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Ocular manifestations in subacute sclerosing panencephalitis

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Review Article

Author Details : Yamini Sahu, Niharika Chaudhary*, Priyanka Singh, Ritika Aggarwal

Volume : 5, Issue : 3, Year : 2019

Article Page : 272-274

https://doi.org/10.18231/j.ijceo.2019.065

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Abstract

Subacute sclerosing panencephalitis is a neurological disorder that leads to progressive neuronal damage caused by persistent measles virus. The usual age of onset of the disease is in childhood between the age of 10 to 14 years. Ocular manifestations are seen in 10 to 50% of the patients of SSPE. Common ocular findings include optic neuritis, papilledema, papillitis, optic atrophy, macular chorioretinitis, cortical blindness. Ophthalmic findings may occur along with or may sometimes precede the neurological involvement by few years. There is higher incidence in rural population, children with lower birth order, having 2 or more siblings. SSPE is twice as common in boys as in girls. Initial presentation starts as mild behavioral changes, progressing to specific motor and neurological disturbances in the form of myoclonic jerks, motor disturbances leading to involuntary muscle contractions, gait abnormalities, speech changes, pyramidal and extrapyramidal signs, amnesia, generalized tonic clonic seizures and partial seizures. Macular retinochoroiditis is the most common ocular finding in SSPE. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic slow wave complexes in EEG, and presence of increased antibody titre against measles virus in the plasma and cerebrospinal fluid (three out of five criteria given by Dyken are fulfilled). Treatment for SSPE is still undetermined. Various drug combination therapies have been used for the treatment of SSPE. Isoprinosine was the first drug reported to be effective against SSPE due to its immunomodulatory effects. Alpha interferon is also used for the management of SSPE. Studies suggest that even beta interferon can be used as a management option. Ribavirin, an antiviral drug, is effective against many RNA virusesSubacute sclerosing panencephalitis is a neurological disorder that leads to progressive neuronal damage caused by persistent measles virus. The usual age of onset of the disease is in childhood between the age of 10 to 14 years. Ocular manifestations are seen in 10 to 50% of the patients of SSPE. Common ocular findings include optic neuritis, papilledema, papillitis, optic atrophy, macular chorioretinitis, cortical blindness. Ophthalmic findings may occur along with or may sometimes precede the neurological involvement by few years. There is higher incidence in rural population, children with lower birth order, having 2 or more siblings. SSPE is twice as common in boys as in girls. Initial presentation starts as mild behavioral changes, progressing to specific motor and neurological disturbances in the form of myoclonic jerks, motor disturbances leading to involuntary muscle contractions, gait abnormalities, speech changes, pyramidal and extrapyramidal signs, amnesia, generalized tonic clonic seizures and partial seizures. Macular retinochoroiditis is the most common ocular finding in SSPE. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic slow wave complexes in EEG, and presence of increased antibody titre against measles virus in the plasma and cerebrospinal fluid (three out of five criteria given by Dyken are fulfilled). Treatment for SSPE is still undetermined. Various drug combination therapies have been used for the treatment of SSPE. Isoprinosine was the first drug reported to be effective against SSPE due to its immunomodulatory effects. Alpha interferon is also used for the management of SSPE. Studies suggest that even beta interferon can be used as a management option. Ribavirin, an antiviral drug, is effective against many RNA viruses.

Keywords: SSPE- Subacute sclerosing panencephalitis.

How to cite : Sahu Y, Chaudhary N, Singh P, Aggarwal R, Ocular manifestations in subacute sclerosing panencephalitis. Indian J Clin Exp Ophthalmol 2019;5(3):272-274

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