Recurrent Drop Attacks as Presenting Manifestation of Subacute Sclerosing Panencephalitis: An Atypical PresentationFunding None.
20 February 2017
22 May 2017
20 July 2017 (eFirst)
Subacute sclerosing panencephalitis (SSPE) is a potentially fatal progressive complication of measles virus infection of the brain occurring in children and adolescents. It typically presents with a progressive decline in cognitive abilities along with periodic myoclonic jerks. We report a case of SSPE with atypical presentation. Our patient presented with intermittent drop attacks without any obvious myoclonic jerks or cognitive decline making his diagnosis difficult. Atypical manifestations of SSPE occur in 10% of patients, and a high index of suspicion is necessary for early diagnosis and management of this dreaded disease.
Keywordssubacute sclerosing panencephalitis - measles virus - drop attacks - cognitive abilities - myoclonic jerks - periodic burst
Written informed consent was taken from the patient/parents/legal guardians/next of kin for reporting this case.
- 1 Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 2002; 78 (916) 63-70
- 2 Mehta NJ, Surti BM, Gamit DB. Case report of atypical presentation of SSPE with ADEM. Natl J Med Res 2015; 5 (04) 323-324
- 3 Jovic JN. Subacute sclerosing panencephalitis with atypical early features. Child Neurol 2010; 38 (19) 19-26
- 4 Panda A, Mehta V, Maheshwari S, Kar S. Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis and pseudotumourcerebri. BMJ Case Rep 2013; 20 (13) bcr009432 . Doi: 10.1136/bcr-2013-009432
- 5 Comert S, Vitrinel A, Gursu HA, Deniz NC, Akin Y. Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis. Indian J Pediatr 2006; 73 (12) 1119-1121
- 6 Sharma S, Sawhney IM, Lal V, Gupta SK. Subacute sclerosing panencephalitis presenting as cortical blindness. J Assoc Physicians India 1995; 43 (06) 434
- 7 Verma R, Gupta R, Junewar V, Panwar A. Isolated vision loss and disappearing lesions as the manifestation of subacute sclerosing panencephalitis. Ann Indian Acad Neurol 2014; 17 (04) 477-478
- 8 Dowell SF, Sejvar JJ, Riek L. , et al. Nodding syndrome. Emerg Infect Dis 2013; 19 (09) 1374-1384
- 9 Spencer PS, Mazumder R, Palmer VS. , et al; other Members of the Oregon-Uganda Nodding Syndrome Research Team. Environmental, dietary and case-control study of Nodding Syndrome in Uganda: a post-measles brain disorder triggered by malnutrition?. J Neurol Sci 2016; 369: 191-203
- 10 Santoshkumar B, Radhakrishnan K. Substantial spontaneous long-term remission in subacute sclerosing panencephalitis (SSPE). J Neurol Sci 1998; 154 (01) 83-88
- 11 Gokcil Z, Odabasi Z, Demirkaya S, Eroglu E, Vural O. α-interferon and isoprinosine in adult-onset subacute sclerosing panencephalitis. J Neurol Sci 1999; 162 (01) 62-64