Subacute sclerosing panencephalitis presenting as neuromyelitis optica

Abstract

SUMMARYSubacute sclerosing panencephalitis (SSPE) is a slowly progressing inflammatory and degenerative disorder of the brain caused by a mutant measles virus. The diagnosis of SSPE is based on characteristic clinical and EEG findings ( periodic complexes) and demonstration of elevated antibody titres against measles in cerebrospinal fluid. SSPE can have atypical clinical features at the onset. The authors here report a case of a 3-year-old child who presented with vision loss followed 15 months later by quadriparesis with bladder involvement. These clinical features resembled that of neuromyelitis optica. However, as the disease progressed, appearance of myoclonic jerks, periodic discharges on EEG and positive cerebrospinal fluid serology for measles led to the final diagnosis of SSPE. BACKGROUND

Myoclonic-Atonic Epilepsy Masquerading as Subacute Sclerosing Panencephalitis: A Clinical Conundrum

“…However, acute-onset GTCS as the type of seizure marking the beginning of illness and the absence of a history of clinical measles in a vaccinated child were the red flags that mandated a further evaluation of the child. With such a debilitating course, one of the possibilities was fulminant SSPE, which comprises approximately 10% of all cases of SSPE [8]. GTCS, focal tonic seizures, and other seizure types have been mentioned in the literature as atypical presentations of SSPE, which delay the diagnosis of SSPE until classical features appear [8][9][10].…”

“…With such a debilitating course, one of the possibilities was fulminant SSPE, which comprises approximately 10% of all cases of SSPE [8]. GTCS, focal tonic seizures, and other seizure types have been mentioned in the literature as atypical presentations of SSPE, which delay the diagnosis of SSPE until classical features appear [8][9][10]. Three patterns of EEG findings have been mentioned, with periodicity being common to all.…”