Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis
- 48 Downloads
Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistant measles virus infection of the central nervous system. In majority of cases onset occurs from 5–15 years of age.1 In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years.2 SSPE generally occurs 5–10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, meales antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum, and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically beddiverse and a thorough differential diagnosis is mandatory for a definite diagnosis.
Key wordsSubacute sclerosing panencephalitis Acute disseminated encephalomyelitis Chronic encephalitis Childhood
Unable to display preview. Download preview PDF.
- 1.Maldonado Y. Subacte sclerosing panencephalitis. In Behrman RE; Kliegman RM, Jenson HB, eds.Nelson Textbook of Pediatrics. 17th edition. Philadelphia: WB Saunders, 2004: 1030–1031.Google Scholar
- 2.Ferichel GM. Subacute sclerosing panencephalitis. In Fenichel GM, ed.Clinical Pediatric Neurology. 4th edition. Philadelphia: WB Saunders, 2001:142.Google Scholar
- 3.Johnston M.V. Encephalopathies. In Behrman RE, Kliegman RM, Jenson HB, eds.Nelson Textbook of Pediatrics. 17th edition. Philadelphia: WB Saunders, 2004: 2027–2028.Google Scholar