Question

A child presents with myoclonus jerks and decreased performance in school. There is a history of fever at the age of 1 year with rash. It is suggestive of which condition?

• A. Subacute sclerosing panencephalitis
• B. Mesial temporal sclerosis
• C. Polio
• D. Measles

Hint:

Think about which late neurological complication follows measles infection in early childhood after a latent period of years.

Answer 1

The Correct Option is A

SSPE -- Subacute Sclerosing Panencephalitis:

Etiology: Persistent, defective measles virus (mutant M-protein) in the CNS. The virus cannot assemble properly but replicates slowly, causing progressive inflammation and demyelination.

Epidemiology: Risk is highest with measles infection before age 2. Latent period = 6-8 years. Preventable by measles vaccination.

Clinical stages:

1. Stage I: Personality changes, declining school performance, forgetfulness
2. Stage II: Myoclonic jerks (periodic, every 5-10 sec), seizures, EEG shows periodic synchronous discharges (Radermecker complexes)
3. Stage III: Progressive dementia, autonomic dysfunction, rigidity
4. Stage IV: Vegetative state, death within 1-3 years of onset

Diagnosis: CSF shows elevated IgG antibodies to measles; CSF-to-serum measles antibody ratio is elevated. EEG: Radermecker complexes pathognomonic.

Treatment: No curative treatment. Inosiplex + intrathecal interferon may slow progression.

\[\boxed{\text{Subacute Sclerosing Panencephalitis (SSPE)}}\]