Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays. A 9-year-old girl presented with poor balance and ataxia following an episode of upper respiratory tract infection. Neurological examination revealed mild hemiparesis and ataxia. Brain magnetic resonance imaging revealed scattered areas of T2 and fluid-attenuated inversion recovery hyperintensities in the white matter consistent with acute disseminated encephalomyelitis. Despite treatment with intravenous methylprednisolone, intravenous immunoglobulins, and plasmapheresis, progressive neurological worsening occurred. Later during the course of her illness, subacute sclerosing panencephalitis was suspected from the appearance of burst-suppression pattern on electroencephalogram, and the diagnosis confirmed by elevated titers of measles antibodies in cerebrospinal fluid. Physicians taking care of children need to be aware of atypical presentations of subacute sclerosing panencephalitis and must have a high index of suspicion to prevent diagnostic delays and avoid unnecessary diagnostic and therapeutic interventions.
Prashanth LK , Taly AB, Sinha S., Ravi V.Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital . J Child Neurol. 2007;22:683-688.
4.
Salehi HR, Delgado E., Wolf SM, McGoldrick PE, Moshé SLSubacute sclerosing panencephalitis. Pediatr Neurol. 2005;33: 280-282.
5.
Sharp GB, Laney SM, Westmoreland BF, Groover RVAtypical electroencephalographic pattern in a patient with subacute sclerosing panencephalitis. Electroencephalogr Clin Neurophysiol. 1991;78:311-313.
6.
Kubota T., Okumura A., Takenaka J., et al. A case of subacute sclerosing panencephalitis preceded by epileptic seizures: evolutional EEG changes. Brain Dev. 2003;25:279-282.
7.
Zwiauer K., Forstenpointner E., Popow-Kraupp T., Hauser E., Jellinger KARapid progressive subacute sclerosing panencephalitis after perinatally acquired measles virus infection. Lancet. 1995;345:1124.
8.
Cruzado D., Masserey-Spicher V., Roux L., Delavelle J., Picard F., Haenggeli CAEarly onset and rapidly progressive subacute sclerosing panencephalitis after congenital measles infection. Eur J Pediatr. 2002;161:438-441.
9.
Duband AC, Jallon P.EEG-mediated diagnosis of an unusual presentation of SSPE. Clin Neurophysiol. 2003;114:737-739.
10.
Sawaishi Y. , Abe T., Yano T., Ishikawa K., Takada G.SSPE following neonatal measles infection . Pediatr Neurol. 1999;20: 63-65.
11.
Vieker S., Schmitt JJ, Behrens C., Weissbrich B., Hartmann H.Subacute sclerosing panencephalitis in two brothers. Neuropediatrics . 2003;34:326-329.
12.
Tuxhorn IEFamilial subacute sclerosing panencephalitis in two siblings. Pediatr Neurol. 2004;31:291-294.
13.
Park SY, Kohl S.Subacute sclerosing panencephalitis in an identical twin. Pediatrics. 1999;104:1390-1394.
14.
Yilmaz D., Aydin OF, Senbil N., Yuksel D.Subacute sclerosing panencephalitis: is there something different in the younger children? Brain Dev. 2006;28:649-652.
15.
Singer C., Lang AE, Suchowersky O.Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature. Mov Disord. 1997;12:342-353.
16.
Kornberg AJ , Harvey AS, Shield LKSubacute sclerosing panencephalitis presenting as simple partial seizures. J Child Neurol. 1991;6:146-149.
17.
Ishikawa A. , Murayama T., Sakuma N., Saito Y.Subacute sclerosing panencephalitis: atypical absence attacks as first symptom. Neurology. 1981;31:311-315.
18.
Dimova PS, Bojinova VSCase of subacute sclerosing panencephalitis with atypical absences and myoclonic-atonic seizures as a first symptom. J Child Neurol. 2004;19:548-552.
19.
Demirbilek V., Benbir G., Cokar O., Yalcin D., Bulut H., Dervent A.Tonic seizures in subacute sclerosing panencephalitis: a video-illustration of two cases. Epileptic Disord. 2005;7: 221-225.
20.
Aydin OF, Senbil N., Gurer YKNonconvulsive status epilepticus on electroencephalography in a case with subacute sclerosing panencephalitis. J Child Neurol. 2006;21:256-260.
21.
Tuncel D., Ozbek AE, Demirpolat G., Karabiber H.Subacute sclerosing panencephalitis with generalized seizure as a first symptom: a case report. Jpn J Infect Dis. 2006;59:317-319.
22.
Ozyurek H., Degerliyurt A., Turanli G.Subacute sclerosing panencephalitis presenting with hemiparesis in childhood: case report. J Child Neurol. 2003;18:363-365.
23.
Korkmaz B., Unal T., Citav N.Transcortical sensory aphasia in a child with subacute sclerosing panencephalitis . Dev Med Child Neurol. 2007;49:558.
