Demographic, clinical, imaging, and EEG characteristics of subacute sclerosing panencephalitis (SSPE)

Abstract

Objective: To describe the demographic, clinical, imaging, and EEG characteristics of Filipino children with confirmed SSPE. Methods: Thirty-three children below 18 years of age diagnosed with SSPE admitted in a tertiary hospital in the Philippines from January 2005 to December 2015 were included in the study. Medical charts were reviewed and the diagnosis were based on clinical features supported with CSF and serum measles antibody titers, neuroimaging, and EEG. Frequency distribution tables were used. Results: Sixty percent were males and forty percent were females. Mean age was 8.3 years. Majority (68%) came from lower socio-economic group. An average duration of symptoms was 86.5 days. Forty-five percent had history of measles infection, 79% were vaccinated against measles and 21% were unimmunized. Clinical signs-symptoms were personality changes (54%), cognitive decline (51%), decreased school performance (45%), gait disturbance (88%), bowel and urinary changes (21%), and fall to ground (76%). Myoclonic seizures (97%) were common, also generalized tonic clonic (36%), focal (15%), tonic (12%), and atonic (9%) seizures were seen. Twenty-one (64%) presented with clinical stage II and almost were CSF and serum measles specific IgG positive (91%). Abnormal neuroimaging findings showed cortical atrophy (15%), white matter hyperintense signal (45%), and ischemic changes (9%). EEG findings include periodic burst suppression (58%), generalized spikes (15%), and slow wave (30%) discharges. Isoprinosine (76%) and interferon beta (3%) were used in some of the patients. Sodium valproate was the most common antiepileptic followed by carbamazepine (36%). Nine (27%) were readmitted and 1 (9%) expired due to severe sepsis. Conclusion: SSPE continues to be a fatal disease. Affected individuals typically present with intellectual deterioration, myoclonus, seizures, and later death. This study demonstrates that we are essentially unable to alter the natural course of the disease despite medical treatment and supportive therapy. There is no ideal treatment for SSPE.