SSPE Case Characteristics When Measles Contracted After Age 5
While SSPE is most commonly associated with measles infection before age 5, cases do occur in older children and adults, though they are less frequent and the clinical presentation follows similar devastating neurological patterns. 1, 2
Epidemiological Context
- The highest risk for developing SSPE occurs when measles is contracted before age 5, with an incidence of 6.5 to 11 per 100,000 measles cases overall 2
- Children who contract measles after age 5 have a lower risk of developing SSPE compared to younger children, though the disease remains possible 2
- The typical latency period between measles infection and SSPE onset is approximately 10 years, regardless of age at initial infection 3
Clinical Presentation Patterns
SSPE presents with insidious onset characterized by:
- Subtle personality changes and declining intellectual performance as initial manifestations 4
- Progressive mental deterioration followed by seizures and myoclonic jerks 4
- Motor signs that advance to coma and ultimately death 4
- Pathognomonic EEG findings showing well-defined periodic complexes with 1:1 relationship to myoclonic jerks 4
Age-Related Prognostic Differences
- Younger children (under age 4) demonstrate rapidly progressive or progressive courses with poor prognosis despite medical treatment 5
- The rate of progression in very young children tends to be more aggressive, with 6 of 9 patients under age 4 showing rapidly progressive disease on admission in one case series 5
- While specific case series for post-age-5 infections are limited in the evidence, the general principle holds that SSPE remains a fatal complication regardless of age at measles infection 1, 3
Diagnostic Approach
When SSPE is suspected:
- Detect intrathecal synthesis of measles-specific antibodies in CSF, which is a crucial diagnostic criterion 4
- Obtain EEG to identify characteristic periodic complexes 4
- Consider PCR testing of CSF for measles virus RNA, though antibody testing is often more reliable 4
- Look for oligoclonal bands in CSF with immunoblotting against measles virus proteins 4
Management Limitations
- No cure exists for SSPE; most therapies focus on supportive needs 2
- Seizures and abnormal movements may respond to carbamazepine 2
- Intrathecal ribavirin has been used with limited success 4
- Treatment options include symptomatic control with antiepileptic drugs, interferon combined treatment, vitamin A, ribavirin, and ketogenic diet for disease modification 3
Critical Prevention Message
Measles vaccination is the only effective prevention strategy for SSPE, and widespread vaccination has essentially eliminated SSPE from countries with high vaccination coverage 1, 6, 2