Fulminant Early Onset Subacute Sclerosing Panencephalitis

Excerpt

To the Editor: A 27-mo-old boy presented with increasing frequency of recurrent drop attacks for 6 wk. He had become bedridden for the past 3 wk. The child had a history of measles like illness at the age of 1 y, despite having received a single dose of measles vaccine at 9 mo. On examination, he was bedridden; unable to speak meaningfully; unable to recognize caregivers; or understand any commands. He had continuous involuntary choreiform movements, and periodic myoclonic jerks. His growth parameters were normal. Cranial nerve examination revealed absent visual fixation, normal fundus, preserved pupillary reflexes, and only a startle response to sound. Motor examination revealed generalized hypotonia and brisk deep tendon reflexes. On investigations, EEG revealed a slow background for age and periodic large amplitude slow wave complexes. MRI brain showed bilateral T2/FLAIR white matter hyperintensities in bilateral deep peritrigonal areas (Fig. 1). Cerebrospinal fluid and serum anti-measles antibody (IgG) was positive in titre of 1:625. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was concluded. The child was started on valproate and clonazepam for myoclonus. Isoprinosine and interferons could not be started in view of financial constraints. At last follow up six months after discharge the child was in vegetative state.

…