Sub Acute Sclerosing Sub Acute Sclerosing Panencephalitis presenting as Panencephalitis presenting as

Hemorrhagic necrotizing retinitisHemorrhagic necrotizing retinitis

Samyak MulkutkarSamyak MulkutkarMohit DograMohit DograReema BansalReema BansalVivek Lal*Vivek Lal*Amod GuptaAmod Gupta

  Advanced Eye Centre and Department of Neurology*Advanced Eye Centre and Department of Neurology*Post Graduate Institute of Medical Education and Research, ChandigarhPost Graduate Institute of Medical Education and Research, ChandigarhCorrespondence: Amod GuptaCorrespondence: Amod GuptaProfessor of Ophthalmology, Advanced Eye CentreProfessor of Ophthalmology, Advanced Eye CentrePost Graduate Institute of Medical Education and ResearchPost Graduate Institute of Medical Education and ResearchChandigarh -160012Chandigarh -160012

dramodgupta@gmail.com

Ocular HistoryOcular History

21-year-old woman21-year-old woman Presented in April 2014Presented in April 2014 H/o sudden loss of visionH/o sudden loss of vision RE: 20 days, LE:15 daysRE: 20 days, LE:15 days BCVA: RE counting fingers, LE no light perceptionBCVA: RE counting fingers, LE no light perception

Past HistoryPast History

H/o Measles infection in childhoodH/o Measles infection in childhood

Ocular examinationOcular examinationat First presentationat First presentation

LE relative afferent pupillary defectLE relative afferent pupillary defect RE: pale optic disc, perifoveal sheathing of vessels, RE: pale optic disc, perifoveal sheathing of vessels,

foveal atrophy. foveal atrophy. LE: Disc edema, dilated tortuous retinal vessels, LE: Disc edema, dilated tortuous retinal vessels,

extensive necrosis of macula, retinal and sub-retinal extensive necrosis of macula, retinal and sub-retinal hemorrhages, sub-retinal fluid. Active retinitis nasal hemorrhages, sub-retinal fluid. Active retinitis nasal to the optic disc.to the optic disc.

Fig.1 Right eye, Fundus photograph Fig.1 Right eye, Fundus photograph showing pale optic disc, featureless showing pale optic disc, featureless macula and atrophic foveamacula and atrophic fovea

Fig.2 Left eye, Fundus photograph showing disc Fig.2 Left eye, Fundus photograph showing disc edema with dilated tortuous blood vessels and edema with dilated tortuous blood vessels and hemorrhagic macular necrosis and exudative fluidhemorrhagic macular necrosis and exudative fluid

Fig.3 Left eye, Fundus photograph Fig.3 Left eye, Fundus photograph showing active retinitis edge nasal to the showing active retinitis edge nasal to the optic discoptic disc

Fig.1Fig.1

Fig.3Fig.3Fig.2Fig.2

OCTOCT RE: cystic changes and RE: cystic changes and

diffuse foveal atrophydiffuse foveal atrophy LE: Disintegration of all LE: Disintegration of all

retinal layers except ILM and retinal layers except ILM and Bruch’s membrane suggestive Bruch’s membrane suggestive of extensive retinal necrosis, of extensive retinal necrosis, and sub-retinal exudation in and sub-retinal exudation in the macula the macula

Neurological examinationNeurological examination No significant clinical abnormality.No significant clinical abnormality. MRI Brain: MRI Brain:

Well defined ovoid area of T2 and FLAIR hyper-Well defined ovoid area of T2 and FLAIR hyper-intensity in sub cortical area, intensity in sub cortical area,

deep white matter in bilateral fronto-parietal region deep white matter in bilateral fronto-parietal region relatively bulky left optic nerve with subtle T2 relatively bulky left optic nerve with subtle T2

hyper-intensity in pre-chiasmatic part and left side hyper-intensity in pre-chiasmatic part and left side of optic nerveof optic nerve