24.
Ondo WG, Verma A.Physiological assessment of paroxysmal dystonia secondary to subacute sclerosing panencephalitis. Mov Disord. 2002;17:154-157.
Shivji ZM, Al-Zahrani IS, Al-Said YA, Jan MMSubacute sclerosing panencephalitis presenting with unilateral periodic myoclonic jerks. Can J Neurol Sci. 2003;30:384-387.
27.
Tan H., Orhan A., Buyukavci M., Kocer I.Pseudotumor cerebri secondary to subacute sclerosing panencephalitis. J Child Neurol. 2004;19:627-629.
28.
Duman O., Balta G., Metinsoy M., Haspolat S.Unusual manifestation of subacute sclerosing panencephalitis: case with intracranial high-pressure symptoms. J Child Neurol. 2004 ;19: 552-555.
29.
Caplan R., Tanguay PE, Szekely AGSubacute sclerosing panencephalitis presenting as childhood psychosis. J Am Acad Child Adolesc Psychiatry. 1987;26:440-443.
30.
Forrest G., Stores G.Subacute sclerosing panencephalitis presenting with psychosis and possible sexual abuse. Eur Child Adolesc Psychiatry. 1996;5:110-113.
31.
Kayal M., Varghese ST, Balhara YPPsychiatric manifestation of SSPE. J Neuropsychiatry Clin Neurosci. 2006;18:560.
32.
Duncalf CM, Kent JN, Harbord M., Hicks EPSubacute sclerosing panencephalitis presenting as schizophreniform psychosis . Br J Psychiatry. 1989;155:557-559.
33.
Senbil N., Aydin OF, Orer H., Gurer YKSubacute sclerosing panencephalitis: a cause of acute vision loss. Pediatr Neurol. 2004;31:214-217.
34.
Caruso JM, Robbins-Tien D., Brown WD, Antony JH, Gascon GGAtypical chorioretinitis as an early presentation of subacute sclerosing panencephalitis . J Pediatr Ophthalmol Strabismus. 2000;37:119-122.
35.
Tandon R., Khanna S., Sharma MC, Seshadri S., Menon V.Subacute sclerosing panencephalitis presenting as optic neuritis. Indian J Ophthalmol. 1999;47:250-252.
36.
Sharma S., Sawhney IM, Lal V., Gupta SKSubacute sclerosing panencephalitis presenting as cortical blindness. J Assoc Physicians India. 1995;43:434.
Ekmekci O., Karasoy H., Gokcay A., Ulku A.Atypical EEG findings in subacute sclerosing panencephalitis. Clin Neurophysiol. 2005;116:1762-1767.
39.
Chung BH, Ip PP, Wong VC, Lo JY, Harding B.Acute fulminant subacute sclerosing panencephalitis with absent measles and PCR studies in cerebrospinal fluid. Pediatr Neurol. 2004;31:222-224.
40.
Arora SC, Al-Tahan AR, Al-Zeer A., Al-Tahan F., Ozo CO, ur-Rahman N.Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis: a case report. J Neurol Sci. 1997;146:13-18.
41.
Bobele GBSubacute encephalopathy in a 5-year-old boy. Semin Pediatr Neurol. 1999;6:168-172.
42.
Woelfle J., Haverkamp F., Albert F.Long-term survival and temporary clinical improvement in subacute sclerosing panencephalitis. Pediatr Neurol. 1996;15:83.
43.
Risk WS, Risk WS 2nd.Subacute sclerosing panencephalitis with remission in a Bosnian refugee child . Pediatr Infect Dis J. 2003;22:757-758.
44.
Prashanth LK, Taly AB, Ravi V., Sinha S., Rao S.Long term survival in subacute sclerosing panencephalitis: an enigma. Brain Dev. 2006;28:447-452.
45.
Anlar B., Saatci I., Kose G., Yalaz K.MRI findings in subacute sclerosing panencephalitis. Neurology . 1996;47:1278-1283.
46.
Brismar J., Gascon GG, Von Steyen KV, Bohlega S.Subacute sclerosing panencephalitis: evaluation with CT and MR. Am J Neuroradiol. 1996;17:761-772.
Senol U., Haspolat S., Cevikol C., Saatci I.Subacute sclerosing panencephalitis: brain stem involvement in a peculiar pattern. Neuroradiology. 2000;42:913-916.
49.
Comert S., Vitrinel A., Gursu HA, Deniz NC, Akin Y.Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis . Indian J Pediatr. 2006;73:1119-1121.
50.
American Academy of Pediatrics.Measles. In: Pickering LK, Baker CJ, Long SS , McMillan JA, eds. Red Book2006 Report of the committee on infectious diseases. 27th ed. Elk Grove Village : American Academy of Pediatrics; 2006:448.
