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Show ORIGINAL CONTRIBUTION Bilateral Macular Retinitis as the Presenting Feature of Subacute Sclerosing Panencephalitis Rajesh B. Babu, MS and Jyotirmay Biswas, MS, FNAMS 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 neuropsy-chiatric 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 unbeatable illness, recognition of this unusual presentation is valuable to allow earlier diagnosis and institution of palliative measures. (/ Neuro- Ophthalmol 2007; 27: 288- 291) Subacute sclerosing panencephalitis ( SSPE) is a progressive inflammatory disease of the central nervous system caused by a persistent aberrant measles virus infection. The annual incidence rate varies from 1 to 4 per million in developed countries ( 1). However, in developing countries the incidence is higher because a larger proportion of the total population is younger than 2 years of age ( 2). In India, the annual incidence rate is reported to be as high as 21 per million ( 1,2). Ocular manifestations of SSPE include optic neuritis ( 3), viral retinitis ( 4), chorioretinitis ( 5- 8), and cortical blindness ( 9,10). We report two patients with SSPE who initially presented with visual loss. CASE REPORTS Case 1 A 20- year- old man presented with sudden painless loss of vision in both eyes of 20 days' duration. The vision Department of Uveitis, Medical and Vision Research Foundations, Sankara Nethralaya, Chennai, India. Address correspondence to Dr. Jyotirmay Biswas, MS, FNAMS, Director, Uveitis and Ocular Pathology, Medical and Vision Research Foundations, Sankara Nethralaya, 18 College Road, Chennai 600 006, India; E- mail: drjb@ snmail. org in the right eye had worsened rapidly over the previous 2 weeks. His general health was normal. He had had measles at the age of 9 months. He appeared well nourished and was cooperative and alert. He was afebrile. General physical examination was completely normal. Best- corrected visual acuities were 20/ 400 in right eye and 20/ 30 in left eye. Ophthalmoscopy ( Fig. 1) showed bilateral macular gray- white retinal lesions with geographic borders and yellow exudates at their edges and bilateral temporal pallor of the optic discs. There was no vitreous inflammation. There were no other ophthalmic abnormalities and the neurologic examination, including mental status, was normal. Fourteen days later, he developed insomnia and psychotic behavior. He was seen by a psychiatrist and was given antipsychotic medications. He subsequently developed tremor of the upper extremities and paresis of the left lower extremity. The white blood cell count was 7,100/ mm3, hemoglobin was 14 g/ dL, and erythrocyte sedimentation rate ( ESR) was 22 mm/ h. Results of anti- streptolysin titers and smears for malarial parasites were negative. Blood glucose, electrolytes, liver and renal panels, thyroid- stimulating hormone, anti- double- stranded DNA antibody, anti- nuclear antibody, blood and urine culture, chest X- ray, echocardiography, and abdominal ultrasound were normal. Cerebrospinal fluid showed 5 cells/ mm3 ( all lymphocytes), 32 mg/ mL protein, and 61 mg/ dL glucose and negative results for Gram and acid- fast stains and polymerase chain reaction ( PCR) for herpes simplex virus ( HSV) and Mycobacterium tuberculosis. Results of arbovirus serology were negative. Electroencephalography ( EEG) showed a disorganized pattern with low- amplitude random dysrhythmic slowing and predominant delta and theta waves. Alpha waves of 9- 10 Hz were intermixed with low- voltage slow waves. Periodic high- voltage slow waves in the delta range were also seen with a right- sided focus and no abnormal sharp waves or spikes. These findings were interpreted as showing a generalized encephalopathy. Brain MRI showed high T2 signal abnormalities in the basal ganglia ( not shown). Serum anti- measles IgG antibody was elevated at 16.59 enzyme immunoassay ( EIA) units ( normal < 10) and 288 J Neuro- Ophthalmol, Vol. 27, No. 4, 2007 Subacute Sclerosing Panencephalitis J Neuro- Ophthalmol, Vol. 27, No. 4, 2007 FIG. 1. Case 1. Optic disc pallor and grayish macular lesions with geographic borders are evident in both eyes. There is no vitreous inflammation. IgM ( 1.02 EI A units) ( normal < 1.0) was normal. CSF anti-measles IgG antibody was 8.5 EIA units ( normal < 1.0) and IgM was normal. A diagnosis of SSPE was made. The patient was treated with sedatives and antiepileptic drugs but became quadriplegic and aphasic and died within 3 months. Case 2 A 25- year- old man presented with decreased vision in both eyes over 20 days. He had been examined 3 years earlier at our hospital with similar complaints and bilateral viral retinitis had been diagnosed. On that occasion, his best- corrected visual acuity was 20/ 60 in the right eye and 20/ 400 in the left eye. He reported no decline in vision from 4 years ago until 20 days earlier. He had had measles