilis was found to be nonreactive. Fundus fluorescein angiography was suggestive of left neuroretinitis and right optic atrophy.

Her vision in the left eye continued to deteriorate to finger counting at 1 metre. Vision in the right eye remained stable at 6/60. Over the next few days she started developing progressive unsteadiness of gait, weakness of both lower limbs followed by weakness of trunk and then both upper limbs. She went on to develop loss of bladder control, mutism and myoclonic jerks. She was transferred to the neurology ward and 4 days later she developed altered sensorium. MRI of the head and spinal cord was normal. A tentative clinical diagnosis of SSPE was made. However, antimeasles antibody titres in blood and cerebrospinal fluid were negative. A right frontal brain biopsy was performed. Microscopic examination of haematoxylin and eosin ( H & E) - stained slides showed intranuclear inclusions in the astrocytes and oligodendroglial cells [Figure - 3]. SSPE was confirmed on immunofluorescent staining for measles virus antigen on the brain biopsy. The patient's systemic and ocular status remained unchanged during her stay in hospital. Neurologists continued steroids and supportive care. The patient showed no improvement in her general condition and was finally taken by her relatives to be nursed at home.

  Discussion Top

SSPE is a true panencephalitis involving both the white and gray matter of the brain. Ocular involvement is a part of the widespread panencephalitis rather than a focal point of this illness.[1] Our patient presented with ocular problems and then developed neurologic complications. Ophthalmic involvement may antedate the neurologic manifestation of SSPE by a few weeks, months, or even years.[4-7]

The ophthalmologic findings of SSPE include cortical blindness, disc oedema (papilledema and papillitis), optic atrophy or temporal pallor, focal retinitis, and nystagmus.[4],[5],[8] Disc oedema had been reported in 20% of SSPE patients studied.[4] However, the most typical of all fundus lesions is the retinal involvement which usually affects the macula or perimacular region. A ground-glass whitening of the retina with ill-defined margins, and a mottling of the underlying retinal pigment epithelium has been described.[4],[5],[8] There is no marked vitreous reaction or involvement of retinal vessels or satellite lesions that might differentiate this from other inflammatory lesions such as Toxoplasmosis or Behcet's disease. Multifocal lesions have also been reported. As the disease progresses the retinitis may subside or become more severe.

The combination of papillitis with macular retinitis in our patient was typical of SSPE. However, until the patient developed neurologic symptoms, the fundus picture suggested a primary intraocular disease such as Toxoplasmosis. Retrospectively our patient had a history of deteriorating scholastic performance over the past 3 years, a factor which is known to mark the onset of SSPE.

Since SSPE is an extremely rare condition and may not have been encountered by most ophthalmologists but may present initially with ocular symptoms and typical fundus lesions we have reported this case to highlight the important features. We wish to emphasise that this disease should be considered in the differential diagnosis of children with macular retinitis, papillitis, or optic atrophy of unknown cause; a detailed history may provide a clue to the correct diagnosis.

  References Top

Wechsler Sl, Meissner HC. Subacute sclerosing panencephalitis. In: Darrell RW, editor. Viral Diseases of the Eye. Philadelphia: Lea and Febiger. 1985. p 251-61.  Back to cited text no. 1
Khare S, Kumari S, Sehgal S. Sero-epidemiology of subacute sclerosing panencephalitis in Delhi. Indian J Med Res 1990;91:94-97.  Back to cited text no. 2
Saha V, John TJ, Mukundan P, Gnanamuthu C, Prabhakar S, Arjundas G, et al. High incidence of subacute sclerosing panencephalitis in South India. Epidemiol Infect 1990;104:151-56.  Back to cited text no. 3
Green SH, Wirtzchafter JD. Ophthalmoscopic findings in subacute sclerosing panencephalitis. Br J Ophthalmol 1973;57:780-87.  Back to cited text no. 4
Gravina RF, Nakanishi AS, Faden A. Subacute sclerosing panencephalitis. Am J Ophthamol 1978;86:106-109.  Back to cited text no. 5
Johnston HM, Wise GA, Henry JG. Visual deterioration as presentation of subacute sclerosing panencephalitis. Arch Dis Child 1980;55:899-901.  Back to cited text no. 6
Jabbour JT, Garcia JH, Lemmi H, Ragland S, Duenas DA, Sever JL. Subacute sclerosing panencephalitis:A multidiscipnnary study of eight cases. JAMA 1969;207:2248-54.  Back to cited text no. 7
Robb RM, Valter GV. Ophthalmic manifestations of subacute sclerosing panencephalitis. Arch Ophthalmol 1970;83:426-35.  Back to cited text no. 8


  [Figure - 1], [Figure - 2], [Figure - 3]


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