MRI Brain shows well defined ovoid area of T2 and FLAIR hyper-intensity in the MRI Brain shows well defined ovoid area of T2 and FLAIR hyper-intensity in the subcortical area and deep white matter in bilateral fronto-parietal regionsubcortical area and deep white matter in bilateral fronto-parietal regionA relatively bulky left optic nerve with subtle T2 hyper-intensity in pre-chiasmatic A relatively bulky left optic nerve with subtle T2 hyper-intensity in pre-chiasmatic part and left side optic nerve is also seenpart and left side optic nerve is also seen

CSF analysisCSF analysis

CSF/ Serum Quotient reference (CSFQ ref): 7.58 CSF/ Serum Quotient reference (CSFQ ref): 7.58 (value of more than 1.5 is considered to be indicative (value of more than 1.5 is considered to be indicative

of Measles specific antibody synthesis in CNS)of Measles specific antibody synthesis in CNS) Serum Measles IgG and IgM levels : within Serum Measles IgG and IgM levels : within

reference valuesreference values

EEGEEG

Intermittent episodes of Delta-Theta slowing with Intermittent episodes of Delta-Theta slowing with occasional spike wave discharges arising occasional spike wave discharges arising independently from bilateral central hemispheres independently from bilateral central hemispheres along with mild diffuse Theta slowing. along with mild diffuse Theta slowing.

Final DiagnosisFinal Diagnosis

SSPESSPE Typical fundus and OCT findingsTypical fundus and OCT findings Elevated CSF/ Serum Quotient reference (indicative Elevated CSF/ Serum Quotient reference (indicative

of Measles specific antibody production in CNS) of Measles specific antibody production in CNS) Subnormal EEG changes Subnormal EEG changes

2 weeks follow up2 weeks follow up

RE Progressive foveal atrophy and optic disc pallorRE Progressive foveal atrophy and optic disc pallor

LE Progressive necrosis of retina sparing ILMLE Progressive necrosis of retina sparing ILM BCVA at 2 weeks: RE counting fingers, LE no light BCVA at 2 weeks: RE counting fingers, LE no light

perceptionperception

Fig.4 Right eye, Fundus photograph at 2 weeks Fig.4 Right eye, Fundus photograph at 2 weeks showing pale optic disc & atrophic foveashowing pale optic disc & atrophic fovea

Fig.4Fig.4

Fig.7Fig.7Fig.6Fig.6

Fig.5Fig.5

Fig.5 Right eye, OCT at 2 weeks follow up showing foveal atrophyFig.5 Right eye, OCT at 2 weeks follow up showing foveal atrophy

Fig.6 Left eye, Fundus photograph at 2 weeks Fig.6 Left eye, Fundus photograph at 2 weeks follow up shows pale optic disc, atrophic follow up shows pale optic disc, atrophic fovea and resolving hemorrhagic retinitisfovea and resolving hemorrhagic retinitis

Fig.7 Left eye, OCT at 2 weeks follow up showing Fig.7 Left eye, OCT at 2 weeks follow up showing characteristic disintegration of all retinal layerscharacteristic disintegration of all retinal layers

ConclusionConclusion

Though ocular findings do not constitute a part of the Though ocular findings do not constitute a part of the diagnostic criteria of SSPE, ophthalmic manifestations may be diagnostic criteria of SSPE, ophthalmic manifestations may be the first sign of the disease.the first sign of the disease.

Necrosis of all retinal layers barring the ILM and Bruch’s Necrosis of all retinal layers barring the ILM and Bruch’s membrane as seen on OCT in our case is a new observation. membrane as seen on OCT in our case is a new observation.

Early diagnosis of SSPE is critical before neurological Early diagnosis of SSPE is critical before neurological involvement, when the disease becomes invariably fatal, and involvement, when the disease becomes invariably fatal, and requires high index of suspicion. requires high index of suspicion.

The OCT findings as noted in this patient may provide a vital The OCT findings as noted in this patient may provide a vital clue to the early diagnosis of SSPE. clue to the early diagnosis of SSPE.