infection at age 7. Otherwise he was healthy. He was afebrile, and general and neurologic examinations were normal. Best- corrected visual acuity was 20/ 200 in the right eye and 20/ 800 in the left eye. Pupillary reactions were sluggish in both eyes. Anterior segment examination and the vitreous were normal. Fundus examination showed bilateral macular retinal pigment epithelial atrophy ( Fig. 2). Within 10 days, the patient developed irritability and reduced attention. A few days later, he became increasingly withdrawn and emotionally labile. Later he developed intermittent, random, low-amplitude, lightning- like jerking movements of the extremities. Blood count and ESR were normal, and blood herpes simplex viral titers were elevated. Serum anti- measles IgG antibody was elevated at 18.20 EIA units ( normal < 10) and IgM was negative ( normal < 1). CSF anti- measles IgG antibody was 22.5 EIA units ( normal < 10) and IgM was normal. EEG showed an encephalitic pattern ( Fig. 3). T2 brain MRI showed a hyperintense signal in the gray matter with loss of gray- white definition and obliteration of sulci of the parieto- occipital lobe ( Fig. 4). He was initially treated for a diagnosis of herpes encephalitis with 1,500 mg/ day intravenous acyclovir in divided doses for 1 week and transferred to a tertiary neurocenter, where the repeat CSF anti- measles antibody titer was found to be 1: 625 and anti HSV IgG antibody was 1: 25 ( HSV- 1 IgG levels: negative 0.90, equivocal 0.91- 0.99, and positive > 1.0). SSPE with atypical features was diagnosed, and the patient was treated with antiseizure medications, inosiplex, and interferons. He was followed for 6 months with no clinical improvement and was lost to follow up. FIG. 2. Case 2. Bilateral macular retinal pigment epithelial scars are evident. 289 J Neuro- Ophthalmol, Vol. 27, No. 4, 2007 Babu and Biswas FIG. 3. Case 2. Electroencephalography shows a disorganized pattern with low- amplitude random dysrhythmic slowing and a periodic high- voltage slow wave in the delta range ( periodic complexes) consistent with encephalitis. DISCUSSION The diagnosis of SSPE is based on clinical features, characteristic EEG changes, elevated CSF globulin levels (> 20% of total protein), raised measles antibody titers in FIG. 4. Case 2. T2 brain MRI shows hyperintense signal in the gray matter with loss of gray- white definition and obliteration of sulci of the parieto- occipital lobe consistent with panencephalitis. blood and CSF, and typical histopathologic findings on brain autopsy. The clinical features consist of personality and behavioral changes and worsening school performance, followed by myoclonic seizures, paresis, dyspraxias, memory impairment, language difficulties, blindness, and eventually obtundation, stupor, and coma ( 10). Most patients give a history of having acquired measles before 2 years of age. The latent period between measles infection and SSPE is 6- 8 years in most cases but may range from 3 months to 18 years. Once SSPE is identified, clinical progression is variable. Death usually occurs within 1- 3 years after the onset of symptoms. Approximately 10% of patients have a fulminant course with death occurring within a few months after onset. Another 10% of patients survive for 4- 10 years ( 1). Atypical presentations include isolated psychiatric manifestations, poorly controlled seizures, or isolated extrapyramidal syndromes such as chorea or hemiparkinsonism ( 11,12). A stroke- like onset has also been described ( 11). A multitude of neuro- ophthalmic and retinal findings are associated with SSPE, and ocular involvement appears to occur in at least 50% of cases ( 3- 9). The most characteristic lesion is a necrotizing retinitis ( 13). It usually manifests as a focal area of retinitis in the macula. Occasionally described as a chorioretinitis, it appears to preferentially affect the retina with secondary involvement of the retinal pigment epithelium and choroid. Most commonly, it appears as a ground- glass whitening of the retina with ill- defined margins and a mottling of the underlying pigment epithelium. Sometimes there may be a red- orange color change in the macular area ( 13). This process may progress to involve most of the posterior pole and peripheral retina. There may be associated retinal changes such as edema, hemorrhage, detachments, venous dilatation, vascular occlusions, and retinal pigment epithelial detachments. There is little or no vitreous inflammation or involvement of retinal vessels or satellite lesions. These features help in distinguishing SSPE from other inflammatory retinal disorders such as toxoplasmosis and Behcet disease. In the cases reported by Takayama et al ( 14), blurred vision was the initial symptom in more than half ( 15,16). Basal ganglion involvement occurs on MRI in 20%- 35% cases, as in our Case 1 ( 15,16). 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