ABSTRACT
Subacute Sclerosing Panencephalitis (SSPE) is a rare, progressive degenerative disease of the brain caused by reactivation of aberrant measles virus. Classical symptoms of SSPE are described as behavioral changes, declining scholastic performance, myoclonic jerks etc. However, at times it presents with varied atypical manifestations, which makes diagnosis dif?cult. Hereby we report two patients with unusual manifestations of SSPE. In ?rst case, 14-year-old boy presented with recurrent falls of 9 months duration. Initially, it was 1-2 times per week, progressing over 9 months to frequency of 40-50 falls per day. In second case, 21-year-old gentleman presented with behavioral changes and memory disturbance of 1-year duration. However, his family members noticed worsening of symptoms 1 month before presentation as he started developing involuntary jerky movements involving the right upper and lower limb. In both of them electroencephalogram (EEG) was showing long interval periodic discharges and cerebrospinal ?uid was showing elevated titers of anti-measles antibody overall suggesting SSPE. Hence it needs much attention for atypical presentations and also considering SSPE in differential diagnosis of unsolved atypical neurological presentations.
ABSTRACT
@#Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus in the brain. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonic jerks and eventually complete neurologic deterioration. In about 10% of patients, the clinical manifestations of SSPE are not typical and that may cause a delay in the diagnosis. We report here 3 cases of SSPE with atypical presentations. Bilateral vision loss, hemiparesis, ataxia and acute encephalopathy with focal seizures were respectively the presenting symptoms at the onset of disease. The typical periodic EEG complexes in two patients and positive CSF measles IgG antibody led to the diagnosis of SSPE.