Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis

Abstract

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system. In majority of cases onset occurs from 5-15 years of age. 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. 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, measles 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 be diverse and a thorough differential diagnosis is mandatory for a definite diagnosis.

Acute Disseminated Encephalomyelitis with Measles

“…However, it is difficult to differentiate from subacute sclerosing panencephalitis (SSPE) presenting as ADEM, but presence of myoclonic convulsions, latent period and no improvement with steroids can differentiate it from SSPE. 10 In myelitic form of ADEM, partial or complete paraplegia or quadriplegia and diminished or loss of reflexes also occur, which is consistent with our findings. 11 ADEM produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter.…”

Molecular Characterization of the Measles Virus Genotypes in JiLin Province, China

“…Despite the development of vaccines against MV and widespread vaccination campaigns, measles remains one of the most contagious diseases and a leading cause of child death worldwide [3]. More than 30 million new cases are reported annually, the majority of which are in children.…”

Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis and pseudotumour cerebri

“…Furthermore features of raised ICP, objective evidence of raised CSF pressure and papilloedema without any evidence of intracranial mass lesion or hydrocephalous raise the possibility of pseudotumour cerebri. Few case reports were described in literatures as an association of ADEM and SSPE 13. The association of ADEM and pseudotumour-like presentation in SSPE as an early manifestation is quite unusual.…”